guidelines management of grown up congenital heart disease · table of contents 1. introduction and...

Guidelines

Management of Grown Up Congenital HeartDiseaseThe Task Force on the Management of Grown UpCongenital Heart Disease of the European Society ofCardiologyTask Force members, Chairperson, John Deanfield*, Erik Thaulow,Carol Warnes, Gary Webb, Frantizek Kolbel, Andreas Hoffman,Keld Sorenson, Harald Kaemmerer, Ulf Thilen,Margreet Bink-Boelkens, Laurence Iserin, Luciano Daliento,Eric Silove†, Andrew Redington, Pascal VouheESC Committee for Practice Guidelines (CPG), Chairperson,Silvia Priori, Maria Angeles Alonso, Jean-Jacques Blanc,Andrzej Budaj, Martin Cowie, Jaap Deckers,Enrique Fernandez Burgos, John Lekakis, Bertil Lindahl,Gianfranco Mazzotta, Joao Morais, Ali Oto, Otto Smiseth,Hans Joachim TrappeDocument Reviewers, CPG Review Coordinator, Jaap Deckers,Werner Klein, Former CPG Chairperson, Maria Angeles Alonso,Carina Blomstrom-Lundqvist, Guy de Backer, Jaromir Hradec,Gianfranco Mazzotta, Alexander Parkhomenko, Patrizia Presbitero,Adam Torbicki

KEYWORDSGrown-up congenitalheart disease;Congenital heartdisease;Management;Specialist centres;Organization of care

* Address for correspondence: John Deanfield, Chairperson Task Force on GUCH of the European Society of Cardiology, GUCH Unit,The Heart Hospital, 16018 Westmorland Street, London W19 8PH. Tel.: +44-20-207-404-50-94; fax: +44-20-207-813-83-62; e-mail:[email protected]

† Representative of the Association for European Paediatric Cardiology, UK

European Heart Journal (2003) 24, 1035–1084

0195-668X/03/$ - see front matterdoi:10.1016/S0195-668X(03)00131-3

Table of Contents

1. Introduction and background .................10371.1. Size and composition of the

GUCH population .............................1038

2. Organization of care ...........................10382.1. Transition from paediatric to

adult care .....................................10382.2. Network of specialist centres with models

of delivery ....................................10392.2.1. An overview of the current situation ...10392.2.2. The beginnings of a solution .............10402.3. Delivery of patient care ....................10402.3.1. Management recommendations .........1040

3. Training of practitioners in grown-upcongenital heart disease ......................1041

4. Medical issues ...................................10424.1. Ventricular function .........................10424.1.1. Echo-Doppler ...............................10434.1.2. Magnetic resonance imaging .............10434.1.3. Radionuclide studies ......................10434.1.4. Invasive-studies ............................10434.2. Arrhythmia and pacemakers ...............10444.3. Cyanosis in the GUCH patient ..............10454.3.1. Haematologic problems ..................10454.3.2. Haemostasis ................................10454.3.3. Renal function .............................10454.3.4. Gallstones ..................................10454.3.5. Orthopaedic complications ..............10454.3.6. Skin ..........................................10454.4. Pulmonary vascular disease ................10454.5. Infective endocarditis .......................10464.6. Imaging in adults with congenital

cardiac disease ...............................10474.7. Interventional catheterization .............10474.7.1. Techniques .................................10474.7.1.1. Balloon dilation .........................10474.7.1.2. Balloon dilatation with

stent implantation ......................10484.7.1.3. Embolization and occlusion

techniques ...............................10484.7.1.4. Percutaneous valve implantation .....10494.8. Pregnancy and GUCH ........................10494.9 Genetic counselling and contraception

in GUCH ........................................10514.9.1. Recurrence risk/genetic counselling ...10524.10. Comorbidity and syndromes ..............10524.11. Emergencies – adults with congenital

cardiac disease .............................1052

5. Surgical issues ..................................10535.1. Cardiac surgery ...............................10535.1.1. General planning of the operation ......10535.1.2. Specific surgical challenges ..............1053

5.1.2.1. Preservation of myocardialfunction ..................................1053

5.1.3. Blood salvage techniques ................10545.1.4. Redo sternotomy incision ................10545.1.5. Pulmonary vascular bed abnormalities .10545.1.6. Aortopulmonary collateral circulation .10545.2. Anaesthesia and post operative care .....10555.2.1. Physiology ..................................10555.2.2. Assessment .................................10555.2.3. Anaesthetic management ................10555.2.4. Monitoring ..................................10565.2.5. Post anaesthesia care .....................10565.3. Non-cardiac surgery .........................10575.3.1. Unoperated congenital heart disease ..10575.3.2. Operated congenital heart disease .....10575.3.3. Cyanotic heart disease ...................10575.4. Transplantation ..............................1058

6. Psychosocial issues .............................10586.1. Intellectual development/education .....10596.2. Employment ..................................10596.3. Insurance ......................................10596.4. Physical activity/sport ......................10606.5. Quality of life .................................10606.6. Patient organizations ........................1060

7. Specific lesions .................................1061

8. Recommendations for Future Developmentsin Europe ........................................1081

8.1. Summary ......................................10818.2. Specific recommendations .................1081

9. European Society of Cardiology Staff .......1082

Acknowledgements ................................1082

Appendices .........................................1082Adult Congenital Heart Disease Survival

Simulator ........................................1082

References ..........................................1082

Preamble

Guidelines aim to present all the relevant evidenceon a particular issue in order to help physicians toweigh the benefits and risks of a particular diag-nostic or therapeutic procedure. They should behelpful in everyday clinical decision-making.

A great number of guidelines have been issued inrecent years by different organizations-EuropeanSociety of Cardiology (ESC), American HeartAssociation (AHA), American College of Cardiology(ACC), and other related societies. By means oflinks to web sites of National Societies severalhundred guidelines are available. This profusioncan put at stake the authority and validity of guide-lines, which can only be guaranteed if they have

1036 Guidelines

been developed by an unquestionable decision-making process. This is one of the reasons why theESC and others have issued recommendations forformulating and issuing guidelines, which arequoted as a preamble or appendix in the finalreports.

In spite of the fact that standards for issuinggood quality guidelines are well defined, recentsurveys of guidelines published in peer-reviewedjournals between 1985 and 1998 have shown thatmethodological standards were not compliedwithin the vast majority of cases. It is therefore ofgreat importance that guidelines and recommen-dations are presented in formats that are easilyinterpreted. Subsequently, their implementationprogrammes must also be well conducted. At-tempts have been made to determine whetherguidelines improve the quality of clinical practiceand the utilization of health resources. In addition,the legal implications of medical guidelines havebeen discussed and examined, resulting in positiondocuments, which have been published by aspecific Task Force.

The Committee for Practice Guidelines (CPG)supervises and coordinates the preparation of newGuidelines and Expert Consensus Documents pro-duced by Task Forces, expert groups or consensuspanels. The committee is also responsible for theendorsement of these guidelines or statements.

1 Introduction and background

As a result of the success of paediatric cardiologyand cardiac surgery over the last three decades,there will shortly be more adults than children withcongenital heart disease. Prior to the advent ofsurgery, less than 20% of children born with con-genital heart malformations survived to adult life.1

Now, most deaths from congenital heart diseaseoccur in adults. The ‘new population’ of patientswith congenital heart disease no longer fits withintraditional divisions of training and practice, whichhave separated adult and paediatric cardiology.Adult cardiologists are not equipped to deal withthe range and complexity of grown-up patients withcongenital heart disease, whereas paediatric cardi-ologists cannot be expected to manage the manyacquired adult diseases in a paediatric medicalenvironment. Up till now, care has been deliveredby a number of enthusiastic centres who havemanaged the complex medical, surgical andpsychosocial needs of the grown-up patients withcongenital heart disease. In most countries, how-ever, an organized system is not yet in place. Thisis needed for continued provision of excellence in

clinical care, accumulation of knowledge about thelate outcome of management strategies in child-hood (with feedback to paediatric practice) as wellas for training.

The lack of information regarding numbers, diag-noses and treatment as well as the regular occur-rence of avoidable medical problems in thispopulation is testimony to the deficiencies of thecurrent system. The need to reintegrate paediatricand adult cardiac services, and in particular toprovide smooth ‘transition’ for adolescents is clear.

In 1994, the European Society of Cardiology rec-ognized the need for specialized care of this chal-lenging group of patients by establishing a workinggroup for Grown-Up Congenital Heart Disease(GUCH). Since then, both the size, and complexityof the GUCH population in Europe has continued toincrease. In 1996, the Canadian CardiovascularSociety commissioned a consensus conference onadult congenital heart disease and its report hasbeen invaluable in representing ‘state of the art’principles of management.2 The American Collegeof Cardiology organized the 32nd Bethesda Confer-ence on care of the adult with congenital disease in20003 and in 1999, the European Society of Cardiol-ogy established this Task Force to evaluate pro-visions for care for grown-ups with congenitalheart disease in Europe and to make recom-mendations for improvement in organization facili-ties, training, and research. Members of the TaskForce include specialists from Europe and NorthAmerica who were chosen to provide a broadview of novel information, and to developing therecommendations of previous publications.

In the first part of the Task Force report (sections1–6), the special healthcare needs of grown-upswith congenital heart disease are discussed andcommon principles of management, including tran-sition from paediatrics, the need for the establish-ment of specialist centres, models of network caredelivery and training of medical and non-medicalstaff are set out. In the second part (section 7), wehave provided structured guides for the manage-ment of common lesions. These represent a consen-sus view of the panellists, and where possible, areevidence-based. They are intended to assist thepracticing clinician in a ‘user friendly’ manner. Werecognise that there are many valid different ap-proaches and that more robust clinical research isvital to provide evidence-based recommendationsin this emerging field. Nevertheless, we believethat our ‘summaries’ will be helpful and enableclinicians to access useful information ‘at a glance’.

This report is intended to promote collaborationbetween the various professional groups involved in

Management of Grown-up Congenital Heart Disease 1037

the care of adolescents and adults with congenitalheart disease, administrators and those who pro-vide resources for health care. Sustained effort toimplement the recommendations of this Task Forcewill be required in order to bring to full fruitionthe huge successes achieved in the treatment ofcongenital heart disease in children over the lastthree decades. The participants humbly acknowl-edge the many unresolved issues and uncertaintiesof grown-up congenital heart disease and areall involved in efforts to improve care for thischallenging and rewarding group of patients.

1.1 Size and composition of the GUCHpopulation

Remarkably few data are currently available on thesize and composition of the population of grown-ups with congenital heart disease. Healthcare plan-ning and resource allocation has been based largelyon estimates of the incidence of congenital heartdisease in infancy, survival through childhood to-gether with the number of ‘new’ cases such asatrial septal defect, coarctation, Ebstein’s anomalyor congenitally corrected transposition whichmay be diagnosed for the first time in childhood,adolescence and adult life. Such estimates werepublished by the Bethesda Conference, which high-lighted the wide confidence intervals of projectednumbers.3 It has been difficult to collect ‘real’numbers because many patients, even those withcomplex defects attend non-specialized clinicsor are completely lost to follow-up.4 In TheNetherlands, for example, it is estimated thatthere should be approximately 20 000 grown-upswith congenital heart disease but only 8000 areseen in hospital clinics.5

There is an increase not only in the size of thepopulation but also in the proportion of patientswith complex lesions. Service planning needs to bebased on the numbers of grown-ups with congenitalheart disease for whom specialist care is essential.Many patients with simpler lesions (e.g. smallventricular septal defect, repaired atrial septaldefects, mild pulmonary stenosis, bicuspid aorticvalve) can be managed either exclusively in adultgeneral cardiac units or jointly with the specialistunit. In this report, we have stratified care recom-mendations into three levels (exclusive follow-up inthe specialized unit level 1, shared care with localinformed adult unit level 2 and predominantly non-specialist care level 3). The proportion of the totalpopulation of grown-ups with congenital heart dis-ease and complex lesions requiring either exclusivespecialized care or close interaction with a general

adult cardiac clinic (level 1 and 2) has been esti-mated at 25–50%. A recent survey in the North EastRegion of the United Kingdom has attempted topredict both the number of patients with congeni-tal heart disease who will survive into adulthood aswell as the number who will need specialist care.This used data from a 10-year period (1985–1994)of a congenital heart disease database togetherwith predicted survival of individual lesions fromthe published literature. When the results wereextrapolated to the whole of the United Kingdom,the predicted annual increase in numbers ofgrown-ups with congenital heart disease was atapproximately 1600, with 800 requiring specializedfollow-up.6

The number of grown-up congenital heart dis-ease patients with individual lesions depends on theincidence at birth, early mortality in childhood aswell as the rate of late death. In the absence ofhard figures we have developed a simple pro-gramme, which enables prediction of late survivalrates by entering estimates for each of these out-come determinants. This is available on the ESCwebsite (Appendix 2) and will be useful for planningof resource requirements and funding. A Europeansurvey of the numbers of grown-ups with congenitalheart disease has been commissioned by theEuropean Society of Cardiology in 24 countries,but few European countries have the necessaryestablished database.

The establishment of specialized centres to man-age the complex grown-up heart disease populationis a priority. These centres will provide the basis forresearch into new areas of cardiology, such as theinteraction between congenital and acquired heartproblems in older patients. Specialized centresshould not ‘disenfranchise’ local physicians, both ingeneral cardiology and primary care who have animportant role in a hierarchical local, regional andsupraregional service. This report provides a modelfor such care delivery, which will need to be modi-fied in each of the different health care systemsoperating in European countries.

2 Organization of care

2.1 Transition from paediatric to adult care

The arrangement of transition from the paediatriccardiology clinic to the adult service is a particularchallenge.7 ‘Adolescence’ has no absolute agelimits and a degree of flexibility is essential, de-pending on the intellectual and emotional maturityof the patient as well as other issues such asthe presence of coexisting disease.7 A specialist

1038 Guidelines

transition clinic is highly desirable to minimizeanxiety for the patient and their families as well asdisruption in care provision. This is the first stepto the creation of a successful service for thegrown-up population with congenital heart diseaseand the following recommendations can be made:

1 Paediatric cardiologists should begin to informpatients and families regarding transition fromaround the age of 12, with a flexible policy oftransition at age 14–16 years. Subsequent trans-fer to the adult service can again occur at aflexible age of approximately 18 years. Eachpaediatric cardiac unit should establish a co-ordinated process to link with a specialist centrefor grown-ups with congenital heart disease.

2 The patient and their family will have developeda firm bond with the paediatric cardiologist overmany years and it is desirable that the paediatriccardiologist is involved in the transition service,together with the adult specialist(s).

3 The transition clinic requires input from adminis-tration and other healthcare professionals. Thenurse specialist is a key person and should beexperienced in counselling of adolescents andtheir families and be responsible for coordinatingtransfer arrangements.

4 The patient and their family should be given adetailed written plan in advance of handover.This should include key information about treat-ment in childhood (such as previous investi-gations and operations). It is part of an educationprocess, which must be tailored to the varyinglevels of maturity and intellect of individualpatients. The adolescent and their family need tounderstand their cardiac condition, healthcareneeds and prognosis. In particular, they shouldknow about their medication, possible side ef-fects and interaction with other drugs (includingalcohol!) and they should be fully informed aboutendocarditis prophylaxis. They also requireguidance concerning exercise, contraception,pregnancy, career planning, travel and insur-ance. They must be properly informed of possiblefuture complications of their condition and likelyassociated symptoms. They must know how tooperate within the adult healthcare system inorder to obtain appropriate medical advice; bothlocally and when they are away from home (dur-ing studies, occupation and travel). This processmust be handled sensitively and cannot occurduring a single consultation. The patient shouldbe able to have private discussions, not onlywith the cardiologist, but also with the nursespecialist. Many children reach adolescence with

little understanding of the implications of theircondition, due to parental over protection andlack of direct discussion. Parents often find itdifficult to let go of their offspring and may needsupport to allow the adolescent to become inde-pendent. Time should be allocated to discusssensitive issues such as contraception, familyplanning, pregnancy, recurrence risk and sport.

5 The carefully considered plan of medical man-agement (including follow-up) should be based onthe patient’s condition and prognosis, educationand availability of local medical services. Unitprotocols are very useful and the plan needs to becommunicated to the primary care physician andother doctors involved (e.g. university healthcareservices). Shared care with local physicians isappropriate for many patients. This is particu-larly important in special circumstances, such asnon-cardiac surgery or emergencies. Close liaisonand good communication needs to be establishedat the transition stage.

6 Transition of care should be a gradual processboth for the patient and the medical practition-ers. It is essential not to bombard the patientwith an overwhelming amount of information,which can induce denial, and lack of attendance.There should be continued opportunities for jointdiscussion between paediatric and adult special-ists (both medical and surgical) and there shouldbe feedback from both the transition and adultclinics to the paediatric cardiology team.

2.2 Network of specialist centres withmodels of delivery

2.2.1 An overview of the current situationThe healthcare needs of many grown-ups withcongenital heart disease are not currently beingmet in Europe and elsewhere. Two fundamentalimpediments to the improvement of their care are:

1 A critical shortage of trained and experiencedprofessionals of the types described below.

2 An inadequate number of centres of excellence tolead the national and regional efforts to providehigh quality care. A structured hierarchical modelof care delivery is required which should beregionalized. The establishment of major fullservice ‘centres’ would contrast with the excessof partial service ‘centres’ currently caring forthis population. To be effective, the majorcentres must have sufficient volumes of patientsand procedures to develop and maintain highlevels of performance.8 There is an urgent need ofleadership from governments and professional


organizations to work towards such a coordinatedsystem aimed at optimizing patient care.

2.2.2 The beginnings of a solutionGiven the shortage of staff with training and exper-tise in the care of grown-ups with congenital heartdisease, improvement must start by developing andemploying the skilled personnel needed to lead andhelp coordinate this work in specialist centres. Thisprocess would serve as a focus for excellencein clinical care, as well as enabling training andresearch.

The specialist unit should be located in an adultmedical environment with multi-disciplinary speci-ality provision and be associated with strong paedi-atric cardiology groups. Indeed, all specialistcentres for paediatric cardiology must have definedcare pathways for the appropriate transfer ofpatients to the grown-up congenital heart diseaseservice. Each specialist centre should serve a popu-lation of approximately 5–10 million people andthey should function within their local medicalcommunities. Cardiologists and primary care physi-cians should be encouraged to establish a referralrelationship with the specialist centres, and thisshould include provision of timely telephone ad-vice, informal consultation, rapid consultant refer-rals as well as collaboration in patient follow-up(the specialist centre should include cardiologist(s)with training in management of grown-ups withcongenital heart disease in a collaborative teamincluding cardiac surgeons, anaesthetists and in-tensivists). The cardiologist should be familiarwith echocardiography (including transoephagealechocardiography) and diagnostic cardiac cath-eterization and at least one per centre should haveexperience in interventional catheterization. Ac-cess to an electrophysiologist with expertise inarrhythmia management in congenital heart dis-ease, pacemaker insertion, ablation and defibril-lator implantation is also essential. Specialistimaging including MRI and CT is required. Thereshould be close links with other specialist depart-ments and, in particular, the provision of a jointservice with obstetrics to manage high-risk preg-nancies. Access to a cardiac pathologist with aninterest in congenital cardiac malformations is alsohighly desirable. A minimum of two congenitalheart surgeons (often shared with paediatric cardi-ology units) is needed, together with the appropri-ate anaesthetists and intensive care and surgicalteams. An association with a transplant centreshould also be established.

Specialist nurses are crucial and often providethe ‘glue’ that connects the various components, as

well as delivering excellence in patient care. All ofthe unit staff need to work in a dedicated environ-ment with appropriate in-patient and out-patientfacilities and a full range of invasive and non-invasive investigational techniques (see below).

2.3 Delivery of patient care

Patients should be transferred to the service forgrown-up congenital heart disease with a clearmanagement and follow-up plan, with transferof appropriate information (see above 2.1). Allpatients should be seen for initial consultation inthe specialist adult cardiac services at least once,to establish a relationship with the team as well asto provide familiarity with the new environment.This process will minimize the number of patientswho are lost to follow-up during this key period.

Subsequently, patient follow-up care can bestratified in to three levels:

1 Patients who require care exclusively in thespecialist centre

2 Patients in whom shared care can be establishedwith the appropriate general adult cardiacservices

3 Patients who can be managed in ‘non-specialist’clinics (with access to specialized care ifrequired).

These levels are used in the care recommen-dations for individual lesions in section 7 of thisdocument.

Patients with congenital heart disease whoshould be seen within the specialist centre includethose with the prospect of premature death, re-operation or complications of their condition andthose whose condition is unfamiliar to general car-diologists. Any patient who develops a new clinicalproblem related to their congenital heart defectshould be referred for re-evaluation in the special-ist unit. Furthermore, consultation should occurwith the specialist unit prior to any intervention ina grown-up with congenital heart disease. A tele-phone call may be all that is required to avoida disaster during a seemingly innocuous proce-dure (e.g. non-cardiac surgery in an Eisenmengerpatient).

2.3.1 Management recommendationsEuropean representatives of the GUCH WorkingGroup of the ESC have had substantial input into thepreviously published for the management of thesepatients prior to this report.2,3 Physicians andpatients who are interested in accessing such infor-mation may connect to it on www.achd-library.com

1040 Guidelines

or access the ESC website (www.escardio.org) forthe current Task Force recommendations. Theseprovide point-to-point summaries of the major con-ditions including management recommendationsand supporting references. Recruitment and train-ing efforts to build professional teams who willbecome regional anchors of care should be begunwithout delay, in order to address the manpowershortage of adequately trained and experiencedexperts in this field.

3 Training of practitioners in grown-upcongenital heart disease

Despite the fact that many European national train-ing guidelines for cardiology recognise the need fortraining in congenital heart disease, most cardiolo-gists have virtually no experience or understandingof management of grown-ups with congenital heartdisease. Most units, therefore, rely on an extendedrole for paediatric cardiologists who work in con-junction with ‘interested’ adult cardiology col-leagues. There is an urgent need to improve thissituation by defining and implementing educationalrequirements for a workforce to staff specializedunits for the growing population of adult patients.Appropriate specialist cardiologists may come fromtrainees in paediatric cardiology or adult cardiol-ogy. They should have the following knowledge andskills:

+ Expertise of congenital heart malformations andmanagement in infancy and childhood

+ Expertise in general medicine and non-cardiacdiagnosis in adults

+ Expertise in adult cardiology including coronaryartery disease management

+ Skill in the following procedures in adults withspecial reference to congenital heart disease:

Echocardiography (including transoesophageal)Cardiac catheterizationPacing and electrophysiologyPostoperative care

+ Understanding of the physiological changes ofpregnancy

+ Understanding of the psychosocial aspects ofadolescence

+ Experience of life style counselling for adoles-cents and adults with congenital heart disease

+ Expertise in clinical research methodology

In the specialist centre, there may be advan-tages to training individuals from both paediatric

and adult backgrounds, to facilitate smooth tran-sition of care for the younger patients as well asprovide good care for acquired problems in theolder patients.

Efforts are being made in several countries todefine training programmes for specialists in thecare of grown-ups with congenital heart disease.The following is proposed for trainees from paedi-atric cardiology:4

+ Three years in general paediatric cardiology+ 6–12 months in general medicine and adult

cardiology+ At least 12–18 months in a specialist grown-up

congenital heart disease centre+ For those aiming at an academic career in

grown-up congenital heart disease, an additionalyear in research or specialist training is required

The specific requirements for training are famili-arity with:

1 Common adult cardiac problems, such as ischae-mic heart disease, hypertension, arrhythmiasand their treatment

2 Coronary angiography (there is no need to learncoronary angioplasty)

3 Recognition and management of arrhythmias re-lated to operations for congenital heart disease

4 Management of pacemakers and practical ex-perience in their insertion. Management ofimplanted catheter defibrillators

5 Management of middle-aged and elderlypatients

6 Problems of pregnancy in relation to cardiacproblems, and effects of drugs on the patientand foetus

7 Understanding the indications and contra-indications of various methods of contraception

8 Advice about exercise in various congenitalheart abnormalities, both un-operated andoperated and what limitations, if any, apply

9 Advice concerning driving of motor vehicles10 Information about Life insurance and mortgages

for patients with various un-operated andoperated forms of congenital heart disease

11 Vocational advice

For the trainee from the adult cardiologicalbackground, training recommendations are:

+ Three years in general cardiology includinggeneral medicine

+ One year of paediatric cardiology in a centre withan active surgical and catheter interventionprogramme


+ At least 12-18 months of training in a specialistGUCH centre for those aiming at an academiccareer in grown-up congenital heart disease

+ Additional year in research or specialist trainingis required

Specific requirements for training are:

1 Foundation courses on congenital heart malfor-mations and echocardiography

2 Experience of echocardiography in infants andchildren, preoperatively and postoperatively.At least 250 echocardiographic examinationsshould be performed and an additional 25transoesophageal echocardiograms

3 Experience of cardiac catheterization and angi-ography of common congenital heart anomalies.A minimum of 100 procedures should be per-formed independently

4 Familiarity with interventional procedures incongenital heart disease with participation in atleast 25 procedures

5 Involvement in management of, and responsi-bility for at least 300 patients with congenitalheart disease. These should include patientsin the early postoperative period and thoseassessed during long-term follow-up

6 Attendance at weekly conferences of paediatriccardiologists and cardiac surgeons and partici-pation in all of the teaching activities of thedepartment

7 Knowledge of genetic implications and famili-arity with genetic counselling

8 Understanding of psychosocial problems of ado-lescence including schooling, bullying, otherbehavioural issues, such as sex and drugs

9 Vocational advice10 Problems of pregnancy in congenital heart

disease11 Contraception advice for congenital heart

disease

It is envisaged that the specialist in grown-upcongenital heart disease will share care with cardi-ologists in non-specialist centres and for this reasonit is recommended that training should be organ-ized for adult cardiologists with ‘an interest’ ingrown-ups with congenital heart disease. Thesecardiologists, in addition to their normal training(which would have included echocardiography andnon-invasive imaging), would:

+ Spend the equivalent of 6 months of full-timetraining in a specialist GUCH centre

+ Attend >20 GUCH clinics at the specialist centreover a 2 year period

+ Maintain skills by direct association with aspecialist centre and participation in CMEaccredited educational programmes

This would facilitate a flexible interactionbetween the informed adult cardiologist and thespecialist unit, to optimize both patient care andconvenience.

It is recommended that a formal certificationprocess should be established by the Europeanboard for the speciality of cardiology, indicatingthat cardiologists (either from paediatric or adultbackgrounds) have fulfilled the training require-ments in GUCH disease.

The training requirements for surgeons workingin the specialist centres also need careful consid-eration and are currently being considered by aseparate committee. The surgeon must have exten-sive experience in congenital and acquired cardio-vascular disorders, before acquiring expertise inthe surgery of grown-ups with congenital heartdisease. Re-organization and centralization of carefor paediatric cardiac surgery is in progress in manycountries, in order to ensure a minimum levelof surgical activity to develop skills and optimiseresults.8

The establishment of specialist units for grown-ups with congenital heart disease will provide en-vironments for training of all staff and researchopportunities. In this small, but growing, sub-specialty, collaboration between centres bothnationally and internationally is essential. Thiswould enable fellows to rotate between centreswithin their structured training programme.

Acceptance of clear training pathways and ac-creditation, as well as provision of the specialistenvironments for patient care and staff develop-ment is the key to a comprehensive clinical servicefor grown-ups with congenital heart disease.

4 Medical issues

4.1 Ventricular function

Accurate measurement of ventricular performanceis an important part of the preoperative assess-ment, perioperative management and laterfollow-up any patient with heart disease. There isstill no consensus as to the ideal technique, modal-ity or index to apply to the analysis of left ventricu-lar (LV) function in the biventricular circulation ofadults with acquired heart disease. The issues areamplified in those with congenital heart disease.Abnormal ventricular geometry, the effects of pre-vious surgery, extraordinary loading conditions,

1042 Guidelines

chronic hypoxaemia etc, all conspire to makemeaningful analysis difficult. Furthermore, rightventricular (RV) dysfunction may be equally ormore important in these patients, and similarlymay be affected by the supplementary circulatoryabnormalities associated with a congenital abnor-mality. Nonetheless and however flawed, theassessment of functional performance, timing ofintervention, and analysis of response is central tothe care of these patients.

While this area remains one of the frontiers of thespeciality, and many of its aspects investigational,any unit dealing with adult CHD will require sub-speciality expertise in quantitative transthoracic andtransoesophageal echo Doppler, magnetic resonanceimaging, radionuclide perfusion analysis, gradedexercise function and invasive haemodynamic assess-ment. It is likely that there will be considerable over-lap with the general adult or paediatric cardiacservice, but specific details of testing, and interpre-tation of results will require appropriate expertise ingrown-up congenital heart disease.

4.1.1 Echo-DopplerTransthoracic echo-windows for parasternal LVshort axis function and ‘four-chamber’ interro-gation for RV and LV long axis function and Dopplerstudies are rarely difficult to obtain. Many of themeasures applied to the assessment of systolic anddiastolic dysfunction in acquired heart disease areapplicable to the ‘corrected’ biventricular circu-lation, although with many caveats. For example,LV shortening fraction should be interpreted withcaution when there is significant regional incoordi-nation (which is extremely frequent) or residualleft-to-right shunting, and Doppler assessment ofLV diastolic function must take account of thepossible influence of coexisting RV dysfunction inthe biventricular circulation. There are more prob-lems when considering the abnormally connectedheart. Reduced shortening of the systemic RV maybe a physiologic adaptation to increased afterload,and the presence of an intratrial baffle may makeDoppler inflow measurements difficult to interpret.Regional incoordination is usual in the systemicventricle of the post-Fontan ‘univentricular’ circu-lation, and diastolic Doppler characteristics mustbe interpreted with an understanding of the inher-ently reduced resting preload. The potential of arelatively new modality, tissue Doppler imaging, islarge and particularly appropriate to the study ofGUCH patients. The demonstration of regionalincoordination is intrinsic to the method.

There is no substitute for sequential data. To alarge extent, the demonstration of change is more

important than an, apparently, grossly abnormalsingle measurement. This reinforces the need for arigid protocol of regular, standardized analysis,with a readily accessible databasing system. Thisapplies equally to all of the techniques described inthis section.

4.1.2 Magnetic resonance imagingVentricular volume measurements, while morerobust than other modalities, are no less immune tothe caveats regarding load etc, than shorteningindices obtained by echocardiography. Similarly,the role of such measurements in clinical decision-making remains to be demonstrated. Taking thepatient after repair of Tetralogy of Fallot as anexample, MRI is unsurpassed in its ability tomeasure RV volumes, image the RV outflow tractand proximal pulmonary arterial tree, and assessvolumetrically the degree of pulmonary regurgi-tation.9 Similarly intrinsic myocardial performancecan be assessed in a way hitherto unexplored byother techniques. Ventricular mass, thickening,vector change, and contractile geometry remainresearch investigations, with potential clinical ap-plications. As with all of the techniques discussed inthis section, sequential data will likely be the mostpowerful and the non-invasive nature of thismethod makes this particularly appropriate. It isnot too early to say that magnetic resonance imag-ing is an essential part of any tertiary or quaternaryGUCH Unit.

4.1.3 Radionuclide studiesIn the ageing population of GUCH patients, an un-derstanding of the indications for, and access tothese techniques is required. At a research level,regional abnormalities of myocardial perfusion at amicrovascular level are being increasingly recog-nized.10 As yet, the direct implications, manage-ment, and potential drug modification of thesefindings remain investigational.

4.1.4 Invasive-studiesCareful evaluation of routine haemodynamicmeasurements is implicit in all forms of complexcongenital heart disease undergoing invasive diag-nostic studies. The more detailed assessment ofventricular performance also remains fundamentalto the assessment of the natural history of pre-and postoperative disease. Simple angiographic,dimension-based, indices of ventricular perform-ance add little to similar indices measurable byechocardiography or MRI. All such measurementsare affected significantly by loading conditions.


The assessment of intrinsic myocardial perform-ance (contractility, diastolic properties etc.) re-quires more sophisticated analysis. While clearlyremaining an investigational tool, conductancecatheterization allows characterization of ven-tricular performance using the elastance model.11

Continuous pressure-volume analysis during inter-ventions, more accurately allows description of theresponse to haemodynamic interventions, thera-peutic interventions, and assessment before andafter surgery.

The adequate assessment of ventricular functionand ventriculo-vascular coupling is one of the mostimportant areas of long term follow up of GUCHpatients. Appropriate selection of technique willallow more robust analysis of natural and unnaturalhistory, as well as modification by intervention.

4.2 Arrhythmia and pacemakers

Arrhythmia is the main reason for the hospitaliz-ation of grown-ups with congenital heart diseaseand is an increasingly frequent cause of morbidityand mortality.12 Factors that predispose to arrhyth-mia include the underlying cardiac defect (e.g.atrial isomerism), haemodynamic changes as partof the natural history (e.g. chamber enlargement,myocardial fibrosis), surgical repair and scarringand residual postoperative haemodynamic abnor-malities. Arrhythmia, may itself, lead to haemo-dynamic decline, particularly in patients with veryabnormal post operative circuits who are now sur-viving into adult life. This strong electrical andmechanical connection emphasizes the need forelectrophysiological assessment and managementto be closely integrated with care of the underlyingheart defect. Correction of residual haemodynamicabnormalities may be the most important tool inthe treatment of arrhythmia.

Supraventricular arrhythmia is more frequentthan ventricular arrhythmia. Sinus node dysfunc-tion is most common after atrial surgery (e.g.Mustard/Senning, Fontan, and atrial septal defectclosure) and supraventricular tachycardia (intra-atrial re-entry tachycardia or atrial flutter) is be-coming more frequent with longer follow-up.13–15

Of note, though under emphasized in the medicalliterature, atrial flutter is a common cause of prob-lems after Tetralogy of Fallot surgery.16 The high-est incidence of ventricular arrhythmia is seen inaortic stenosis and after repair of Tetralogy ofFallot. Patients with the combination of sustainedventricular tachycardia and abnormal haemo-dynamics are at the highest risk of syncope andsudden death. QRS prolongation has been observed

with right ventricular dilatation and pulmonary re-gurgitation in Tetralogy of Fallot follow-up, and thismay be a useful marker for risk stratification.17

Pharmacological treatment of arrhythmia maybe limited by haemodynamic side effects, concomi-tant sinus node dysfunction and by desire for preg-nancy. Many standard anti-arrhythmic drugs haveproved very disappointing in grown-ups with con-genital heart disease and amiodarone is usuallythe most effective. Side effects, however, are aparticular problem in this population.18

Catheter ablation and surgical approaches havebeen increasingly applied.19 Despite sophisticatedcurrent mapping techniques, success rates remainlower than those in structurally normal hearts,largely because arrhythmia circuits are compli-cated and often multiple.20 This situation mayimprove with technological refinements. One ofthe most challenging groups has been the ‘failing’Fontan patients and a combined electrophysiological-surgical revision strategy has met with somesuccess.21 A similar approach is often needed forEbstein’s anomaly.

Pacing in grown-ups with congenital heart dis-ease is often difficult due to limited, abnormalaccess to the heart as well as the abnormal cardiacanatomy itself. The right atrial appendage is oftenabsent or distorted and active fixation electrodesare usually required.22 Furthermore, intracardialshunts and thromboembolic risk may preclude anendocardial approach. A rate responsive system isrequired and dual chamber pacing is desirable. Inthe latter, a sophisticated mode switch algorithmshould be available because of the high incidenceof supraventricular tachycardia and atrial flutter.Anti-tachycardia pacemakers have been dis-appointing but new algorithms in the current gen-eration of devices may prove more successful forboth treatment and prevention of supraventriculararrhythmias.23

Implantable cardioverter defibrillator trials inpatients with ischaemic heart disease or dilatedcardiomyopathy have shown survival benefit inselected subgroups and it is likely that these de-vices will be used with increasing frequency inpatients with congenital heart disease who areconsidered at risk of sudden death. This has majorfunding implications and emphasizes the impor-tance of identification of patients at high risk ofmalignant arrhythmia and sudden death.

The need for risk stratification, understanding ofanatomy and function, choice of drug, catheter/implantable defibrillator or surgical interventionemphasizes the importance of very close inte-gration of the electro-physiologist with the GUCH

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cardiologist and surgeon in the specialized team. Itshould be appreciated, however, that electrophysio-logical experts with the particular skills requiredfor patients with congenital heart disease arerare and both training and resources need to beincreased.

4.3 Cyanosis in the GUCH patient

Right to left shunts and the resulting hypoxaemiahave profound haematological consequences,which affect many organs.

4.3.1 Haematologic problemsThe increase in red cell mass, which accompaniescyanosis, is a compensatory response to improveoxygen transport. The white cell count is usuallynormal, and the platelet count may be normal or,more often, reduced. The increased red cell massand consequent increased viscosity increases therisk of stroke, though in adults this is only slightlyraised.24 Most patients have a compensated eryth-ropoiesis with stable haemoglobin that requires nointervention. Therapeutic phlebotomy, therefore,is usually unnecessary unless the haemoglobin is>20 g/dL and the haematocrit is >65%. At theselevels of increased red cells, patients often experi-ence symptoms of the ‘hyperviscosity syndrome’primarily consisting of headache and poor concen-tration. These symptoms may be relieved by re-moval of one unit of blood, always with an equalvolume replacement of dextrose or saline. Thera-peutic phlebotomy, however, is a ‘two-edgedsword’ since erythropoietin may stimulate the bonemarrow to produce more red cells. It is recom-mended, therefore, that therapeutic phlebotomybe performed no more than 2–3 times per year.Repeated phlebotomy depletes the iron stores andmay result in the production of iron-deficient redcells. These iron-deficient microcytes are less de-formable than iron-replete red cells and increasethe risk of stroke by increasing blood viscosity.

The treatment of iron deficiency in a patientwith destabilized erythropoiesis is challenging sinceoral iron frequently results in a rapid and dramaticincrease in red cell mass. Administration of onetablet of ferrous sulfate (or gluconate) is recom-mended with a recheck of the haemoglobin in 7–10days. The iron should be discontinued if there is adramatic increase in red cell count.

4.3.2 HaemostasisReduced platelet count and abnormal plateletfunction together with clotting factor deficienciescombine to produce a bleeding tendency in

cyanotic patients, either spontaneously or peri-operatively. Gingival bleeding, menorrhagia, andpulmonary hemorrhage (manifesting as haemopty-sis) are common. The latter is sometimes fatal.For these reasons, the use of anticoagulants andantiplatelet agents should be confined to well-defined indications, with careful monitoring of thedegree of anticoagulation. When the hematocrit is>60%, the citrate concentration in the coagulationtests needs to be adjusted; otherwise the resultsmay be inaccurate.

4.3.3 Renal functionIn chronic cyanosis, the renal glomeruli are mark-edly abnormal and are frequently hypercellular andcongested.25 This results in a reduction of theglomerular filtration rate and increased creatininelevels. Proteinuria is common. Abnormal urateclearance frequently results and this, in addition tothe increased turnover of red cells, leads to hyper-uricemia and sometimes frank gout. Hyperuricemiawithout gout is usually well tolerated, however,and does not require intervention.26

4.3.4 GallstonesBilirubin may be produced from the breakdown ofhaeme in chronic cyanosis, and calcium bilirubinategallstones are common in this adult population.27

4.3.5 Orthopaedic complicationsHypertrophic osteoarthropathy with thickened, ir-regular periosteum occurs in adults. This is some-times accompanied by aching and tendernessespecially in the long bones of the legs. Scoliosisis another important complication, which attimes may be sufficiently severe to compromisepulmonary function.

4.3.6 SkinAcne on the face and trunk frequently accompaniescyanosis. It is not just a cosmetic concern as it is apotential source of sepsis and endocarditis.

4.4 Pulmonary vascular disease (Fig. 3)

In the last 20 years, early diagnosis and improvedinfant cardiac surgery have reduced the number ofadolescents and adults with pulmonary vasculardisease. However, a considerable number of suchpatients still attend clinics for grown-ups with con-genital heart disease. Despite the fact that theirunderlying condition is irreversible and progressive,there is a considerable return in terms of morbidityand mortality from careful management.


Many patients with Eisenmenger syndrome main-tain a good quality of life into early adulthood butsubsequently the reduction of effort tolerance andincreased cyanosis is usually progressive.28 Themost important clinical events are pulmonary andcerebral complications. Haemoptysis may be due toboth pulmonary infarction or to ruptured bloodvessels. As in other cyanotic patients, Eisenmengerpatients are vulnerable to spontaneous bleeding(e.g. gums, gastric mucosal as well as heamoptysis)and have abnormal haemostasis including prolon-gation of prothrombitime, APTT and abnormalplatelet function. The decision to anticoagulatesuch patients is, therefore, difficult and controver-sial. While patients with primary pulmonary hyper-tension have demonstrated improved survival withanticoagulation, no data exists for Eisenmengerpatients and the risks may outweigh any potentialbenefits. Haemoptysis may be precipitated bystress/excitement or a chest infection and can becopious and fatal. It remains difficult to identifypatients with particular risk of pulmonary throm-boembolic complications. Similarly, cerebral com-plications (stroke or abscess) are not necessarilyassociated with higher haematocrit levels. Withgood management, patients may survive well intoadult life. In a large European collaborativestudy, the mean age of death of patients withEisenmenger syndrome and simple congenital heartdisease was 32.5 years compared to 25.8 years inthose with complex defects.28 Right ventricularfunction and age at clinical deterioration wereadditional prognostic determinants. Eisenmengerpatients are at particular risk from pregnancy,dehydration, cardiac and non-cardiac surgery,general anaesthesia, anaemia, chest infections,altitude, intravenous lines and vasodilating drugs.There is approximately a 50% maternal fatality inconjunction with pregnancy and death often occursafter delivery.24 Pregnancy is therefore contra-indicated, even in those with ‘moderate’ pul-monary hypertension. Other, seemingly innocuous,non-cardiac procedures are also risks factor fordeath.24 Careful consideration should therefore begiven to the indications for any intervention andprocedures should only be carried out in specialistcentres with experienced cardiac anaesthetists.A general policy of ‘non-intervention’, unlessabsolutely indicated, is recommended to avoiddestabilizing the ‘balanced physiology’.

A number of the treatments have shown haemo-dynamic improvement, and in some cases, outcomebenefits in primary pulmonary hypertension andmay also be applicable in grown-ups with con-genital heart disease and Eisenmenger Syndrome.

Systemic vasodilators such as calcium channelblockers should however be given with cau-tion. Prostacycline, endothelin antagonists andSildenafil may have a role, but no convincingclinical trial evidence is yet available. Long-termoxygen therapy at home for a minimum of 12–15 hper day may improve symptoms significantly butdoes not modify survival. Ultimately, lung trans-plantation needs to be considered for somepatients.29

4.5 Infective endocarditis

The ESC has its own Task Force on infective endo-carditis and this document should be referred to formore extensive information.30

Most grown-ups with congenital heart disease,but not all, have a life-long risk of infectiveendocarditis. Education of the patient and theirphysicians about the risks and importance of earlydiagnosis needs constant reinforcement. There maybe a variety of portals of entry of infection, inaddition to dental and surgical procedures. Theseinclude body piercing, acne and tattooing. Inter-ventional cardiac catheterization is infrequentlyassociated with endocarditis, but antibioticprophylaxis is usually given. Similarly, insertion ofintrauterine contraceptive devices should be cov-ered and antibiotics are generally recommended atchildbirth.

Delay in diagnosis and referral is common andantibiotics are frequently prescribed before thediagnosis of endocarditis is considered or bloodcultures are taken. Two blood cultures are ad-equate for microbiological isolation in approxi-mately 95% of patients with the usual organismswho have not received antibiotics, but more shouldbe performed to exclude clinically suspected in-fected endocarditis. It is easy to miss vegetationsin adults using transthoracic echocardiography,particularly when this is performed by operatorsinexperienced in the investigation of congenitalheart disease. The use of transoesophagealechocardiography increases the detection ofvegetations considerably.

Prompt referral to the specialist unit is usuallyindicated as haemodynamic deterioration may berapid and surgical management may be required.Infective endocarditis accounted for approximately4% of admissions to a specialized unit for grown-upswith congenital heart disease in the UK.4

Not all patients are at risk and antibiotic prophy-laxis recommendations vary. Infected endocarditisis not reported in secundum atrial septal defect,totally anomalous pulmonary venous connection

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and is extremely rare after the closure of ventricu-lar septal defect, in pulmonary valve stenosis orsmall patent ductus arteriosus. Prophylaxis policiesafter interventional catheterization proceduresinvolving device implantation vary, and are not‘evidence based’. Antibiotic treatment and overallpatient management should be undertaken in col-laboration with an infectious disease specialist.Fungal endocarditis may be particularly difficult totreat and other unusual organisms may be respon-sible. The cardiac surgeon needs to be involvedearly, as surgical replacement of infected pros-thetic material may be required. The ESC and AHAhave published specific antibiotic regimes.30,31

4.6 Imaging in adults with congenitalcardiac disease

There has been a shift form cardiac catheterizationto non-invasive imaging modalities for patients withgrown-up congenital heart disease, which is similarto that which has occurred in paediatric cardiologyover the last two decades. These include echocar-diography (transthoracic and transoesophageal)and MRI, which are essential in specialist GUCHcentres. Cardiac catheterization is now reservedfor resolution of specific anatomical or physiologi-cal questions (e.g. coronary arteries, pulmonaryvascular resistance) or for intervention (see be-low). Transthoracic echo in adults is less effectivethan in children at describing complex intracardiacand extracardiac anatomy and physiology. Trans-oesophageal echocardiography often providesdefinitive information but has the disadvantage ofusually requiring sedation or anaesthesia. MRI isincreasingly becoming the investigation of choicefor grown-ups with congenital heart disease provid-ing 3D reconstruction of anatomy, improved tempo-ral and special resolution as well as physiologicalinformation (Fig. 1). It has proved particularly use-ful for evaluation of right ventricular volume andmass, right ventricle to pulmonary artery conduit/valve function as well as for the study of pulmonaryarteries, coarctation and systemic and pulmonaryvenous anomalies.32,33 Interventional proceduressuch as balloon dilatation and radio frequency ab-lation can now be carried out using MRI/catheterfluoroscopy techniques, further extending the roleof this imaging modality. Until recently, computedtomography (CT) played only a minor role in theevaluation of congenital heart disease. Newer tech-nologies however, such as ultra fast CT and spiralCT have reduced scanning time and provide excel-lent imaging which can be seen as complementaryto echo cardiography and MRI.34 All of these imag-

ing techniques require staff with expertise in com-plex congenital heart disease, in order to avoid thehigh rates of diagnostic errors reported in routineadult laboratories.35 This has planning, training andfunding implications.

4.7 Interventional catheterization

The rapid increase in the type and applicationof interventional techniques in congenital heartdisease of childhood has been mirrored in theadult population. With the exception of calcifiedvalves, balloon dilatation of congenitally stenoticaortic, pulmonary and rheumatic mitral valves, isrelatively successful.

Many of the techniques developed in childrenwere contemporaneously, or subsequently adaptedto the treatment of grown-ups with congenitalheart disease. Relatively few techniques have be-come unequivocally established as first choice overtheir surgical counterparts. The decision to per-form an intervention should therefore undergoa similar process of peer-review, and multi-disciplinary discussion as with surgery. Randomizedstudies against the surgical alternative are almostnon-existent; registry data are limited, and localseries are potentially affected by selection andreporting bias. Nonetheless, an interventional pro-gramme is central to any GUCH unit and at least onemember of the team should be trained in interven-tional cardiology. Previous interventional experi-ence in children is probably more relevant thanprevious experience in coronary intervention, butincreasingly, specific experience in GUCH interven-tion is most desirable. The same can be said forallied medical support (anaesthesia, echocardiog-raphy etc.) and technical support within the car-diac catheter laboratory. The laboratory shouldbe equipped with biplane screening and storageequipment, should be large enough to allow simul-taneous transoesophageal echocardiography, andbe equipped with appropriate catheters, wires anddevices.

4.7.1 Techniques4.7.1.1 Balloon dilationBalloon dilation of congenitally stenotic aortic, pul-monary and rheumatic mitral valves has been verysuccessful. Aortic valvoplasty is usually performedusing a single balloon technique. As in childhood,residual aortic incompetence is the most importantcomplication. A balloon to aortic ratio of <1:1,antegrade dilatation via transeptal puncture, andadenosine cardioplegia have all been used toreduce the incidence of valve damage.36 Single,


double, or Inoue balloon dilatation of pulmonaryvalve stenosis is usually successful, but a balloon:annulus ratio of <1.1:1 should be used to avoidpulmonary arterial damage. The role of balloon dila-tation of native or postoperative aortic coarctationremains controversial.37 Dilatation of previouspatch aortoplasty carries the highest risk of aorticrupture and should be performed with surgicalstandby. Balloon dilatation of native aortic diseasecan often be achieved with excellent results, al-though failure to relieve stenosis (10–20%) aneu-rysm formation (5–10%) and restenosis (5–10%)have all been reported.38 For this reason, therehas been an increase in vogue towards balloondilatation with simultaneous stent implantation.4.7.1.2 Balloon dilatation with stent implantationThis has become the technique of first choice forbranch and distal pulmonary arterial stenosis.39

Balloon dilatation of right ventricular outflow tractobstruction, and stenotic right ventricular to pul-monary arterial conduits is less widely accepted.Stent implantation also has a role in the treatmentof resistant stenoses in surgical venous pathways40

(Mustard/Senning/Fontan). Systemic arterial stentimplantation is more controversial. The utility ofstent implantation for coarctation of the aorta,stenotic aorto pulmonary collaterals in complexpulmonary atresia, and stenotic systemic to pul-

monary shunts, while reported, remains to beproven. The need for anticoagulation after stentimplantation is not known. Most would fullyanticoagulate patients after pulmonary and sys-temic venous implantation and in those witherythrocytosis.4.7.1.3 Embolization and occlusion techniquesUnwanted, usually acquired, venous and arterialcollaterals and fistulous communications are read-ily occluded using coil embolization techniques.Similarly, the small arterial duct in adults can beclosed using detachable coils, although in largerlesions (4–16 mm), a detachable plug device isrequired (Fig. 4).

There are several devices available for closure ofsecundum atrial septal defect and patent foramenovale. Transcatheter closure of atrial septal defecthas become standard practice in most congenitalunits, for selected cases. Depending on the device,defects of up to 40 mm can be closed, providingthere is suitable atrial and septal anatomy. Many ofthe unanswered questions pertaining to surgicalclosure of ASD, are relevant to transcatheterclosure. The degree of acceptable pulmonary hy-pertension, the need for concomitant atrial an-tiarrhythmia surgery and likelihood of symptomaticresponse, which depends on age at closure are yetto be resolved. Closure of patent foramen ovale in

Fig. 1 Magnetic resonance angiography image of severe aortic coarctation in an adult. See section 4.6 — Imaging.

1048 Guidelines

patients with early onset, cryptogenic, transientischaemia attack (TIA) or cerebrovascular accident(CVA) can be achieved with extremely low morbid-ity, but with little evidence base.41 Recent trialshave suggested the importance of aspirin in deter-mining the level of risk of CVA in the presenceof patent frame ovale.42 Treatment should beindividualized and decision-making part of a multi-disciplinary process, which includes a specialistneurologist.

Many of these same ‘ASD’ devices can be used forocclusion of other unwanted intra and extracardiaccommunications. Transcatheter closure of congeni-tal ventricular septal defect is rarely indicated,but some ischaemic VSD’s may be amenable toclosure. Baffle leaks, systemic arterial, coronary,and venous fistulas communications have all beenclosed using these devices.4.7.1.4 Percutaneous valve implantationIt is now possible to insert into the right ventricularoutflow, a stent mounted bovine jugular venousvalve, via a cardiac catheter.43 Clinical implan-tation has already been performed and offers theexciting prospect of non-surgical management of

pulmonary regurgitation after repair of Tetralogyof Fallot and related conditions. Further work isrequired to develop a valve, which is suitable forthe dilated outflow tract found in many adultpatients. An implantable valve that can be used inthe systemic circulation also appears possible.

4.8 Pregnancy and GUCH

Most grown-ups with congenital heart disease cantolerate a pregnancy with proper care.44,45 Pre-pregnancy counselling and evaluation is mandatoryand should include a physical examination, assess-ment of haemodyanamic status (usually includingechocardiography) and functional capacity. Exer-cise testing may be particularly helpful in thisregard. A complete family history should also betaken to offer informed genetic counselling. Areview of medications is necessary to avoid anydrugs that may be deleterious to the foetus (e.g.ACE inhibitors). The risks to the mother of morbid-ity and mortality and, when appropriate, the im-pact of pregnancy on long-term survival should bediscussed. In addition, the risks to the foetus ofinheriting congenital heart disease should be con-sidered. Patients can be stratified into high,medium and low risk. The highest maternal risk isassociated with Eisenmenger syndrome, with amaternal mortality of ≥50% (often after delivery).46

Fig. 2 Unsightly scars from multiple cardiac procedures thatcan cause psychosocial problems. See Section 6 — PsychosocialIssues.

Fig. 3 Chest radiograph of a patient with Eisenmenger’ssyndrome. See Section 4.4 — Pulmonary Vascular Disease.


The number of mothers with complex post opera-tive circulations (such as Fontan or Mustard/Senning) remains small and risk stratification istherefore difficult.47,48 High-risk patients includethose with:

+ Significant aortic stenosis (mean gradient>40 mmHg, valve area <0.7 cm2)

+ Significant coarctation, particularly with aorto-pathy

+ Significant mitral stenosis+ Reduced systemic ventricular function+ Mechanical prosthetic valve+ Pulmonary hypertension+ Marfan’s syndrome+ Cyanotic heart disease

Appropriate medical management throughoutpregnancy for high-risk patients may include:

+ Iron and prenatal vitamins to reduce anaemia.+ Monitoring maternal and foetal well-being.+ Foetal cardiac ultrasound, which should be of-

fered to the mother to evaluate potential foetalcardiovascular abnormalities.

+ For very high-risk patients, bed rest may benecessary as pregnancy advances with delivery ata tertiary care centre.

+ For most patients, vaginal delivery is preferableto caesarean section with few exceptions, unlessthere are obstetric indications.

+ Cardiac monitoring during delivery is appro-priate in those with ‘fragile’ haemodynamicstatus.

The management of patients with mechanicalvalves is particularly challenging, particularly thosewith mitral tilting disc prostheses.49,50 Heparintreatment may be associated with thromboemboliccomplications, whether administered subcutane-ously or intravenously. The role of low molecularweight heparin is not established. Warfarin treat-ment may be safer for the mother, but can beassociated with foetal embryopathy. This risk ap-pears to be small if the Warfarin dose is <5 mg perday.51

Cyanotic heart disease also poses a significantrisk for the foetus and this is proportional to thedegree of maternal hypoxia. Low birth weightfor gestational age and prematurity are commonwith maternal cyanosis, and for those mothers withan arterial oxygen saturation #85%, only 12% ofpregnancies are successful.52 Patients who shouldbe counselled against pregnancy include thosewith:

Fig. 4 Transcatheter closure of patent arterial duct using an amplatzer device. See Section 4.7 — Interventional Catheterization.

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+ Eisenmenger syndrome (50% maternal mortality).Termination of pregnancy is safer, preferablywith cardiac anaesthesia.

+ Marfan’s syndrome with dilated aortic root.+ Severe aortic stenosis/coarctation.+ Systemic ventricular ejection fraction <35%.

Women with congenital heart disease consideredat high risk should be managed in a specialized unit,which includes an obstetrician, a cardiologist ex-pert in grown-up congenital heart disease, ananaesthetist and a paediatrician. This team shouldbe involved from early pregnancy and plan monitor-ing of the pregnancy, mode of delivery and postdelivery care. This specialized team can also pro-vide a consultation service for obstetricians andphysicians managing ‘lower’ risk women in othercentres.

4.9 Genetic counselling and contraceptionin GUCH

The risks of pregnancy vary greatly in congenitalheart disease and must be weighed against the risksof contraception. Systematic studies, however, oncontraception in women with congenital heart dis-ease are lacking.52 Patient compliance and sexualbehaviour must be considered, as they will affect

contraceptive efficacy and the incidence of compli-cations. Usually, the patient chooses her own pre-ferred method, but the physician should provideinformed advice. Often, there is no ideal methodand the least hazardous one is indicated.

Barrier methods are safe from a cardiovascularstandpoint and have a high degree of contraceptiveefficacy in compliant couples and women >35years. Low dose oestrogen combined oral contra-ceptive pills are very efficacious, but their thrombo-genic properties may make them hazardous incertain situations, such as after the Fontan oper-ation or in patients with atrial fibrillation/flutter.Combined oral contraceptives are contraindicatedin patients at risk of paradoxical embolism,unless they are also receiving anticoagulants.The oestrogen containing contraceptive pill shouldnot be used in patients with pulmonary or systemichypertension. Medroxyprogesterone injection(Depo-Provera®), subcutaneous deposition oflevonorgestrel (Norplant®) or progesterone onlypills are effective, but may cause fluid retentionand should not be used in patients with heartfailure. Depression and breakthrough bleeding mayprevent the use of progesterone pills and there is ahigher failure rate than with the combined oralcontraceptives.53

Fig. 5 International Society for Heart & Lung Transplantation results 1982–2000. Congenital Heart Disease comprised 25% of hearttransplants in 11–17 year olds group and was a risk factor for survival (OR 1.59). Results in adults with congenital heart disease havenot been reported separately. See Section 5.4 — Transplantation.


Intrauterine devices have been associated withan increased incidence of pelvic inflammatory dis-ease and it has been suggested that they carry a riskof endocarditis, especially in cyanotic women,those with artificial valves/shunts/conduits andthose with previous endocarditis. However, the re-ported number of women with intrauterine devicescomplicated by endocarditis is very low and con-cerns mainly old-fashioned intrauterine devices.The levonorgestrel-releasing intrauterine device(Levonaova®) has a low incidence of pelvic inflam-matory disease and a contraceptive efficacyequivalent with that of combined oral contracep-tives.54 It has been approved for use in womenat risk of endocarditis. If used in that setting,antibiotic prophylaxis should be given at insertionas well as at extraction. The local release of pro-gesterone reduces the bleeding problems encoun-tered with other intrauterine devices. In womenwho have not experienced pregnancy, intrauterinedevices are not a first choice.

Surgical sterilization may be considered in awoman at high risk from pregnancy, but should notbe undertaken without discussion of potentialmedical advances, which might later allow preg-nancy at an acceptable risk.

4.9.1 Recurrence risk/genetic counsellingThere is extensive information about recurrencerisk of congenital heart disease in siblings but untilrecently, few data about recurrence risk in coupleswhere the mother or father has a congenital heartdefect. The recurrence rate of congenital heartdisease in offspring ranges from to 2–50% and thereis a higher risk when the mother, rather than thefather, has cardiac disease.55–57 The highest recur-rence risks are in single gene disorders and/orchromosomal abnormalities, such as Marfan’ssyndrome, Noonan’s syndrome and Holt-Oram syn-drome. Genetic counselling should be available inthe specialized units for grown-ups with congenitalheart disease and specific genetic testing is likelyto evolve as understanding of the genetic basis ofcongenital heart disease improves. The specialistunit should also offer foetal echocardiographyat 16–18 weeks gestation and chorionic villoussampling or amniocentesis may also be indicated inselected cases. The potential for drugs to affect thefoetus should always be considered. In particular,angiotensin converting enzyme inhibitors and an-giotensin II receptor blockers should not be usedin pregnancy and withdrawal of amiodarone andWarfarin should be considered. Protocols for stop-ping Warfarin and managing anticoagulation arerequired.

4.10 Comorbidity and syndromes

Congenital and acquired comorbidity is common ingrown-ups with congenital heart disease and has animportant effect on outcome and treatment. Cog-nitive and intellectual impairment may be a featureof co-existing heritable or chromosomal syn-dromes, which are present in 15–20% of congenitalheart disease. Such patients are surviving into adultlife with increasing frequency due to a more activeapproach to their treatment in childhood. Alterna-tively, late problems may result from neurologicalcomplications in the perinatal and perioperativeperiod and are likely to pose a serious burden on thefamilies and to produce an important demand onmedical and social institutions. This has not yetbeen addressed or funded in most European coun-tries. Knowledge of the individual characteristicsof such syndromes assists cardiac diagnosis andprovides a clue to the presence of extra cardiacproblems.

Skeletal deformities are common in cyanoticstates, especially when a lateral thoracotomy in-cision was performed in early life. There may alsobe visible chest or breast deformities, which havepsychosocial impact (Fig. 2).

Chronic cyanosis is associated with pulmonary,metabolic and haematological problems (see sec-tion 4.3) and sequelae from previous operationsor catheterization may include arterial or venousperipheral occlusions and scars on the chest.

Management of comorbidity in grown-ups withcongenital heart disease deserves a thorough un-derstanding of pathophysiology by the cardiologyteam as well as close communication with thevarious non-cardiac specialists involved (Table 1).

Acquired heart disease will occur with increasingfrequency as the population with congenital heartdisease ages. Coronary artery disease and/or sys-temic hypertension can affect the haemodynamicsof the congenital heart disease, requiring treat-ment of both the congenital and acquired dis-orders. This emphasizes the importance of theclose interaction between the specialist unit forgrown-ups with congenital heart disease and mem-bers of the general adult cardiology department.

4.11 Emergencies – adults with congenitalcardiac disease

Acute cardiac and non-cardiac emergencies occurin grown-ups with congenital heart disease andtheir optimal management may be above the abilityof emergency room, general medical or adult car-diology staff.58 The most frequent are arrhythmia,

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infections, heart failure, cerebral ischaemia or aor-tic root problems. It may be possible to provideappropriate initial treatment in the local hospital(usually after consultation with the specialist cen-tre) but patients with more severe complications ormore complex congenital heart disease will usuallyrequire transfer to the specialist centre. Geo-graphical planning of the hierarchical care deliverymodel is thus very important. At present, thelimited available expertise has developed in anunplanned fashion and long distances betweenspecialist centres may delay diagnosis and treat-ment and actually discourage appropriate transfer.

5 Surgical issues

5.1 Cardiac surgery

Adolescents and adults with congenital heart dis-ease who require surgery fall into three categories:(1) those who have not previously undergone oper-ation, (2) those who have had palliative surgery and(3) those who have had reparative surgery. In eachcategory, there are several consideration, whichmake surgery, in this population, different fromother types of cardiac surgery (either adult surgeryfor acquired heart disease or paediatric surgery forcongenital defects). There are strong arguments forconcentrating surgical management of grown-upswith congenital heart disease into specialist unitsfor both care provision and training. Surgery can beperformed safely only by teams who have extensiveexperience in the management of congenital heartdefects in infants and children as well as knowledgeof the principles of conventional adult cardiacsurgery.

5.1.1 General planning of the operationThe first operation may be required in patients withsimple defects (e.g. atrial septal defect) but moreoften is needed in patients with complex malfor-

mations (e.g. pulmonary atresia with ventricularseptal defect). In patients who have previouslyundergone palliative or reparative surgery, the con-sequences of previous operations may add to thecomplexity of the management of the primary de-fect. Thorough cardiac evaluation is mandatory inall cases. Surgical planning requires knowledge ofthe basic congenital malformation, of the previoussurgical procedures and of the potential residual orrecurrent lesions after these operations. The surgi-cal team needs to be intimately involved in thereview of the diagnostic information, the decision-making and planning of the patients overall man-agement. It is mandatory that the previous surgicalreports be available.

In adult cardiac surgery for acquired heart dis-ease, the risk and benefits of most surgical pro-cedures are well established. In contrast, everygrown up with congenital heart disease posesspecific problems and the risk/benefit ratio of anyproposed surgical procedure is often difficult toassess. It may therefore be difficult to communi-cate the necessary information about risks andbenefits to the patient.

5.1.2 Specific surgical challengesSome surgical problems apply to all first operationsor re-operations in adults with congenital heartdisease.

5.1.2.1 Preservation of myocardial functionIn most patients, preoperative ventricular functionis abnormal as a result of ventricular morphology,ventricular hypertrophy and long-standing pressureor volume overload. Myocardial fibrosis and ischae-mia as well as the sequelae of previous operationsmay also influence ventricular function and requiremeticulous preoperative assessment.

To achieve optimal intraoperative preservationof myocardial function, some recommendationscan be made:

Table 1 Syndromes associated with mental deficits

Syndrome General features Cardiac defect

Alcohol Syndrome Facial and growth anomaly, mentalretardation

ASD, VSD (30%)

Down (trisomy 21) Mental retardation, typical facies,lymphedema

AVSD (VSD, aortic valve anomaly) in 40%

Noonan Turner like phenotype, normalchromosomes, mental retardation

Coarctation, HCM, ASD, PS

Turner Chromosome XO, skeletal and mentaldeficits

Coarctation in 35%, ±bicuspid aortic valve

Williams Beuren Facial dysplasia, hypervitamnosis D,hypocalcaemia, mental retardation

Supravalvar aortic stenosis, sometimes with multiplepulmonary artery stenoses


1 Aortic cross clamping should be avoided wherepossible. Most operations involving the rightheart (e.g. replacement of right ventricle topulmonary arteryvalved conduit or extracardiacFontan procedure) can be carried out undernormothermic cardiopulmonary bypass withmono or bicaval cannulation and a beating heart.

2 When aortic cross clamping is necessary, cross-clamp time should be kept as short as possibleand particular attention should be paid to cardio-plegic myocardial preservation.

Strategies for myocardial protection and cardio-plegia should include:

a Use of the appropriate cardioplegic solution.b Induction of myocardial hypothermia using a cold

blood cardioplegic solution.c Maintenance of diastolic arrest and hypothermia

using multi-dose blood cardioplegia. This is par-ticularly important in cyanotic adults, in whomnon-coronary collateral vessels to the heart mayresult in wash out of cardioplegia and myocardialre-warming.

d Enhanced warm blood reperfusion administeredprior to aortic unclamping under careful pressuremonitoring.

e Adequate venting of the heart to avoid ventricu-lar distention, wall tension increase and subse-quent inadequate delivery of cardioplegicsolution.

5.1.3 Blood salvage techniquesAutologous transfusion should be encouraged andbe used wherever possible. In cyanotic patients,preoperative phlebotomy may be indicated to im-prove haemostatic status and the blood should bekept for autologous transfusion. Patients undergo-ing re-operation after initial reparative surgery areless likely to be cyanotic but they can usuallypredonate an adequate amount of blood and freshfrozen plasma. Iron repletion is indicated in mostcases managed in this way. Erythropoietin adminis-tration may allow rapid and timely increases inhaematocrit. Patients with congenital heart dis-ease are prone to intraoperative and postoperativebleeding because of relatively long suture lines, anincrease in tissue vascularity, intrinsic haemostaticdefects and prolonged periods of cardiopulmonarybypass. Replacement of consumed or inactivatedclotting factors, particularly platelets, is an impor-tant requirement. Aprotinin has proved effectivein reducing intraoperative bleeding, particularly inpatients undergoing re-operation. The routine in-traoperative use of a cell saver system is important.Ultrafiltration (either conventional during cardio-

pulmonary bypass or modified after cessationof bypass) has been shown to result in a signifi-cant reduction in the need for postoperativeblood transfusion as well as to improve myo-cardial and lung function and extraction of solubleinflammatory mediators.

5.1.4 Redo sternotomy incisionReopening a sternal incision remains a crucial step,particularly when an enlarged right ventricle or anextracardiac conduit may be apposed or adherent tothe back of the sternum, or when the aorta liesanteriorly (transposition of the great arteries). Ifparticular difficulty is anticipated, cannulation ofthe femoral vessels, institution of bypass and decom-pression of the heart before reopening the sternumare prudent precautions. When re-operation is an-ticipated, at previous operation, implantation of aretrosternal prosthetic membrane greatly facilitatesreopening of the sternum, although this sometimesmakes more difficult the subsequent identificationof the mediastinal and cardiac structures.

5.1.5 Pulmonary vascular bed abnormalitiesIn most patients undergoing surgery for acquiredheart disease, the pulmonary vascular bed is nor-mal. This is not the case in many adults with con-genital heart disease and management of elevatedpulmonary vascular resistance is particularly impor-tant in the early post operative period. Severedistortion of the pulmonary arteries may be presentin previously operated patients (pulmonary arterybanding, aortopulmonary shunt or previous surgeryinvolving the pulmonary arteries). Preoperativedilatation and stent implantation is sometimesindicated. Alternatively, these lesions may berepaired adequately using pericardial patches.Pulmonary arteriovenous fistulae may develop inpatients after a long-standing classic Glenn shunt.Rarely, large fistulae can be occluded with devicessuch as coils. More often, however, there are mul-tiple small fistulae, which are not amenable toocclusion. Such lesions may regress after reparativesurgery, but may be the source of severe, oftenlethal postoperative complications. In all patients,the pulmonary vascular bed (anatomy and physiol-ogy) must be evaluated very carefully, as abnor-malities may preclude further surgery or be animportant cause of failure after surgery in grown-ups with congenital heart disease.

5.1.6 Aortopulmonary collateral circulationIn cyanotic patients, aortopulmonary collateralvessels may complicate peri-operative and post-operative management. Important collateral circu-lation, if not curtailed, results in excessive return

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to the left atrium. It obscures visualization of theoperative field, washes out cardioplegia, compro-mises systemic perfusion and causes volume over-load of the left side of the heart during thecritical, post-operative period. The size, locationand end-parenchymal distribution of the collateralvessels must therefore be established precisely.Adequate management may include (1) pre-operative occlusion (by interventional catheteriza-tion) or surgical ligation at the beginning ofoperation, (2) surgical unifocalization of large col-lateral vessels which are the sole sources of bloodflow to significant portions of the lungs, (3) deephypothermic low flow cardiopulmonary bypassto prevent the deleterious effects of multiplesmall collateral vessels not amenable to surgicalocclusion.

5.2 Anaesthesia and post operative care

Grown-ups with congenital heart disease requiringcardiac and non-cardiac surgery present a spectrumof severity ranging from well patients with minorproblems to those with extreme deviations fromnormal cardiovascular physiology. There is how-ever, little evidence-based information to enablechoice of anaesthetic technique in these patients.Anaesthesia demands particular attention to endo-carditis prophylaxis, avoidance of air emboli, ap-propriate placement of vascular catheters andregulation of intra-vascular volume and systemic/pulmonary flow. The most important aspect ofperi-operative care is the involvement of a clinicalteam with detailed understanding of the patient’scardiac defect, functional status and anticipatedperi-operative stresses.

5.2.1 PhysiologyCardiovascular impairment and increased anaes-thetic risk may be due to hypoxaemia, pulmonaryvascular disease, cardiac failure or arrhythmia.Polycythaemia is the major adaptive response tohypoxaemia. Blood viscosity increases exponen-tially with haematocrit and may be further in-creased with iron deficiency.59 Polythaemicpatients must not become dehydrated and shouldreceive intravenous fluids from the night beforesurgery. In patients with decreased pulmonaryblood flow, hypoxaemia should be minimized byensuring adequate hydration, maintaining systemicarterial pressure, minimizing transient elevationin pulmonary vascular resistance and avoiding in-creases in oxygen consumption. Elevated pulmon-ary blood flow, on the other hand, may increase

excessively cardiac work or decrease systemicperfusion. The strategy in this situation is to main-tain ventricular performance and optimise pulmo-nary to systemic flow ratio. In the presence of asystemic to pulmonary shunt (e.g. Blalock-Taussig)pulmonary flow will vary with the pressure gradi-ent. Increases in pulmonary blood flow decreasepulmonary compliance and increase airway resist-ance and work of breathing. Significantly increasedpulmonary blood flow leads to airway obstructionand non-compliant lungs. Reactive pulmonary vas-culature can be treated with anaesthetic drugs,positive pressure hyperventilation, oxygen and pul-monary vasodilators. If pulmonary blood flow isreduced, it is important to prevent further reduc-tions. Airway dead space volume is increased withpositive pressure ventilation, increased alveolarpressure or decreased left atrial or pulmonaryartery pressures.

5.2.2 AssessmentPre-operative assessment should define baselineproblems and identify patients at increased risk.The cardiac anaesthetist should be involved inpre-operative conferences and overall planning ofthe management strategy. Access to all previousoperation notes is invaluable. Clinical evaluationshould be supplemented by laboratory data,ECG, chest X-ray, echocardiography and cath-eterization information. Lung function testsshould be performed if the patient has scoliosis.Previous surgery may have resulted in recurrentlaryngeal and phrenic nerve injuries or Horner’ssyndrome.

5.2.3 Anaesthetic managementSedative pre-medication is popular. Oxygen con-sumption is reduced although caution must beexercised in the presence of hypoxaemia. Selec-tion of induction agent is an individual choice.While there is a well-known influence of cardiacshunts on rapidity of inhalation induction, theclinical significance is rarely large. Vasodilatationis common during most induction techniques andwill increase right to left shunting. However, theeffect of this on arterial saturation will be par-tially offset by the reduction in total oxygenconsumption and consequent increased mixedvenous oxygen saturation. In general, the choiceof drugs is less important than appropriatehaemodynamic goals. Narcotic based anaesthesiais quite suitable in the presence of ventriculardysfunction. Ketamine may depress cardiacfunction if maximal sympathetic stimulation ispresent.


5.2.4 MonitoringThe use of invasive monitoring depends on themagnitude of surgery and the underlying cardiovas-cular pathophysiology. Placement of a pulmonaryartery catheter can be technically difficult onaccount of anatomical abnormalities and might ac-tually be dangerous in the presence of reactivepulmonary vasculature. Transoesophageal echocar-diography is useful for following ventricular per-formance, valve function and blood flow. Somepractical considerations for monitoring are impor-tant. End tidal CO2 will under estimate paC02 in thepresence of right to left shunting or common mix-ing. Previous systemic to pulmonary shunt place-ment requires blood pressure monitoring on thecontra-lateral side. Accuracy of pulse oximeters isnot guaranteed below levels of 80%. Vascular ac-cess may be problematic due to venous thrombosisor interrupted vena cava with azygos continuation.Central venous necklines pose a significant throm-boembolic risk in the Fontan circulation and should

be removed as soon as feasible. Femoral veins canbe used for central drug administration.

5.2.5 Post anaesthesia carePost operative care is usually provided in a highdependency or intensive care environment andstandard management principles apply to mostpatients. Experience in the management of pul-monary vascular resistance, shunt lesions, ven-tricular outflow tract obstruction and rightventricular dysfunction is however particularlyimportant. There is a trend in modern intensivecare units to emphasise fluid administration andsystemic oxygen delivery in high-risk surgicalpatients. This approach may not be well-toleratedby GUCH patients with poor ventricular function.

Cardiopulmonary interaction is important. Con-trol of arterial blood gases (particularly PaCO2) isfundamental to regulating pulmonary blood flow.While low airway pressures and weaning from ven-tilation are beneficial to trans-pulmonary flow, it is

Table 2 Energy expenditure, dynamic and static components in sports

Examples of sports Energy expenditure in kcal/h(for 70 kg body weight)

statica dynamica

Light (<3 Met)Slow walking 200 − +Hiking 400 − +Golf 350 − −Cricket 350 − +Curling 300 − −Brisk walking 350 − +Gymnastics 400 ++ −Moderate (3–6 Met)Tour cycling 400 + ++Swimming 500 + ++Walking uphill with load 500 − +Horseback riding 200/450/550 + −walk/trot/gallop + −Diving 450 ++ +Alpine skiing 350 − +Volleyball 350 − +Tennis double 400Social dancingHard (>6 Met)Tennis single 600 − ++Jogging 700 + +Cross country skiing 700 − ++(classic) 800 + ++Cross country skiing 800 ++ ++(skating) 800 ++ ++Race cycling 500 + +Rowing 600 + ++Boxing 700 ++ ++Team handball, footballRace Skating

a− low, + moderate, ++ high.

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important to remember that the duration of theinspiratory phase during positive pressure venti-lation has more influence than the absolute level ofpeak inspiratory pressure. Indeed shortening theinspiratory time (with consequent increase in peakinspiratory pressure) is usually the best strategy tomaximise pulmonary flow. The alveolar ventilationCO2 response curve is normal. Hence adequateanalgesia is important and appropriate. This isparticularly important in the presence of labilepulmonary artery pressures. The hyperbolic rela-tionship between SaO2 and Qp:Qs is often not un-derstood by general intensive care staff. As aresult, the significance of inappropriately highsaturations in a complete mixing circulation maynot be appreciated or raise concern.

5.3 Non-cardiac surgery

The risks of noncardiac surgery depend on thenature of the underlying cardiovascular abnor-mality, the extent of the surgical procedure, andwhether or not it is an elective procedure or anurgent one. Preoperative planning must includeconsultation with a specialist GUCH centre.60 Theprecise haemodynamic and anatomic abnormalitiesneed to be understood, and the evaluation usuallyincludes a Doppler echo assessment of ventricularfunction and pulmonary artery pressure. The risksof the operation must be explained to the surgeonincluding the risks of haemodynamic instability,haemorrhage, hypotension, and hypovolaemia,along with the risk of endocarditis.61 Additionalcoexistent problems, which frequently accompanycongenital heart disease, such as renal dysfunction,must also be evaluated in addition to those ac-quired disorders to which adult patients arevulnerable (systemic hypertension, ischaemicheart disease, arrhythmia (both ventricular andsupraventricular), peripheral varicosities, etc.).Planning the procedure carefully is of the utmostimportance. Urgent noncardiac operations carry ahigher risk, and complications may be more fre-quent in patients undergoing respiratory or nervoussystem procedures. Intraoperative monitoring maybe helpful in detecting early haemodynamicchanges so that appropriate treatment can be initi-ated promptly. Continuous intra-arterial monitor-ing enables sudden changes in intravascular volumeand haemodynamics to be detected, and facilitatesperiodic arterial blood gas determinations. The de-cision of whether or not to utilize a central venousline or pulmonary artery pressure recording (with orwithout oximetry) must be determined in eachindividual case and the risk-to-benefit ratio as-sessed. The latter may be associated with an

increased risk of ventricular arrhythmia andparadoxical embolism in patients with pulmonaryhypertension and right-to-left shunt.

5.3.1 Unoperated congenital heart diseasePatients with significant obstructive lesions (aorticstenosis, coarctation, and pulmonary stenosis) arevulnerable to intraoperative hypotension especiallyif ventricular function is depressed. Rapid fluidinfusion may precipitate pulmonary edema. Elec-tive noncardiac surgery may be performed moresafely after repair or replacement of the obstruc-tive valve or arterial lesion. A patient with ven-tricular dysfunction from any cause is morevulnerable to perioperative complications (e.g.,congenitally corrected transposition of the greatarteries when the systemic ventricle is the morpho-logic right ventricle).

5.3.2 Operated congenital heart diseasePatients with repaired congenital heart disease arevulnerable to arrhythmia and haemodynamic de-terioration, particularly when they have impairedventricular function, which may be exacerbated bythe loss of atrial transport. A multidisciplinary ap-proach to their perioperative management will helpto minimize the complications.

5.3.3 Cyanotic heart diseasePatients with cyanotic congenital heart disease aremost at risk from noncardiac surgery, especiallyif they to have pulmonary hypertension.60 Theincreased risk of haemorrhage secondary to theinherent haemostatic abnormalities can be tempo-rarily reduced by preoperative phlebotomy, if thehaematocrit is >65%. One unit accompanied by iso-volumic fluid replacement may be performed, andthe blood can be saved for potential autologoustransfusion. For cyanotic patients a fall in systemicvascular resistance can increase the right-to-leftshunt and increase hypoxia. This is not toleratedwell and can potentiate cardiovascular collapse.Lengthy operations associated with haemodynamicinstability necessitating large volume fluid replace-ments are also associated with increased periop-erative mortality. Spinal anaesthesia, similarly, canresult in a fall in systemic vascular resistance andreduced venous return. This method of anaesthesiais thus best avoided in cyanotic patients. For anypatient with a right-to-left shunt, there is an in-creased risk of a paradoxical embolus and air filtersshould be placed on all intravenous lines. Bacterialendocarditis prophylaxis should be given whenappropriate.60,61


5.4 Transplantation (Fig. 5)

Transplantation is the final palliation for manygrown-up patients with congenital heart diseaseand must be considered when the short-term prog-nosis is reduced or the quality of life unacceptable.Transplantation may involve either heart, heart-lung, single or double lung with intracardiac repair.

The most common congenital lesions that ulti-mately may require transplantation include failedFontan, Mustard or Senning procedures, congeni-tally corrected transposition, complex pulmonaryatresia and Eisenmenger complex. In addition, anincreasing number of recipients will include thoserequiring retransplantation after primary trans-plantation for congenital lesions in childhood.

Risk stratification scores are available for termi-nal congestive heart failure62 but may not applyto grown-ups with congenital heart disease. Thismakes decisions regarding timing of transplantationdifficult.

In addition to standard pretransplant work up,attention needs to be directed towards specificissues in grown-ups with congenital heart disease.Sensitization from previous transfusions may in-crease the early risk of rejection and graft failure.Treatment to reduce the HLA antibody level may berequired before placement on the waiting list. As-sessment of pulmonary vascular resistance is diffi-cult in patients with low cardiac output, residuallesions, shunts or collaterals. Detailed planning ofthe surgical approach is crucial in many complexlesions and all anatomic details, including the sys-temic and pulmonary venous return need to bedefined. MRI is particularly informative. Non-adherence to post transplant medications is amajor problem in young adults and pre-existingpsychosocial issues may require careful evaluationbefore listing. Other risk factors that are specific tothe failing ‘Fontan’ patient include protein losingenteropathy and pulmonary arteriovenous fistulae.An increasing number of patients with terminalcardiopulmonary lesions have underlying chromo-somal anomalies, which may further complicatedecision-making.

Surgical issues in previously operated patientsinclude difficulties with cannulation, dissection,abnormal anatomy, need for an additional conduitfor reconstruction, bleeding and prediction of thetime needed to prepare the recipient. This oftenleads to longer ischaemia and bypass times, whichmay jeopardize graft function and survival.

The current 1-, 5- and 10-year survival aftercardiac transplantation in non-GUCH patients is 80,70 and 55%.29 For heart-lung transplantation the

survival is only 65, 45 and 30%, respectively.29 Theoutcome after heart transplantation is significantlylower in many grown-ups with congenital heartdisease, including Fontan patients, primarily due toa higher early attrition.63–66 Late attrition aftertransplantation has not improved significantly dur-ing the last 10–20 years despite introduction of newimmunosuppressants. Chronic rejection (graftcoronary vasculopathy in hearts and obliterativebronchiolitis in lungs) and malignancy remain themain concerns. Immunologic progress and newdrugs will hopefully improve this in the future.

The increasing donor shortage and inferior re-sults may influence donor organ allocation togrown-ups with congenital heart disease patients.The increasing number of patients surviving withpalliated lesions and those with previous trans-plants will worsen the donor situation dramaticallyand many patients will never get to transplantationuntil viable alternatives such as reliable long-termmechanical support systems or xenotransplantationbecome available. This problem is of particu-lar concern for grown-ups with congenital heartdisease requiring heart-lung grafts.

6 Psychosocial issues (Fig. 2)

In addition to provision of care for complex medicaland surgical problems, the specialist service forgrown-ups with congenital heart disease must pro-vide support for the many psychosocial problems inthis population.67 These include a high level ofanxiety about the underlying heart condition andprognosis, difficulties with social interaction aswell as specific issues regarding employment, insur-ance and physical activity.68 Staff with appropriateexpertise should form part of the specialist teamand are frequently called upon to act as advocatesfor the patients who may face unfair discrimi-nation. Further studies are required to investigatethe relationship between the underlying disability,level and type of follow up care, emotional statusand affect on psychosocial health and perform-ance. These should examine well-characterizedpopulations of patients, use validated question-naires, grading of physiological performance andappropriate control groups for comparison. Cur-rently, validated congenital heart disease specificmeasures are not available. This information willassist in the training of professionals and enable theprovision of counselling services, specific to theneeds of adolescents and adults with congenitalheart disease.

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6.1 Intellectual development/education

Intellectual development may be influenced bygenotype, the presence of syndromes, as well asthe disturbed haemodynamics of the cardiac defectand its treatment. Many patients with syndromesinvolving learning disabilities of varying degree nowsurvive into adult life, including William’s syn-drome, Down’s syndrome and 22q11 deletion. Theyhave specific educational needs. Cognitive functionmay be affected by early neurological compli-cations of low cardiac output, acidosis, and hypoxiaas well as by the consequences of cardiac surgery,anaesthesia, hypothermia, circulatory arrest andcardiopulmonary bypass. Intellect may be furtherinfluenced by chronic cardiac failure, arrhythmiaand/or cyanosis as well as absence from school.

Studies of intellectual outcome in young adultswith relatively simple congenital heart defectshave been very encouraging. Indeed, level of edu-cational attachment may be superior to that in thegeneral population, reflecting the high level ofmotivation of grown-ups with congenital heart dis-ease and the support of their families and healthcare providers.69 Data in patients with complexdefects are more limited. Some studies have shownbelow average I.Q. scores in cyanotic congenitalheart disease, including Tetralogy of Fallot andtransposition of the great arteries.70 Individual fac-tors such as duration of chronic hypoxia and el-evated haematocrit may have a limited impact but,in general, combinations of adverse factors appearto have long-term detrimental effects. Up to 10% ofchildren with congenital heart disease will have acerebovascular infarction, most within the first 2days of life.71 Early correction of congenital heartdisease, as is currently undertaken, may reducethese neurological complications, but may in itself,have neurocognitive effects which will becomemanifest in later life.

6.2 Employment

Ability to obtain and maintain employment willdepend on intellectual and physical capacity, moti-vation, and interaction with peers as well as poten-tial discrimination by society. Several reports ofemployment status of grown-ups with congenitalheart disease have shown that unemployment ismore common in patients with complex lesions.72

Approximately 10% are considered totally disabled.The economic consequences of unemployment varyin different countries, because of the marked dif-ferences in the levels of social and welfare pro-grammes across Europe. Nevertheless, even underthe most generous systems, unemployment has

major adverse effects including lowered self-esteem and social contact, especially in thispotentially vulnerable population.

Appropriate employment counselling is part ofthe responsibility of the medical profession, as-sisted by other members of the specialist team,including nurse counsellors. Advice needs to berealistic and based on physical and intellectualcapacity as well as on specific issues, such asarrhythmia, which may preclude certain types ofjob (e.g. car driving). There are often specificexclusions for jobs, particularly in the publicsector, which varying between countries, (e.g.armed forces and police). The ability of the patientto undertake the demands of different occupationsmay need to be tested in formal programmes. Otherservices such as vocational and physical rehabili-tation as well as job training may broaden thepatient’s range of employment options.

Studies have shown discrimination against jobapplicants with congenital heart disease. These arebased on concerns about performance, absentee-ism, premature retirement and medical insurance.Legislation against such behaviour by potentialemployers varies between countries, includingaffirmative action to employ disabled persons. Fur-thermore, prohibition of discrimination on the basisof higher insurance costs is often included and iscrucial in countries with an insurance-based systemfor medical care. The practitioner can greatly in-crease patients’ employment chances by interact-ing directly with the prospective employer, asobjective outcome data for many conditions arestill not available.

6.3 Insurance

Life insurance and health insurance availabilityvary greatly both within and between countries.Despite attempts to standardize and improve thesituation, patients may still need to shop around toobtain the best deal. Physicians and organizedpatient groups, such as the Grown-Up CongenitalHeart Association in the UK, provide extremelyuseful advice and assistance in directing patients to‘friendly’ companies.

Life insurance remains an important componentof financial planning, not least in relation to mort-gage and house purchase. Life insurance may bedenied, be made available at normal rates or have aheavily loaded additional premium.73 A study in1993 in the UK evaluated both life and healthinsurance available to adults with congenital heartdisease.74 The findings revealed a surprising dis-cordance between insurance policy and medical


outcome data. For example, patients with repairedaortic coarctation were insurable at normal rates,whilst patients were often denied insurance aftersuccessful repair of ventricular septal defect.Health insurance was usually available, but almostalways excluded benefit for the underlying cardiaccondition. This can have a major impact on deliveryof optimal medical care to grown-ups with congeni-tal heart disease in an insurance-based medicalsystem. In an earlier German survey among a cohortof GUCH patients, more than 30% were refused lifeinsurance.75 Further studies are in progress in theUK and France to obtain up to date information.Insurance recommendations from cardiac societiesexist in Italy and Switzerland, but not in the otherEuropean countries surveyed. This is clearly anunsatisfactory situation and could be improved byestablishment of formal practice guidelines andoutcome analysis, which could be shared with in-surers. Strategies for insurance of patients withmore complex lesions and worse outcomes need tobe developed if medical care is not to be compro-mized by financial considerations.

6.4 Physical activity/sport

Participation in sports and regular physical exercisehave well documented beneficial effects on fitness,psychological well-being, confidence and social in-teraction as well as on the later risk of acquiredcardiac disease. Recommendations on exercise ingrown-ups with congenital heart disease need to bebased on the ability of the patient as well as on theimpact of physical training on cardiac haemo-dynamics76,77 (e.g. ventricular remodelling, myo-cardial ischaemia). Counselling should include anappreciation of the type of energy expenditureinvolved in different sports and teaching of amethod to enable the patient to limit his or heractivities (Table 2). These include the Borg scale ofperceived effort, a target heart rate range (60–80%of maximum heart rate achieved during testingwithout symptoms or haemodynamic deterioration)and a simple breathing rule (activity can be carriedout safely as long as breathing still permits com-fortable speech). Impact sport should be avoided inpatients with Marfan’s syndrome or other aorticanomalies, those on oral anticoagulants or thosewith pacemakers. Formal testing, assessing the im-pact of exercise levels relevant to the patients’expectations during normal day life, should be un-dertaken and protocols derived from conventionaladult exercise testing programmes need to beadapted.78 Doctors should use these results indiscussion with patients. In general, medicalpractitioners are conservative and are often

unnecessarily proscriptive in their recommen-dations; this may have important adverse effects onquality of life.

Exercise may have acute, chronic and potentiallyharmful haemodynamic effects in patients withcongenital heart disease. These include fluid deple-tion, blood pressure rise or fall, tachycardia and/orarrhythmia as well as long-term effects on ventricu-lar hypertrophy and function. Of most concern isthe risk of sudden death during or after exercise.Most cases of sudden death during physical activityin the young are due to a previously unrecognizedcardiac disorder and sudden death in patients withknown congenital heart disease is very rare (1 in10 000 patients in a recent survey in nine specialistcentres for grown-ups with congenital heart dis-ease). Potentially lethal situations may occur witharrhythmia and haemodynamically vulnerable cir-culations (e.g. preload jeopardized circuits such asafter Mustard/Senning or Fontan operation or withheart failure. Advice to perform social exercise to alevel of comfort, but not to attempt competitivesports is applicable in most situations.79,80

6.5 Quality of life

Few studies of quality of life have been performedusing validated measures in large populations ofadolescent or adults with congenital heart disease.Most have limited relevance to modern manage-ment.81 Most patients, when questioned, will saythat they are ‘asymptomatic’. Whilst many are ableto enjoy the full range of normal life activities,patient symptom reporting should be interpretedwith caution as they may always have been limitedand thus ‘do not know any better’.82 Alternatively,they may be reluctant to admit limitations and todiscuss problems. Allocation of sufficient time toobtain a good history (often by the nurse counsel-lor) is essential and may need to be supplementedby objective testing.

Psychosocial adjustment to adult life depends,not only on the type and severity of the congenitalheart defect, but also on the attitude and behav-iour of family, friends and the GUCH team. Sensi-tive handling and education can be enormouslyvaluable and the advocacy role of the GUCH teamcannot be over emphasized.

6.6 Patient organizations

In many European countries, patient organizationshave been established and are active in spreadingmedical information, educating patients and secur-ing patient rights. These organizations, due to their

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contact with media, may often have a greaterpolitical impact than normal health channels.Patient specific websites are invaluable foreducation as well as helping to direct patientsto appropriate specialist centres. Many patient

organizations have been effective in raising moneyfor medical research and new expensive treatmentapproaches. Regular contact between specialistunits for grown-ups with congenital heart diseaseand these organizations is therefore important.

7 Specific lesionsThis section summarises the current management strategy for the commonest lesions seen in grown-upswith congenital heart disease. Many recommendations are based at clinical experiences rather thanevidence trial randomized clinical trials. We have therefore chosen not to use categories of strength ofendorsement as in other ESC Task Forces.

Atrial septal defect

Criteria Comments

1. Introduction and background + common defect which may be diagnosed first in adult life

2. Survival/adult life + small defects-excellent prognosis + large defects — reducedsurvival, depending on age at treatment

3. Haemodynamic issues + PHT + RV dilation/failure + potential for paradoxical embolism+ reduced LV compliance

4. Arrhythmia/pacing + atrial arrhythmia (atrial fibrillation and flutter) + sick sinussyndrome + pacing rarely required

5. Investigations ECG + baseline — if clinically indicated (arrhythmias)

Chest X-ray + baseline — otherwise little value

ECHO/TOE + baseline-location, size, RV size, PA pressure,Qp:Qs, associated lesions + TOE usuallyperformed in older patients and at device closure

Catheterization + device closure + PVR assessment

MRI + rarely helpful

Holter + if symptomatic arrhythmia

Exercisefunction

+ baseline — little value

6. Indications for intervention + large defects (>10 mm) unless pulmonary vascular disease(PVR>8 Um2, L-R shunt <1.5, no response to pulmonary vasodilators)+ paradoxical embolism

7. Interventional options + surgery or device closure (stretched diameter <38 mm)

8. Post treatment outcome + low risk procedure unless PVD + late intervention less successful

9. Endocarditis + very rare + prophylaxis not indicated

10. Pregnancy/contraception/recurrence/fetal

+ no contra-indications unless PVD + no restrictions forcontraception + consider fetal echocardiography

11. Recurrence/genetics + 3% of first degree relatives + familial ASD (with long PR interval)+ autosomal dominant

12. Syndromes + Holt Oram — upper limb deformity + autosomal dominant

13. Sport/physical activity + no restrictions unless moderate/severe PVD


14. Insurance + category 1 + generally no problem if defect closed early

15. Follow-up interval + early repair (<30 years) — no problems — discharge + laterepair — regular f/u

16. Follow-up care + level 2

17. Unresolved issues + surgery vs device closure + when to close in PHT + concomitantMaze procedure + upper age limit for surgery + PFO closure inpatients with suspected paradoxical embolism

Ventricular septal defect — unrepaired

Criteria Comments

1. Introduction and background + significant ventricular septal defects usually repaired in childhood+ see ventricular septal defect and PVD (Eisenmenger) butdiminishing+ small ventricular septal defect or postoperative septaldefect common in adults + Eisenmenger patients becoming lessfrequent

2. Survival/adult life + excellent for small ventricular septal defect + large ventricularseptal defect may have pulmonary vascular disease (Eisenmenger)+ may develop aortic regurgitation

3. Haemodynamic issues + left-right shunt + LV dilatation and impaired function + aorticregurgitation+ pulmonary vascular resistance in uncorrected large ventricularseptal defect

4. Arrhythmia/pacing + rare

5. Investigations Chest X-ray + baseline-cardiomegaly

ECG + routine + rhythm chamber enlargement

ECHO + number size and location of defects + LV/RVfunction + aortic regurgitation

TOE + if TTE image inadequate

Catheter + pulmonary vascular resistance + associatedlesions.


Holter + only if symptomatic

Exercise test + only if symptomatic + sports counselling

6. Indications for intervention + left — right shunt with left heart volume overload + reversiblepulmonary hypertension + aortic regurgitation + associatedabnormalities (RV outflow tract, subaortic stenosis) + previousendocarditis

7. Interventional options + surgery + catheter closure in muscular VSD(s)

8. Post treatment outcome + good surgical results

9. Endocarditis + prophylaxis in all

1062 Guidelines

10. Pregnancy/contraception

+ no contra-indications in uncomplicated VSD + pregnancycondra-indicated in pulmonary vascular disease (Eisenmengerdisease)

11. Recurrence/genetics + occasionally familial + usual recurrence risk + common cardiacanomaly in syndromes e.g. Down’s

12. Sport/physical activity + no restriction in small ventricular septal defect

13. Insurance + small ventricular septal defects category 1

14. Follow-up interval + infrequent follow-up unless haemodynamic abnormalities (e.g.aortic regurgitation)

15. Follow-up care + small ventricular septal defect 3, pulmonary vasculardisease (Eisenmenger) 2, aortic regurgitation/complicatedhaemodynamics 1

16. Unresolved issues + optimal management of Eisenmenger patients

Repaired ventricular septal defect

Criteria Comments

1. Introduction and background + common lesion + most patients now adults

2. Survival — adult life + excellent survival + occasional residual shunt + some develop RVor LV outflow tract obstruction + some develop aortic regurgitation

3. Haemodynamic issues + residual shunt + ventricular function + aortic regurgitation + newhaemodynamic abnormalities (RV outflow obstruction)

4. Arrhythmia/pacing + rare AV block, ventricular arhythmia

5. Investigations Chest X-ray + baseline — cardiomegaly

ECG + rhythm

Echo + residual VSD(s) + LV/RV function + aorticregurgitation

TOE if TTEinsufficient

+ TOE only it TTE inadequate

Catheter + rarely required


Holter + only if symptomatic

Stress test + only if symptomatic + sports counselling

6. Indications for intervention + if residual VSD; see ‘unrepaired VSD’

7. Interventional options + see ‘unrepaired VSD’

8. Post treatment outcome + see ‘unrepaired VSD’

9. Endocarditis + prophylaxis if residual VSD + questionable in closed VSD

10. Pregnancy/contraception + no contra-indications in uncomplicated closed VSD Pregnancycontra-indicated in PVOD (Eisenmenger)

11. Recurrence/genetics + see: ‘unrepaired VSD’


12. Sport/physical activity + no restriction in closed VSD

13. Insurance + category 1

14. Follow-up interval + can discharge if closed VSD without any residual abnormalities+ infrequent follow-up for minor residual lesions

15. Follow-up care + Eisenmenger 2, small VSD 3, aortic regurgitation/complicatedhaemodynamic 1

16. Unresolved issues —

Postoperative complete atrio-ventricular septal defect

Criteria Comments

1. Introduction and background

2. Survival/adult life + unoperated survivors develop PVD + surgical results markedlyimproved + status after repair depends mostly on left AV valvefunction + many patients have Down’s syndrome

3. Hemodynamic issues + left AV-valve regurgitation (±stenosis) + pulmonary vasculardisease + late sub-aortic stenosis

4. Arrhythmia/pacing + risk of complete heart block low (<2%) + atrial arrhythmiasespecially with left AV-valve dysfunction

5. Investigations Chest X-ray + cardiomegaly + pulmonary vascular markings+ pulmonary vascular disease

ECG + routine (LVH, RVH, CVH) + superior QRS-axis+ right bundle branch block + conductancedisturbances

ECHO/TOE + most useful investigation for + left AV valvemorphology and function + ventricular function+ residual lesions (shunt, sub-aortic stenosis)

Catheter + rarely required unless reoperation considered

MRI + rarely indicated

Holter + only in symptomatic patients

Exercise testing + rarely indicated

Additionalinvestigations

+ significant left AV valve dysfunction+ significant residual shunt + subaorticstenosis

6. Indications forreintervention

+ significant left AV-valve dysfunction + significant residualshunt + sub-aortic stenosis + progressive/symptomaticAV-Block

7. Interventional options + re-operation may require valve replacement

8. Post treatment outcome + excellent long-term results unless + actuarial survival after20 years >80% + left AV valve regurgitation (stenosis) + pulmonaryvascular disease + late sub-aortic stenosis

9. Endocarditis + prophylaxis in all cases

1064 Guidelines

10. Pregnancy/contraception + pregnancy contra-indicated in PVD (Eisenmenger)+ anticoagulation management in patients with prosthetic valves+ avoid oestrogen containing pill in pulmonary hypertension

11. Recurrence/genetics/syndromes

+ above average recurrence risk Down’s syndrome in >50% ofcomplete AVSD + app. 10–14% CCD in mothers with AVSD

12. Physical activity/sports + no restrictions if good repair and no significant arrythmias

13. Insurance + category 2 if well repaired

14. Follow-up interval + 1–2 yearly intervals with ECG and ECHO in stable cases

15. Follow-up care + level 2 unless significant haemodynamic problems

16. Unresolved issues + only limited data regarding long term prognosis

Postoperative partial atrio-ventricular septal defect (p-AVSD)

Criteria Comments


2. Survival/adult life + similar to secundum atrial septal defect unless significant to leftAV-valve regurgitation + unoperated p-AVSD have reduced lifeexpectancy + PVOD may develop late + status after repair dependson left AV valve function

3. Hemodynamic issues + before repair + size of shunt + degree of AV valve regurgitation+ after repair + residual shunt and left AV valve regurgitation+ subaortic stenosis

4. Arrhythmia/pacing + atrial arrhythmias rare unless left AV-valve regurgitation+ complete heart block very rare + pacing rarely required unlesssick sinus syndrome

5. Investigations ECG + superior-QRS axis + right bundle branch block+ rhythm follow-up

Chest X-ray + routine + cardiomegaly

ECHO/TOE + most useful investigation both for and afteroperation + left AV valve function + ventricularfunction + residual lesion

Catheter + rarely required unless reoperation considered


Holter + rarely indicated

Exercise testing + rarely indicated


—

6. Indications for intervention + all cases to be considered for intervention unless pulmonaryvascular disease + reoperation for significant left AV-valveregurgitation + residual shunt or subaortic stenosis+ progressive/symptomatic arrhythmias


7. Interventional options + surgery with valve repair or replacement + closure of re-/residualASD + Pacemaker (DDD) in progressive/symptomatic complete block

8. Outcome + excellent long term provided left AV-valve repair satisfactory

9. Endocarditis + prophylaxis indicated if left AV-valve regurgitation is present

10. Pregnancy/contraception + well tolerated in repaired cases + contraindicated in rare caseswith PVOD (Eisenmenger) + anticoagulation management inpatients with prosthetic valves + avoid oestrogen containing pill inpulmonary hypertension


+ none

12. Physical activity/sports + no restrictions if good repair and no significant arrythmias


14. Follow-up interval + 2 yearly intervals with ECG and ECHO in stable cases

15. Follow-up care + unoperated level 1, postoperative level 2

16. Unresolved issues + long term function of a non-reconstructed AV-valve is uncertain

Pulmonary stenosis

Criteria Comments


2. Survival/adult life + excellent if relieved effectively + poor if severe valve PSuntreated

3. Haemodynamic issues + PS severity + PR severity + leaflet dysplasia + right ventricularfunction

4. Arrhythmia/pacing + atrial arrhythmias in RV failure and tricuspid regurgitation+ pacing not indicated

5. Investigations Chest X-ray + baseline otherwise little value unless RV failure

ECG + rhythm RV + hypertrophy

ECHO/TOE + investigation of choice for RVOT gradientpulmonary regurgitation RV size/functiontricuspid regurgitation

Catheter + rarely needed except for balloon dilatation

MRI + rarely needed + assess RV size/function and RAdilation in sevre pulmonary regurgitation

Holter + not routinely indicated

Exercise + not routinely indicated


+ none

6. Indications for intervention + valve gradient >30 mmHg at rest or for symptoms

1066 Guidelines

7. Interventional options + balloon valvuloplasty almost always + surgery if valvecalcified/dysplasty

8. Post treatment outcome + excellent long-term results unless early failure significantpulmonary regurgitation uncommon

9. Endocarditis + low risk. Prophylaxis may not be required in mild cases

10. Pregnancy/contraception/fetal

+ routine pregnancy unless moderate to severe PS or right to leftshunt through ASD or PFO

11. Recurrence/genetics + 4% approximately

12. Syndromes + Noonan + congenital rubella + Williams + Alagille

13. Sport/physical activity + unrestricted unless severe

14. Insurance + category 1 after successful treatment or mild PS

15. Follow-up interval + can discharge if mild with ECHO. Every 1–3 years if more thanmild, PR, or desaturation.

16. Follow-up care + mild PS: 3, Excellent early result: 2, Residual gradient orsignificant PR: 2

17. Unresolved issues + none

Tetralogy of fallot — postoperative

Criteria Comments

1. Introduction and background + common lesion. Most Fallot patients are now adults

2. Survival/adult life + survival rate after surgery excellent (normal in selected groups)+ occasionally unoperated patients survive into adulthood.

3. Haemodynamic issues + pulmonary regurgitation/PS and RV function + tricuspidregurgitation + aortic regurgitation + residual lesions

4. Arrhythmia/pacing + late complete heart block rare + ventricular premature beatscommon in asymptomatic patients + sympotomatic VT rare + atrialarrhythmias common and relate to poor haemodynamics + smallincidence of late sudden death

5. Investigations Chest X-ray + baseline and occasionally follow-up+ cardiomegaly + RV outflow

ECG + routine + rhythm + access/QRS width (usuallycomplete right bundle branch block)

ECHO/TOE + regularly for PR/RVOTO/RV sizefunction/tricuspid regurgitation? Aorticregurgitation/LV function

Catheter + preoperative for residual lesions, coronaryanatomy intervention for dilatation/stent ofpulmonary artries + possibly in future forimplantable pulmonary valve

MRI + may become investigation of choice for RV sizefunction and pulmonary regurgitation

Holter + for symptoms and in poor haemodynamics


Exercise + exercise capacity, arrhytnmias


+ electrophysiological study for syncope,sustained arrhythmia (atrial or ventricular), RFA

6. Indications for intervention + significant RVOT or PA branch stenosis + aortic regurgitation+ residual VSD, significant pulmonary regurgitation (with symptomsand RV dilatation)

7. Interventional options + surgery, surgery with ablation, balloon dilatation/stenting, RFablatin catheter intervention for pulmonary valve insertion

8. Post treatment outcome + most patients well + RV function may not normalize afterpulmonary valve replacement + arrhythmia may persist + risk ofsudden death



+ no contra-indication to pregnancy in well repaired patients+ monitor ventricular function and arrhythmia + no additional fetalrisk

11. Recurrence/genetics + 1.5% for father, 2.5–4% for mother with TOF, 16% of Fallotpatients have deletion of chromosome 22q11- recurrence risk 50%.

12. Syndromes + 22q11

13. Sport/physical activity + no contra-indication to sport unless documented arrhythmia+ significant ventricular dysfunction


15. Follow-up interval + one/two yearly with ECG, ECHO±Holter, exercise test

16. Follow-up care + 1 if documented residual abnormalities/arrhythmia, 2 otherwise

17. Unresolved issues + risk stratification for sudden death + indication for implantabledefibrillator + timing of reoperation for pulmonary regurgitation

Conduits

Criteria Comments

1. Introduction and background + conduits used in repair of complex congenital heart disease+ usually RV-PA (e.g. PA/VSD, Truncus, ToF, TGA/VSD/PS)

2. Survival/adult life + all conduits in children deteriorate and require replacement(usually <10 years) + longevity of replacement unclear

3. Haemodynamic issues + stenosis of valve, subvalve or anastomosis to PA + pulmonaryregurgitation with RV volume overload + LV-Aortic pathway incomplex repairs

4. Arrhythmia/pacing + ventricular arrhythmias, surgical heart-block

5. Investigations Chest X-ray + baseline and follow-up + conduit calcification+ cardiomegaly

ECG + routine + rhythm

1068 Guidelines

ECHO + investigation of choice for follow-up of RV+ pressure gradient across conduit and PI + LVaortic pathway + ECHO may underestimategradient

TOE + not routine

Catheterization + evaluation for surgery + balloon dilatation orstenting

MRI + very useful for investigation of conduit function+ may become investigation of choice

Holter + only if arrhythmia suspected

Exercisefunction

+ not routine + useful for objective evaluation ofexercise tolerance

6. Indications for intervention + significant symptoms or conduit obstruction

7. Interventional options + usually surgical replacement of conduits + occasionally balloondilatation or stenting

8. Post treatment outcome + fate of replaced conduit uncertain + need long term follow-up


10. Pregnancy/contraception + pregnancy tolerated if haemodynamics stable + no contraceptionissues

11. Recurrence/genetics + usual recurrence rate for congenital heart disease + higher if22q11 deletion

12. Syndromes + 22q11 deletion

13. Sport/physical activity + avoid contact sports + otherwise no restrictions ifhaemodynamics good

14. Insurance + level 2

15. Follow-up interval + yearly with ECHO, ETT for ventricular function, arrhythmiasurveillance (ECG, Holter if symptoms) + significant conduitdysfunction may be present in mildly symptomatic patients

16. Follow-up care + category 1

17. Unresolved issues + type of conduit (homograft versus xenograft) + role of balloondilatation stenting

Aortic valve stenosis (unoperated)

Criteria Comments

1. Introduction and background + common especially biscupid aortic valve (1–2% of population)+ may occur with other lesions

2. Survival/adult life + normal if mild obstruction

3. Hemodynamic issues + degree of stenosis may progress + associated aortic regurgitation+ LV hypertrophy and function

4. Arrhythmia/pacing + VT and VF may occur during exertion with severe obstruction

5. Investigations ECG + LVH and repolarization changes


Chest X-ray + baseline + calcification

ECHO + investigation of choice + LV mass/function+ aortic valve/size/morphology/area + LV toaortic gradiant + aortic regurgitation

TOE + rarely of value except in endocarditis

MRI + rarely of value

Catheter + not for diagnosis + for coronary angiographyand balloon dilatation

Exercise testing + for repolarization changes and symptoms+ surgical decision making

6. Indications for intervention + symptoms: severe LV pressure overload + severe aortic stenosis

7. Interventional options + balloon valvuloplasty if valve uncalcified + rarely good option inadult + mechanical valve replacement, homograft or Rossprocedure depending on patient‘s age, sex, preferences and localexpertise

8. Outcome + recurrence common late after valvotomy + very good inuncomplicated cases of valve replacement.

9. Endocarditis Prophylaxis indicated in all

10. Pregnancy/contraception + low risk in asymptomatic patents even with moderate obstruction+ high risk in patients with severe obstruction + transcatheterintervention may be indicated in unplanned pregnancy

11. Recurrence/genetics/Syndromes

+ bicuspid valve may be familial + association with coarctation+ recurrence rate may be higher in syndromes.

12. Physical activity/sports + no competitive sports if obstruction is moderate or severe


14. Follow-up interval + depends on severity and progression rate ECG/ECHO±exercise test

15. Follow-up care + mild 3-moderate/severe 1

16. Unresolved issues + late outcome after the Ross operation

Postoperative valvar aortic stenosis

Criteria Comments

1. Introduction and background + common lesion + most interventions in children are balloondilation or open aortic valvotomy, aortic valve replacement,mechanical or biological prostheses or Ross procedure may havebeen performed

2. Survival — adult life + excellent

3. Hemodynamic issues + obstruction + regurgitation + LV function + pulmonary homograft(Ross)

4. Arrhythmia/pacing + arrhythmia rare + more common in LV hypertrophy + may causesudden death

1070 Guidelines

5. Investigations ECG + routine LVH + conduction disturbances+ repolarization changes

Chest X-ray + cardiomegaly

ECHO + see valvar aortic stenosis unoperated+ prosthesis function and paravalvular leak

TOE + useful in assessment of paravalvular leaks andsuspected endocarditis


Cath + rarely indicated (see valvar aortic stenosisunoperated)

Exercise testing + surgical decision making for timing ofreintervention

6. Indications forreintervention

+ recurrent obstruction (native valve or prothesis) + regurgitation+ occassionally haemolysis

7. Interventional options + mechanical valve, homograft or Ross operation + prosthesis maybe preffered by olderly + homograft may be preferred inendocarditis

8. Outcome + very good but anticagulant problems with mechanical valve andlate failure


10. Pregnancy/contraception + anticoagulants may cause embryopathy


+ see aortic valve stenosis unoperated

12. Physical activity/sports + high level activity possible in uncomplicated cases with goodLV function + contact contra-indicated in patients onanticoagulants


14. Follow-up interval + yearly

15. Follow-up care + Ross 1 otherwise 2

16. Unresolved issues + long-term outcome of Ross procedure + best anticoagulationprotocol in pregnancy

Subaortic stenosis unoperated

Criteria Comments

1. Introduction and background + uncommon form of obstruction + may be discrete or extend toadjacent structures + often progressive.

2. Survival/adult life + normal if obstruction not severe

3. Hemodynamic issues + progression very common + may cause aortic regurgitation+ associated lesions common (e.g. VSD)

4. Arrhythmia/pacing + See ‘aortic valve stenosis’


5. Investigations Chest X-ray + baseline

ECG + routine LVH and repolarization changes

ECHO + investigation of choice + visualise obstruction+ gradient across LV outflow tract + LVmass/function + aortic regurgitation

TOE + may be useful to define anatomy


Cath + rarely indicated (see ‘aortic valve stenosis’)

Exercise test + for repolarization changes and symptoms

6. Indications for intervention + progressive obstruction + lower threshold and aortic valvestenosis + aortic regurgitation

7. Interventional options + surgical resection

8. Outcome + recurrence possible


10. Pregnancy/contraception + low risk if no severe obstruction


+ may occur left heart abnormalities e.g. coarctation, Shone’ssyndrome + familial cases described

12. Physical activity/sports + no restriction if mild obstruction or after resection


14. Follow-up interval + depends on severity and progression rate usually 1–2 yearly


16. Unresolved issues + recurrence rate after resection + optimal timing of surgery

Unoperated coarctation

Criteria Comments

1. Introduction and background + may present in infancy or later in adolescence

2. Survival/adult life + rarely undiagnosed in childhood but long term survival ispossible

3. Haemodynamic issues + hypertension + premature atherosclerosis + LV hypertrophy/failure + aortic dissection + associated aortic/MV lesions

4. Arrhythmia/pacing + rare problems

5. Investigations ECG + LVH repolarization changes

Chest X-ray + cardiomegaly + ascending aorta dilation + ribnotching

ECHO + assessment of arch anatomy/gradient+ associated lesions LVH and function

TOE + rarely provides additional information

MRI + investigation of choice

1072 Guidelines

Holter + not indicated unless for ambulatory blood pressure

Exercise test + hypertension on exercise + arm/leg gradient+ inducible repolarization abnormalities

Catheterization + if MRI unavailable for arch anatomy + for coronaryangiography when indicated for intervention

Additional + screen for intracerebral vascular anomalies

6. Indications for intervention + resting or exercise induced hypertension + resting gradient >30 mmHg

7. Interventional options + balloon/stenting + surgical repair

8. Post treatment outcome + residual hypertension common despite adequate relief ofobstruction + accelerated atherosclerosis + reduced life expectancy



+ repair prior to pregnancy if possible + transcatheter interventionmay be indicated in unplanned pregnancy (worsening BP, LV failure)+ avoid oestrogen containing pill + growth retardation common+ spontaneous fetal loss increased

11. Recurrence/genetics + recurrence may be familial + 22q11 deletion in complex forms

12. Syndromes + Turners (present in approx 30%) + Williams (present in approx10%) + Shones (associated LV inflow/outflow abnormalities)

13. Sport/physical activity + should be restricted prior to repair

14. Insurance + category 3 for significant unoperated coarctation

15. Follow-up interval + most patients referred for intervention on diagnosis + 1 yearly ofmild cases with BP at rest and exercise/ECHO/Doppler/MRI


17. Unresolved issues + influence of age at operation on long-term outcome + influence ofdrugs on vascular phenotype in successful cases + role of interventionfor mild gradients + role of stenting as adjunct to balloon

Operated coarctation

Criteria Comments


2. Survival/adult life + long-term survival still reduced despite adequate early repair

3. Haemodynamic issues + persistent and late developing hypertension at rest and exercise+ aortic valve dysfunction + rare dissection

4. Arrhythmia/pacing + not an issue

5. Investigations ECG + LVH±repolarization changes

Chest X-ray + cardiomegaly + ascending aorta dilation + ribnotching

ECHO + assessment of arch anatomy/gradient+ associated lesions LVH and function

TOE + rarely provides additional information


MRI + investigation of choice

Holter + not indicated unless for ambulatory blood pressure

Exercise test + hypertension on exercise + arm/leg gradient+ inducible repolarization abnormalities

Catheterization + if MRI unavailable for arch anatomy + for coronaryangiography when indicated for intervention

Additional + screen for intracerebral vascular anomaliesadvocated by some

6. Indications for intervention + significant recoarctation (gradient >30 mmHg at rest) + aorticaneurysm

7. Interventional options + balloon/stenting for anatomically suitable recoarctation+ surgery for complex situations±aneurysms

8. Post treatment outcome + excellent but late hypertension and prematureatherosclerosis/CVA/MI/heart failure



+ relieve residual coarctation prior to pregnancy or duringunplanned pregnancy + monitor closely for hypertension + avoidoestrogen containing pill if rest or exercise hypertension

11. Recurrence/genetics —

12. Syndromes —

13. Sport/physical activity + no restrictions if adequate relief of obstruction/no residualhypertension


15. Follow-up interval + yearly with same investigations as for unoperated coarctation


17. Unresolved issues + influence of age at repair, type of repair of intervention on latehypertension + late outcome of balloon/stenting + pathophysiologyof late hypertension

Patent arterial duct

Criteria Comments


2. Survival/adult life + normal life expectancy in closed PDA + rare PVD for large PDA

3. Haemodynamic issues + usually none — LV dilatation/pulmonary hypertension insignificant PDA

4. Arrhythmia/pacing + none

5. Investigations Chest X-ray + baseline + cardiomegaly + ductal calcification

ECG + usually normal + LVH with large PDA

ECHO/TOE + usually diagnostic + TOE rarely indicated

1074 Guidelines

Catheter + for closure coronary angiography in older patients

MRI + not indicated

Holter + not indicated

Exercise + not indicated


+ none

6. Indications for intervention + controversial for silent of very small PDA + continuous murmur+ LV dilatation

7. Interventional options + catheter closure Intervention of choice + several device options+ surgery for rare cases

8. Post treatment outcome + excellent + residual shunt in up to 10%

9. Endocarditis + not required after complete closure + prohylaxis indicatedotherwise


+ no problems unless pulmonary vascular disease

11. Recurrence/genetics + none

12. Syndromes + congenital rubella

13. Sport/physical activity + no restrictions unless PVD

14. Insurance + category 1 for small PDA or after closure

15. Follow-up interval + discharge 1 year after closure

16. Follow-up care + level 3 unless PVD (1)

17. Unresolved issues + indication of closure for small PDA

Ebstein’s anomaly

Criteria Comments

1. Introduction and background + wide spectrum of pathologic anatomy which determines onset ofseverity of symptoms

2. Survival/adult life + extremely variable natural history + infant survivors usually reach

3. Haemodynamic issues + cyanosis at rest and/or exercise (right — left shunt at atrial level)reduced exercise capacity + congestive heart failure (tricuspid stenosis/regurgitation/small RV) + associated lesions + LV abnormalities

4. Arrhythmia/pacing + atrial arrhythmias are common + increase with age + related topre exultation and atrial dilatation + risk of sudden death

5. Investigations Chest X-ray + marked cardiomegaly + right atrial enlargement

ECG + baseline (characteristic pattern) + follow-up forrhythm

ECHO/TOE + severity of tricuspid valve displacementdysplasia and regurgitation + RV size + associatedlesions + LV function


Catheter + rarely required unless for coronary angiographyin older patients or at EPS

MRI + rarely required

Holter + useful for arrhythmia monitoring

Exercise + baseline and follow-up + cyanosis + exercisetolerance + arrhythmia


+ EPS for arrhythmia diagnosis and RFA

6. Indications for intervention + decrease in exercise tolerance + heart failure + increase incyanosis + arrhythmia

7. Interventional options + surgery for tricuspid valve repair or replacement + RFA forarrhythmias/pre exultation

8. Post treatment outcome + symptomatic improvement usual + tricuspid valvereplacement — reoperation, thrombotic complications + ongoingarrhythmia problems frequent + risk of sudden death remains+ anticoagulants for atrial arrhythmia and prosthetic tricuspid valve



+ well tolerated unless cyanosis or heart failure + fetus at risk incyanosed mother

11. Recurrence/genetics + 6% in affected mother. 1% in affected father. Familiar occurrencedocumented.

12. Syndromes + rare

13. Sport/physical activity + recreational sport in asymptomatic patient

14. Insurance + unoperated asymptomatic or well post operative category 2

15. Follow-up interval + depends on clinical status + annual follow-up withECHO/Holterexercise test

16. Follow-up care + level 1 (operated and unoperated)

17. Unresolved issues + recurrence of arrhythmias + long-term fate of repairs

Fontran

Criteria Comments

1. Introduction and background + palliative procedure for single ventricle physiology in which allsystemic venous return directed to the lungs – multiplemodifications

2. Survival/adult life + improved survival with strict selection criteria + late failure evenin best cases

3. Haemodynamic issues + function of systemic ventricle (preload deproved) + pulmonaryvascular resistance + obstruction in Fontan connection + atrialenlargement + pulmonary venous obstruction + AV valveregurgitation + chronic venous hypertension+ desaturation/paradoxical embolus in fenestrated Fontan+ pulmonary arterio venous malformations in some

1076 Guidelines

4. Arrhythmia/pacing + atrial arrhythmias common + increase with follow-up + sinus nodedysfunction + pacing – ventricular pacing requires epicardial system

5. Investigations Chest X-ray: + baseline and follow-up + cardiomegaly+ pulmonary vascular markings

ECG: + rhythm

ECHO/TOE: + most useful investigation for + ventricularfunction + AV valve regurgitation + residualshunts + obstruction of Fontan connections+ thrombus in atrium + routine TOE (2 yearly maybe indicated or of arrhythmia present)

Catheter: + for haemodynamic assessment and angiographyin clinical deterioration

MRI: + obstruction of Fontan connection + occasionallyuseful for RA size and anastamieos

Holter: + routine and for symptomatic arrhythmia

Exercise testing + reaction activities only

Additionalinvestigations:

+ blood/stool for PLE

6. Indications for intervention + cyanosis + obstruction to Fontan connection + systemic AV valveregurgitation + ventricular failure + arrhythmia + pulmonaryvenous obstruction

7. Interventional options + consider conversion to TCPC or transplant in failing Fontan+ closure of fenestration + AV malformations + RFA+ Supraventricular arrhythmia + AV sequential pacing

8. Post treatment outcome + variable success with catheter ablation of atrial arrhythmias+ PLE has <50% 5 year survival + Fontan conversion resultsunclearatrial arrhythmias common.



+ pregnancy possible with perfectly selected patients and propercare + high maternal risk in ‘failing Fontan’ + higher miscarriagerate + fetal risk of CHD may be higher + avoid oestrogen pill ifejection fraction <40%, residual shunt, or spontaneous contrastin RA + ACE inhibitors should be withdrawn if on anticoagulants –need meticulous management

11. Recurrence/genetics + non

12. Syndromes + none

13. Sport/physical activity + recreational sports only


15. Follow-up interval + at least yearly review with ECHO, ECG Holter, exercise testing,blood testing


17. Unresolved issues + indications for and results of Fontan conversion + outcome ofTCPC in modern era + role of anticoagulation + medical therapy forfailing systemic ventricle + role of ACE inhibitors


Marfan’s syndrome

Criteria Comments

1. Introduction and background + abnormal fibrillin gene on chromosome 15q + autosomaldominant inheritance + cardiac defect largely determined outcome

2. Survival/adult life + death from cardiac problems + life expectancy reduced butimproved by good cardiac follow-up and surgery

3. Haemodynamic issues + acute aortic dissection — risk higher if the aortic sinuses >55mm+ aortic regurgitation + mitral valve prolapse/regurgitation

4. Arrhythmia/pacing + atrial and ventricular arrhythmia in mitral valveprolapse/regurgitation

5. Investigations Chest X-ray + not helpful for follow-up of aorta

ECG + rarely useful

ECHO/TOE + most valuable investigation for serial follow-upof aortic root dimensions, valve function (aorticand mitral)

Catheter + rarely indicated

MRI + excellent investigation for aortic arch anddescending aorta + compliments echocardiography

Holter + not routine

Exercise testing + not routine


+ non-cardiac assessment (ophthalmic,orthopaedic etc.)

6. Indications for intervention + beta blockers for aortic dilatation + surgery if aortic diameter>55 mm or rapid increase + significant aortic regurgitation+ significant mitral regurgitation

7. Interventional options + urgent surgery for dissection + aortic root and valve replacement+ valve sparing operation may be indicated

8. Post treatment outcome + Surgery improves life expectancy but other dissections stillpossible + beta blockers delay/prevent progression

9. Endocarditis + prophylaxis in valve regurgitation and after aortic surgery


+ pregnancy contraindicated if aorta is >45 mm + pregnant womenshould be on beta blockers + caesarean section to be discussed ifaorta is dilated

11. Recurrence/genetics/syndrome

+ approximately 50% (autosomal dominant)

12. Sport/physical activity + strenuous exercise contra-indicated high altitude and divingcontra-indicated (spontaneous pneumothorax)


14. Follow-up interval + annual follow up for aortic dilatation + more frequent evaluationif aortic diameter increasing


1078 Guidelines

16. Unresolved issues + role of early beta blockade + long term results of surgeryincluding valve sparing

Postoperative transposition (Mustard/Senning)

Criteria Comments

1. Introduction and background + common lesion — most Mustard/Sennings patients nowadults — operation replaced by arterial switch mid 1980’s

2. Survival/adult life + low early mortality + significant late morbidity/mortality fromarrhythmia/baffle obstruction/RV failure with risk of sudden death

3. Haemodynamic issues + intra-atrial baffle obstruction (systemic and pulmonary venous)more common in Mustard than Senning + tricuspid regurgitation/RVfailure relatively rare but important to detect early

4. Arrhythmia/pacing + progressive loss of sinus rhythm on Holter with follow-up + slowjunctional rhythm may rarely require pacing + tachyarrhythmias(predominantly atrial flutter) may be related to high incidence oflate sudden death + pacing may be required if antiarrhythmic drugs

5. Investigations ECG + RVH with basic rhythm (often junctional)

Chest X-ray + useful for cardiomegaly + pulmonary venousobstruction

ECHO/TOE + TTE for ventricular function/tricuspidregurgitation + TOE essential if questions remainregarding baffle function

MRI + rarely required if TOE available

Holter + occult arrhythmia + not predictive of SD

Exercise test + exercise tolerance + evaluation of arrhythmia

Catheterization + for intervention and assessment of new onsetsymptoms

Additional EP study/RFA for refractory atrial arrhythmias

6. Indications for intervention + baffle obstruction + baffle leaks + tricuspid valve dysfunction+ RV failure

7. Interventional options + balloon/stenting for pathway obstruction + transcatheter closurefor baffle leaks + tricuspid valve/replacement + conversion toarterial switch (pulmonary artery banding) + transplantation

8. Post treatment outcome + risk of sudden death despite lack of symptoms or overthaemodynamic disturbance



+ pregnancy not contra-indicated in most cases + monitor RVfunction throughout + no contraceptive issues + long-termconsequences on RV function not known

11. Recurrence/genetics + familial recurrence of TGA rare



13. Sport/physical activity + generally normal activities + maximal exercise tolerance likely tobe diminished


15. Follow-up interval + yearly


17. Unresolved issues + risk stratification for sudden death + fate of systemicRV/tricuspid valve + indication/conversion/transplant strategies

Congenitally corrected transposition

Criteria Comments

1. Introduction and background + rare lesion + usually associated with other abnormalities + mayoccur with dextrocardia

2. Survival/adult life + common to survive to adult life + associated lesions common(VSD, PS, left AV valve regurgitation) determine outcome

3. Haemodynamic issues + cyanosis with VSD and PS + PVD if VSD and no PS + systemicventricular failure with systemic A-V valve regurgitation + referralbefore systemic ventricular dysfunction

4. Arrhythmia/pacing + spontaneous CHB (2% per year) and post surgical heart block+ endocardial pacing in the morphologic LV + atrial arrhythmiascommon + ventricular arrhythmias with systemic ventriculardysfunction + epicardial pacing if potential for paradoxical embolus

5. Investigations Chest X-ray: + baseline + follow-up for associated lesions+ cardiomegaly

ECG: + rhythm

ECHO/TOE: + size and function of systemic ventricle+ morphology of left A-V valve + associated lesions

Catheter: + for pulmonary haemodynamics and anatomy ofassociated lesions

MRI: + rarely required

Holter: + for occult arrhythmia detection

Exercisefunction:

+ helpful for timing of surgery + oximetry+ exercise tolerance

Additionalinvestigations:

+ occasionally MUGA for ventricular function

6. Indications for Intervention + +>moderate systemic AV valve regurgitation + significantassociated lesions + pacemaker for complete AV block withsymptoms, profound bradycardia or chronotropic incompetence

7. Interventional options + valve replacement + pulmonary artery banding + ‘double switch’(controversial in adults)

8. Post treatment outcome + good if left A-V valve replacement before systemic ventricularfunction deteriorates + atrial arrhythmias common


1080 Guidelines


+ pregnancy not contra-indicated if asymptomatic + monitorventricular function and rhythm + long-term consequences onsystemic ventricular function unknown + avoid oestrogen containingcontraceptive pill if cyanosed/pulmonary hypertension

11. Recurrence/genetics + 4%


13. Sport/physical activity + no restriction on recreational activities

14. Insurance + Category 3 in most cases

15. Follow-up interval + yearly, with ECHO, exercise test±Holter

16. Follow-up care + level 1 (pre and postoperative)

17. Unresolved issues + classical repair of VSD and PS versus ‘double switch.’

8 Recommendations for FutureDevelopments in Europe

8.1 Summary

This task force seeks to describe the increasingpopulation of grown-ups with congenital heart dis-ease, promote a review of their needs and makeproposals for service delivery. The representativeswere chosen from Europe, Canada and United Statesbecause they had pioneered the subject in theircountries, as well as contributed to previous guide-line documents. It is recognized that this is a ‘speci-ality in evolution’ and that new information is likelyto be acquired rapidly. There is an urgent need toestablish a comprehensive hierarchical service withdedicated specialist units and defined referral links.This report proposes a framework, which should beadapted by health care systems in European coun-tries and developed further. Cases of congenitalheart disease in adults will strongly outnumberthose in children and the number will continue togrow. We hope the contents of this report will be astimulus to investment to enable patients to bemanaged in adequately staffed and funded expertunits and to continue to receive the excellent levelof health care provided to them during childhood.

8.2 Specific recommendations

1 Patients should be transferred to appropriateadult care at a flexible age of 16–18 years.

2 There should be a transitional service for 12–16year olds to facilitate this process.

3 Each paediatric cardiology and adult cardi-ology unit should have a defined referral pathinto the grown-up congenital heart diseaseservice.

4 A hierarchical system for care delivery should beco-ordinated by specialist regional units forgrown-ups with congenital heart disease.

5 These regional units should be sited within adultcardiology programmes in multidisciplinaryteaching environments.

6 The specialist units should be fully staffed andresourced, to provide optimal investigation andtreatment for patients with complex congenitalheart disease.

7 After definition of level of care required at thespecialist unit, patients should be looked after,either exclusively in the specialist unit (level 1),have shared care between the specialist unitand trained local cardiology units (level 2) or befollowed in their local unit (level 3).

8 Follow up plans should be clearly communicatedto the patient, their primary care physician andlocal cardiology units. Patients should be pro-vided with a ‘health passport’ containing keyinformation on their condition, treatment, andoutcome issues and follow-up plan.

9 Training programmes for specialist staff andcardiologists with an ‘interest’ should be de-fined and implemented.

10 Most specialist examinations, interventionalcatheterizations and surgical procedures shouldbe performed at the specialist centres.

11 The specialist centres should provide the frame-work for training and the establishment of acomprehensive national database for research.

12 The specialist centres’ staff should have exper-tise in medical and psychosocial management.

13 As far, as possible, the provision of health caredelivery for grown-ups with congenital heartdisease and training of staff should be standard-ized and co-ordinated through the EuropeanSociety of Cardiology.


9 European Society of Cardiology Staff

Alan Howard, Chief ExecutiveKeith McGregor, Scientific DirectorVeronica Dean, Practice Guidelines CoordinatorDominique Poumeyrol-Jumeau, Practice Guide-

lines Assistant.

AcknowledgementsTask Force Members would like to thank Dr MatthewBarnard, Consultant Anaesthetist in the Grown-UpCongenital Heart Disease Unit, Middlesex Hospital,London, UK, for his contribution to section 5.

Our sincere thanks to Paula Hurley, ResearchCoordinator in the Cardiothoracic Unit at GreatOrmond Street Hospital, London, UK, who hasworked tirelessly in the coordination, compositionand preparation of this Task Force report.

We would also like to thank colleagues, patientsand their families who have provided invaluableinformal advice.

Appendix A

Adult congenital heart disease survivalsimulator

Thank you for downloading this simple simulationprogram. It is intended to model the rate ofattrition of a population, such as a particularcardiac condition for which cardiac surgery hasbeen performed.

Because surgery has improved considerably twophases for which different mortality rates can beselected have been incorporated. You need toselect a death rate per year (percent) and thelength of the first phase, and then the death ratefor the second phase, and the total length of followup for both phases.

This will run in Microsoft Excel. Because thereare modules of automated code, when loading thefile, Excel will alert the user that the file containsMacros. This should not concern the user, but itdoes not have to be said that every computer usermust take responsibility for using an up to datevirus checker. The programme was free of virusaccording to the most up to date McAfee Virus Scan,but no responsibility can be taken during furtherdistribution.

This is a non-commercial program and must not beredistributed for profit. Full copyright remains withDr Graham Derrick ([email protected]).The source code can be made available to thosewho request it, with the request that any modifi-cations are returned to Dr Derrick.

This simulation program can be consulted anddownloaded from the ESC web site at the follow-ing address: www.escardio.org, in the Scientific& Clinical Information/Guidelines & ScientificStatements/Task Force Guidelines’ section.

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