Progress in Cardiovascular Diseases 53 (2011) 324–326www.onlinepcd.com

Editorial

The Future of Adult Congenital Heart Disease Care in the United States

In the past several generations, pediatric cardiologyand pediatric cardiac services have greatly expanded andmatured, improving the outcomes of children andadolescents with congenital heart defects. In the nextgeneration, adult congenital heart disease (ACHD) carewill also mature and improve. From an Americanperspective, we can imagine the elements of progressand of the changing topography of the patient profile.

The changing profile of ACHD care

Some patient groups will become smaller because ofimprovements in patient care. There will be fewer cyanoticpatients and fewer patients with pulmonary hypertensiondue to congenital heart defects. Mustard and Senningpatients will diminish in number because their procedureshave been replaced by the arterial switch. AtriopulmonaryFontan patients will become fewer for the same reasons astheir numbers are reduced by the passage of time, by theoccurrence of complications, and by revisions to more up-to-date Fontan surgical strategies.

Other patient groups will increase in numbers. Arterialswitch patients will dominate the transposition profile.Patients with hypoplastic left heart syndrome will reachadult life in numbers never before seen and will constitutea growing proportion of the growing number of Fontanpatients. The number of implantable cardiac defibrillatorswill increase as we get a better handle on predicting riskfactors for sudden death and reducing such mortality.More and more women with congenital heart defects willhave successful pregnancies.

The elements of progress

Adult congenital heart disease clinics in the UnitedStates will grow in size, quality, and the range of servicesthey offer. Because of the development of such clinics,more adult cardiologists will be able to participate astrainees in ACHD clinics to enhance their competence inmanaging patients with congenital heart defects. At thesame time, cardiologists with special qualifications to care

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for patients with ACHD will become more numerous inthe United States and be available as regional resources tohelp lead the management teams for complex patientswith ACHD.

Training opportunities to learn how to assess and carefor patients with ACHD will become more numerous.Instead of being limited to ACHD clinic exposure and totextbooks, case-based learning through publications andon the Internet will become increasingly important.Indeed, more flexible and more accessible learningopportunities will become part of the continuing educationmenu for ACHD providers of all types.

As ACHD clinics grow, there will be a need to expandACHD diagnostic services. First among these is echocardi-ography. Protocols for doing ACHD echocardiograms(echos) will be developed and publicized.More sonographerswill become interested in this field and will becomecompetent in doing echos on patients with ACHD. Likewise,echocardiographers will become more conversant with howto properly conduct and interpret ACHD echo studies. Echoqualitywill improve.Magnetic resonance imaging, computedtomography, and 3-dimensional echocardiography will alsomake important progress as these other technologies competefor a place in future care algorithms.

Pediatric cardiology programs will take an increasinglyprominent role in ACHD care. Their first contribution willbe to reduce the “loss to care” of children and adolescentswith complex congenital heart defects. They will alsomake sure that the “lifelong surveillance and caremessage” is repeatedly received and understood bypatients and parents alike. Some pediatric institutionswill take the lead in organizing ACHD care. This may be apreferred strategy for keeping patients in care becausepatients may not have to change institutions to transfer tohigh-quality adult care. Some pediatric institutions willshow increasing leadership in managing other long-termmedical conditions of their adolescent patients andensuring that these patients continue to receive high-quality care as young adults and beyond.

The American ACHD community has producedguidelines for patient care.1 It is quite striking that thepediatric cardiology community has not produced such

324

Abbreviations and Acronyms

(A)CHD = (Adult)Congenital heart disease

MRI = Magnetic resonanceimaging

325Editorial / Progress in Cardiovascular Diseases 53 (2011) 324–326

guidelines. Surely, it isinevitable that the pedia-tric cardiology communi-ty will produce coherentguidelines aiming at opti-mizing the quality ofpatient care.

Quality markers will increasingly become incorpo-rated into daily practice, thanks to extensive data banksand the burgeoning evidence base of what constitutesquality care of patients with ACHD. Patient outcomeswill improve.

Patients will be able to communicate with theirproviders both physically and electronically, with lessdisruption to their daily lives. Patients will becomeincreasingly important partners in care through theavailability and use of personal health records. Throughsuch resources, patients will be able to review datapertaining to their care and share or restrict access to iteasily for providers with an interest in their information.Patient education will improve. Helpful and high-qualityinformation will become available over the Internet indifferent languages for interested patients and theirproviders. Couples will routinely receive preconceptioncounseling. Female patients with congenital heart disease(CHD) will be educated as to contraceptive optionsappropriate to their situations and will be protected fromunwanted pregnancy. There will be fewer teenagepregnancies in pediatric cardiac practice. Pregnantpatients with CHD will receive safer and more effectivereproductive care.

Nurse practitioners and physician assistants will play alarger role in the care of patients with ACHD. Adultcongenital heart disease cardiologists will come from bothpediatric cardiology and adult cardiology backgroundsand will regularly work together in ACHD clinics. Eachgroup will work to receive the recognition and respect ofthe other for their particular roles and achievements.There will be greater social justice, and more patients withCHD will be able to access ongoing surveillance fromqualified practitioners.

Primary care physicians will become partners in thecare of patients with CHD and will help care to becomemore holistic and comprehensive.

The manpower needs of the ACHD community willbe evaluated on an ongoing basis by a multisocietygroup. The various types of personnel needed toprovide ACHD care will be identified. Trainingprograms will be organized and funded. Manpowerprojections will be developed and continually updated.These individuals will be credentialed in a consistentway, and recertification programs will be established.Such progress will be required in a range of disciplinesincluding cardiology, nursing, echocardiography, mag-netic resonance imaging, electrophysiology, and inter-ventional catheterization.

Our Understanding of the genetic basis of CHD willimprove, and the implications for these various mutationswill become evident and lead to better patient management.

Adult congenital heart disease care clinics willincreasingly become positive contributors from a businessstandpoint to their institutions and practice groups.

Challenges

One of the challenges to improving care in the UnitedStates has to do with the development of enough large,high-volume, high-quality ACHD centers to really anchorthe care of complex patients across the country. At themoment, the amount and quality of care are not wellanchored in many regions and in most American urbancenters. It is possible that the future will witness thedevelopment of small- to medium-sized ACHD clinicswith less complete and less advanced sets of consultingteam members compared to what the 32nd BethesdaConference had recommended,2-6 namely, the develop-ment of 30 to 50 regional centers of excellence in theUnited States.

Patient advocacy will be an important factor in helpingto develop ACHD care facilities that can provide high-quality and comprehensive care, rather than leaving itstrictly up to a practice model to do so. The AdultCongenital Heart Association has been an importantcatalyst to progress in the past decade, and it and otherorganizations like it will need to keep up the good work ifprogress is to be intelligent over the next generation. InBritain, there is presently a national effort to reduce thenumber of congenital heart centers to optimize patientoutcomes. The National Health Service has been urged todo so by the patient association, which has chosen qualityof care and improved patient outcomes over convenienceand proximity. Courageous decisions of a similar naturehave already been made in Sweden. If this ever couldoccur, such emphasis would be an important part of theway forward for the United States.

Another challenge to the field will be to establishincreasingly closer links and relationships betweenpediatric cardiology and ACHD care providers. Themodel of a continuum of care from cradle to grave willneed to be translated into real models, where pediatricproviders, adolescent providers, and adult providers workwith each other in creative ways and become a communityconcerned with optimizing care across the age spectrum.Pediatric providers and their institutions need to becomeincreasingly active partners in participating in adult careand in facilitating the transfer of pediatric and adolescentpatients to competent adult care, some of which will beprovided by members of their own pediatric communitywho have developed a career interest and competence inalso managing adult patients.

Should ACHD care be anchored in adult institutionsand adult cardiology practice or in pediatric institutions in

326 Editorial / Progress in Cardiovascular Diseases 53 (2011) 324–326

partnership with pediatric cardiologists? Successful mod-els will undoubtedly be developed in both ways. However,the most successful alignment is likely to have pediatriccardiac care providers as active partners in ACHD care.Given the power, resources, and focus of providers dealingwith coronary artery disease and heart failure, it is unlikelythat many ACHD clinics will develop successfully in theadult environment.

Another challenge will be to strengthen and consolidateACHD research. The days of single-center observationalstudies have largely passed. There will be a need for morebasic science and translational research and for popula-tion-based and outcome research using data resources thatare increasingly and abundantly available. The focus willneed to be on designing multicenter research studies andearning research grant support. Research will need to getbeyond the cardiac and cardiology aspects of ACHDresearch and become more holistic and inclusive.

Conclusion

The next generation will see important changes inAmerican ACHD care and, hopefully, much progress.Some progress will be mandated by new and betterscience. Some will flow from the changing treatments ofchildren with CHD. Some will depend upon inspiredleadership in the ACHD community and in new alliancesand partnerships between ACHD and other groups withcommon interests. Much will depend on the effectivenessof patient advocacy efforts in the United States. Changesin ACHD care will vary between countries and between

regions in America. The potential is great, but thehazards are real. Let us venture forward with courageand determination.

Gary WebbCincinnati Adolescent and Adult Congenital Heart Center

Cincinnati Children's Hospital Heart InstituteCincinnati, OH, USA

E-mail address: gary.webb@cchmc.org

References

1. Warnes CA, Williams RG, et al: ACC/AHA 2008 guidelines for themanagement of adults with congenital heart disease: a report of theAmerican College of Cardiology/American Heart Association TaskForce on Practice Guidelines (Writing Committee to DevelopGuidelines on the Management of Adults With Congenital HeartDisease). Developed in collaboration with the American Society ofEchocardiography, Heart Rhythm Society, International Society forAdult Congenital Heart Disease, Society for Cardiovascular Angiog-raphy and Interventions, and Society of Thoracic Surgeons. J Am CollCardiol 2008;52:e1-e121.

2. Warnes CA, Liberthson R, et al: Task force 1: the changing profileof congenital heart disease in adult life. J Am Coll Cardiol2001;37:1170-1175.

3. Foster E, Graham TP Jr, et al: Task force 2: special health care needsof adults with congenital heart disease. J Am Coll Cardiol 2001;37:1176-1183.

4. Child JS, Collins-Nakai RL, et al: Task force 3: workforce descriptionand educational requirements for the care of adults with congenitalheart disease. J Am Coll Cardiol 2001;37:1183-1187.

5. Landzberg MJ, Murphy DJ Jr, et al: Task force 4: organization ofdelivery systems for adults with congenital heart disease. J Am CollCardiol 2001;37:1187-1193.

6. Skorton DJ, Garson A Jr, et al: Task force 5: adults with congenitalheart disease: access to care. J Am Coll Cardiol 2001;37:1193-1198.