HLHS Current Consideration &

Expectation SHA 2017

Ahmed Abdullah Jamjoom

A spectrum of cardiac malformations characterized by

underdevelopment of the left heart with significant hypoplasia

of the left ventricle including atresia, stenosis or hypoplasia of

the aortic or mitral valve, or both , and hypoplasia of the ascending

aorta and aortic arch

Society of Thoracic Surgeons Congenital Heart Surgery Nomenclature and Database defines

HLHS

Incidence • 0.2 per 1000 live births • HLHS accounts for 2.5% of congenital heart defects

but is responsible if left untreated for up to 25-40% of all deaths within the first week of life

• Without surgical palliation, 95% of children die within the first month of life

• A male predominance ( male to female ratio 1.5:1 )

• Prevalence of CHD 2.1 and 10.7 per 1,000 persons

• The incidence of severe CHD 5.4 per 1,000 live births per year

• HLHS 9.1% of total severe CHD

• 0.5 per 1000 live births VS 0.2 per 1000 live births

Congenital Heart Dis. 2012 May-Jun;7(3):277-82. Incidence of severe congenital heart disease at the province of Al-Qassim, Saudi Arabia. Al-Mesned A1, Al Akhfash AA, Sayed M.

Pathogenesis • Intrauterine infarction, infection, and

LV cardiomyopathy have been suggested

• Anatomical abnormality of the atrial septum or foramen oval

• Abnormal development of the left ventricle itself (Endocardial fibroelastosis)

• Abnormality of the mitral (parachute, arcade) and/or the aortic valve (bicuspid or unicuspid)

• Fetal demise is reported, most pregnancies reach term gestation

Genetics • Commonly occurs sporadically in otherwise

clinically normal neonates

• Recurrence risk of left heart disease in first degree relatives of infants with HLHS is 2-4%

• HLHS has been associated with Turner syndrome (XO), Jacobsen syndrome (deletion of distal 11q), trisomy 13, and trisomy 18

Impact of Patient Characteristics on Results of Norwood Operation for Hypoplastic Left Heart Syndrome Bahaaldin Alsoufi, Makoto Mori, Scott Gillespie, Brian Schlosser, Timothy Slesnick, Brian Kogon, Dennis Kim, Ritu Sachdeva, Kirk Kanter Annals of Thoracic Surgery 2015, 100 (2): 591-8

Diagnosis • Echocardiographic imaging,

pre - or postnatal

• Mortality is not reduced if a prenatal diagnosis is made

• Morbidity reduction when HLHS is diagnosed before birth

Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome Shaine A Morris, Mary K Ethen, Daniel J Penny, Mark A Canfield, Charles G Minard, David E Fixler, Wendy N Nembhard Circulation 2014 January 21, 129 (3): 285-92

• Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is pre surgical

• Efforts to improve prenatal

diagnosis and delivery near a large volume CSC may significantly improve neonatal HLHS survival

Pre-natal Diagnosis

• Potential fetal intervention

• Fetal listing for heart transplantation

• Genetic testing and evaluation for extra

cardiac anomalies

• Counseling to discuss the option of pregnancy termination or comfort care after birth

Fetal Intervention In HLHS • Only applicable to small

number of cases

• Long-term outcome still in question

(Circulation. 2010;121:1256-1263.)

Current Status of Fetal Cardiac Intervention Doff B. McElhinney, MD; Wayne Tworetzky, MD; James E. Lock, MD

Presentation

• Honeymoon period immediately after birth because of adequate systemic perfusion through the PDA and relatively high PVR

• PDA begins to close and PVR decreases, infants become symptomatic with a decrease in systemic perfusion

• Infants with a restrictive or no ASD at birth present with severe cyanosis and respiratory distress

Choice of management

• Staged palliative surgery is the management choice for HLHS over cardiac transplantation and compassionate care

• Debate to whether compassionate care should be offered • Most parents choose staged surgical palliation, and compassionate

care is rarely selected

Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery Erin A Paul, Kristina Orfali, Thomas J Starc Pediatric Cardiology 2016, 37 (8): 1446-145

Attitudes and practices of cardiologists and surgeons who manage HLHS Milan Prsa, Crystal D Holly, Franco A Carnevale, Henri Justino, Charles V Rohlicek Pediatrics 2010, 125 (3): e625-30

Transplantation • Highest waitlist mortality of all solid organ recipients 17%

• Primary heart transplant for HLHS is rarely offered in this era and is

not recommended

• 8% of the Patients who had undergone Norwood procedure were listed for heart transplantation

• 74% received heart transplantation 23% died before transplantation 3% were delisted • 38% between the Glenn and Fontan 35 % between the Norwood and Glenn 27 % post Fontan • Overall survival at 1, 5, and 10 years following transplantation was

82%, 68%, and 59%, respectively

Ethics of Cardiac Transplantation in Hypoplastic Left Heart Syndrome

Alexander A. Kon Pediatr Cardiol (2009) 30:725–728, DOI 10.1007/s00246-009-9428-z

Stage I

• Include the Norwood procedure, the Sano

procedure, and the hybrid approach • Objectives of initial surgery Unobstructed blood flow from the RV to the body Controlled source of pulmonary blood flow Unobstructed way between the PV and the RV

Stage II Cavopulmonary shunt

• The original shunt is removed and the superior vena cava is anastomosed end-to-side to the pulmonary artery

• Patients whose stage I was performed in a hybrid

fashion require a more extensive stage II operation

Stage III Fontan procedure

• Creates a venous pathway that directs the IVC flow

into the PA • Although this is the final stage of palliation for HLHS,

proportion of patients may require additional intervention

• Failure of the Fontan may require takedown of the Fontan or heart transplant

HLHS Stage I palliative surgery Early Mortality Series

23%

Variation in Outcomes for Benchmark Operations: An analysis of the STS Congenital Heart Surgery Database Jeffrey Phillip Jacobs, MD,1 Sean M. O’Brien, PhD,2 Sara K. Pasquai, MD,2 Marshall Lewis Jacobs, MD,3 Francois G. Lacour–Gayet, MD,4 Christo I. Tchervenkov, MD,5 Erle H. Austin, III, MD,6 Christian Pizarro, MD,7 Kamal K. Pourmoghadam, MD,8 Frank G. Scholl, MD,9 Karl F. Welke, MD,10 and Constantine Mavroudis, MD3

Ann Thorac Surg. 10.1016/j.athoracsur.2011.06.008

• RV-PA group was superior to the BT shunt for the

primary endpoint of death or transplant at 12 m, at 32 m this was no longer significant

• The need for ECMO during the Norwood hospitalization was greater in the BT shunt group

• Unintended cardiovascular interventions on the shunt or neoaorta were more common in the RV-PA group

• Echo measures of RV end-DV, EF were superior for the RV-PA group up to Stage II, but had equalized by 14 m

• PA size was larger in the BT shunt group

SVR (Single Ventricle Reconstruction) Trial A Pediatric Heart Network, 15-center, National Institutes of Health–sponsored, randomized trial compared the BT shunt and RV-PA

Mortality Outcome • 5 year survival 70% for infants who undergo stage I repair

• For children who survive to the age of 12 months, long-term survival is

approximately 90%

• Mortality rates associated with stages II and III are low

• 1, 5, and 10 year survival rates of 75, 70, and 65 %, respectively

• HLHS with genetic disorders, prematurity, low birth weight, living in poor neighborhood, ECMO , unplanned reoperation Mortality increases

Improved Survival of Patients Undergoing Palliation of Hypoplastic Left Heart Syndrome: Lessons Learned From 115 Consecutive Patients

James S. Tweddell, MD; George M. Hoffman, MD; Kathleen A. Mussatto, RN; Raymond T. Fedderly, MD; Stuart Berger, MD; Robert D.B. Jaquiss, MD; Nancy Nancy S. Ghanayem, MD; Stephanie J. Frisbee, MSc; S. Bert Litwin, MD Circulation is available at http://www.circulationaha.org DOI: 10.1161/01.cir.0000032878.55215.bd

• Exercise intolerance

• Neurodevelopmental delay

• Growth

• Thrombotic complications

• Fontan Failure • Quality of life

Morbidity Outcome

Neurodevelopment in children with hypoplastic left heart syndrome Anne Sarajuuri, Eero Jokinen, Riina Puosi, Leena Mildh, Ilkka Mattila, Aulikki Lano, Tuula Lönnqvist Journal of Pediatrics 2010, 157 (3): 414-20, 420.e1-4

Electronic Referral Report To KFSH-J Pediatric Cardiology January 2013- January 2017 TOF Single ventricle Heart VSD HLHS AVSD TGA Aortic arch Hypoplasia, Coarctation ASD-secundum Partial AVSD SAM, LVOTO

Hypoplastic Left Heart Syndrome: Experience With Palliative Surgery

WILLIAM 1. NORWOOD, MD, FACC’ JAMES K. KIRKLIN, MD* STEPHEN P. SANDERS, MD+ Boston, Massachusetts

Persistent effort to improve the long-term outlook of neonates with hypoplastic left heart syndrome is to be encouraged for the following reasons: (1) It is the fourth most common congenital cardiac anomaly entering the

New England Regional Infant Cardiac Program (after ventricular septal defect, tetralogy of Fallot and transposition of the great arteries)

(2) Unlike many forms of congenital cardiac disease, the syndrome has a very low incidence rate of associated extracardiac anomalies

Prevalence Report of Congenital Cardiac surgery Operations KFSH-J January 2013- January 2017 Tetralogy repair 104 9.52 % Cavopulmonary anastomosis 101 9.25 % VSD-perimembranous repair 82 7.51 % Fontan operation 81 7.42 % Stage I – Norwood 71 6.50 % AVSD-complete repair 58 5.31 % TGA repair-arterial switch 49 4.49 % Aortic arch repair 31 2.84 % ASD-secundum repair 30 2.75 % Partial AVSD 26 2.38 %

Norwood Procedure 135

BT Shunt

(n=76)

56.3%

RV-PA shunt

(n-=52)

38.5%

Stage II (Glenn) (n=29) 51.3%

Stage II (Glenn) (n=22)

42.3%

Norwood Cavopulmonary

Anastomosis (n=7) 5.2%

EC Fontan (n=2) 3.8%

EC Fontan (n=12) 15.8%

Lost Follow

up (n=12) 15.8%

Early Mortality

(n=17) 22.4%

Lost Follow

up (n=8) 15.4%

Early Mortality

(n=13) 25%

I. S. Mortality

(n=4) 5.3%

I. S. Mortality

(n=3) 5.8%

Annual cases

BT shunt Vs RV-PA Shunt

Diagnosis BT shunt (n=76) RV to PA conduit (n=52) P-value

HLHS 53 46 0.028

AA,MA 15(19.8%) 27(51.9%) 0.001

AS,MA 27(35.5%) 11(21.2%) 0.02

AA,MS 11(14.5%) 8(15.4%) 0.757

Others 23(30.3%) 6(11.5%) 0.02

Diagnosis

BT shunt (n=76) RV to PA conduit (n=52) P-value

Gender (male) 43(56.6%) 30(57.7%) 1.0

Age(days) 47.71±35.28 37.02±32.8 0.163

Weight (Kg) 3.3±0.73 3.03±0.67 0.029

Height (cm) 50.9±7.9 50.03±4.5 0.47

Pre Operative Data

BT shunt (n=76) RV to PA conduit (n=52) P-value

Aortic cross clamp 84.39±30.46 69.12±30.78 0.01

Total bypass time 163.97±54.34 134±76.58 0.02

LOS 56.6±37.18 48.46±37.71 0.659

ICU stay 45.55±33.26 38.46±33.39 0.688

ECMO 14(18.4%) 10(19.2%) 1.0

Early Mortality 17(22.4%) 13(25%) 0.832

Intra – Post Operative Data

BT shunt (n=76) RV to PA conduit (n=52) P-value

Time for Stage II 274.79±107.92 200.24±76.8 0.007

Stage II 29(38.2%) 22(42.3) 0.714

Stage III (EC Fontan) 12(15.8%) 2(3.4%) 0.043

Inter Stage Data

Postoperative

intervention

BT shunt (n=76) RV to PA conduit

(n=52)

P-Value

Aortic arch

intervention

10 (13.2%) 5(9.6%) 0.15

PA stent 2(2.6%)

3(5.8%) 0.12

Shunt stent 5(6.6%) 3(5.8%) 0.78

Atrial septostomy 2(2.6%) 0 0.134

Collateral coiling 2(2.6%) 1(1.9%) 0.421

Aortic arch repair 4(4.2%) 1(1.9%)

0.357

Surgery to the Shunt

2(2.6%) 2(3.8%) 0.561

Recoarctation After Norwood I Procedure for Hypoplastic Left Heart Syndrome: Impact of Patch Material Keti Vitanova, Julie Cleuziou, Jelena Pabst von Ohain, Melchior Burri, Andreas Eicken, Rüdiger Lange Annals of Thoracic Surgery 2017, 103 (2): 617-621

P-Value RV to PA conduit

(n=52)

BT shunt (n=76) variable

0.832 13(25%) 17(22.4%) Early Mortality

0.677 3(5.8%) 𝟒(𝟒. 𝟐%) Interstage Mortality

0.697

16(30.8%)

21(27.6%) Overall mortality

Mortality Data

Predictors of early mortality - Low body weight - Prolonged bypass time and - ECMO

HLHS Evolution 1982 1985 1990 1995 2000 2017

Stage I Norwood

Fontan Modification

Improvements in Intraoperative Support

Improvements in Postoperative Support

Pre natal Diagnosis

PGE Echo ICU

Evolving Strategies Fetal Intervention

Stem Cell Home Monitoring

Conclusion • Norwood operation can be successful with good results that warrant offering

treatment to most newborns with HLHS in KSA

• Survival beyond the neonatal period and into adulthood is possible

• Lack of heart transplantation should not be considered sufficient reasons to deny surgical care to HLHS patients

• The last four decades, from comfort care to long-term survival, should make patients with HLHS, their families, and the physicians who care for them hopeful that outcomes will continue to improve

With all the challenges and obstacles this is the care we should pursue !

THANK YOU

Thank You