LONG TERM OUTCOMES OF PALLIATIVE CONGENITAL HEART DISEASE

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1 LONG TERM OUTCOMES OF PALLIATIVE CONGENITAL HEART DISEASE S Bruce Greenberg, MD, FACR, FNASCI Professor of Radiology and Pediatrics Arkansas Children's Hospital University of Arkansas for Medical Sciences

2 Disclosures: None

3 Outline Incidence of complex congenital heart disease in adults Evolution of palliative procedures and physiology Long term outcomes and future concerns

4 Incidence of congenital heart disease Hoffman JIE and Kaplan S. The incidence of congenital heart disease. JACC2002; 39:

5 Worldwide increasing prevalence ACHD Marelli et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation Shiina et al. Prevalence of adult patients with congenital heart disease in Japan. International Journal of Cardiology

6 Adult CHD beyond age 60 Number of patients 60 years of age under follow-up. (A) Overview over the whole cohort. (B) Relative number of patients stratified by the complexity of congenital heart defect (year 2000 is indexed as 100%). There was a 12-fold increase of patients with simple defects, and a six- to seven-fold increase in patients with moderate severe complexity. Tutarel O, et al. Congenital heart disease beyond the age of 60: emergence of anew population with high resource utilization, high morbidity, and high mortality. European Heart Journal 2014: 35:

7 CHD prevalence in USA 2010 Total 2.4 million Adults 1.4 million Adults between 2000 and % increase 85% increase with severe congenital heart disease Gilboa SM, Devine OJ, Kucik JE, et al. Congenital heart defects in the United States. Estimating the magnitude of the affected population in Circulation 2016; 134:

8 Palliation for single ventricle physiology Fontan Tricuspid atresia Norwood Hypoplastic left heart syndrome

9 Anomalies palliated by Fontan Morphology Range Percent Tricuspid atresia , 22, 31, 16 Hypoplastic left heart syndrome , 21, 5, 40 Double inlet left ventricle , 15, 22, 19 Double outlet right ventricle 8 8, 8, X, X Heterotaxy/discordant AV connections 6-8 6, 8, X, 7 Unbalanced AVC 4-6 6, 4, X, 6 Other , 23, 42, 11 Dabal RJ, Kirklin JK, Kukreja M, et al. The modern Fontan operation show no increase in mortality out to 20 years: a new paradigm. J Thorac Cardiovasc Surg 2014; 148: Anderson PAW, Sleeper LA, Mahony L, et al. Comtemporary outcomes after the Fontan procedure. JACC 2008; 52: Khairy P, Fernandes SM, Mayer JE, et al. Long-term survival, modes of death, and predictors of mortality inpatients with Fontan Surgery. Circulation 2008; 117: Tweddell JS, Neresian M, Mussatto KA, Nugent M, et al. Fontan palliation in the modern era: factors Impacting mortality and morbidity. Ann Thorac Surg 2009; 88:

10 Fontan growth in the next decade 2.3: 10,000 live births evaluated for Fontan Adults 60% increase in Fontan patients NYHA functional class III expected to double Van Melle JP, Wolff D, Horer J, et al. Surgical options after Fontan failure. Heart 2016; 102:

11 Fontan mortality accelerates in 3 rd decade of life

12 Evolution of the Fontan Atrio-pulmonary connection Total cavo-pulmonary connection

13 Fontan mortality (operations ) Boston: 262 patient with follow up mean 12.2 years Mortality 29% Transplant, take down or revision 12% Late attrition in early survivors Sudden death (probable arrhythmia origin) Thromboembolic Heart failure Khairy P, Fernandes SM, Mayer JE, et al. Long-term survival, modes of death, and predictors of mortality inpatients with Fontan Surgery. Circulation 2008; 117:

14 Fontan survival (operations ) Milwaukee: 256 Survival at 10 year follow up 94% Event free survival at 10 years 64% Events include Death, heart transplant, Fontan take down Heart failure (NYHA class III-IV) Pacemaker, antiarrhythmic medication Protein-losing enteropathy, stroke, thrombus Tweddell JS, Neresian M, Mussatto KA, Nugent M, et al. Fontan palliation in the modern era: factors Impacting mortality and morbidity. Ann Thorac Surg 2009; 88:

15 Fontan survival (operations ) Birmingham: 207 Survival at 10 year follow up 88% % % Survival at 20 year follow up 76% Dabal RJ, Kirklin JK, Kukreja M, et al. The modern Fontan operation show no increase in mortality out to 20 years: a new paradigm. J Thorac Cardiovasc Surg 2014; 148:

16 Fontan survival (operations ) Dabal RJ, Kirklin JK, Kukreja M, et al. The modern Fontan operation show no increase in mortality out to 20 years: a new paradigm. J Thorac Cardiovasc Surg 2014; 148:

17 Fontan mortality accelerates in 3 rd decade of life

18 40 th Anniversary of Fontan palliation

19 Suboptimal physiology Elevated central venous pressure Hepatic congestion Splanchnic congestion Reduced cardiac output Increased pulmonary resistance from absent pulsatile flow Reduced pre-load Abnormal ventricle anatomy

20 Increased pulmonary vascular resistance Non-pulsatile pulmonary flow Remodeling of intra-acinar vessels Intima: acellular thickening Media: apoptosis of smooth muscle Lumen narrowing Inability to vasodilate Ridderbos F-JS, Wolff D, Timmer A, et al. Adverse pulmonary vascular remodeling in the Fontan circulation. J Heart Lung Transplant 2015; 34:

21 Systemic to pulmonary vein collaterals Adults with Fontan palliations and angiograms 58% systemic to pulmonary vein collaterals Source of collateral blood flow Right brachiocephalic vein 35% Azygous vein 20% Superior vena cava 13% Inferior vena cava 10% Other 22% 19 y.o.with atrio-pulmonary Fontan & systemic vein to pulmonary vein collateral Lluri G, Levi DS, Aboulhosn J. Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation. Intern J Cardiology 2015; 189:

22 Fontan and gastrointestinal vulnerability Elevated systemic venous pressure Low pressure gradient from portal vein to hepatic veins Chronic venous congestion Sinusoidal dilation, atrophy and necrosis Cirrhosis, regenerating nodules, hepatocellular carcinoma 19 year old with heterotaxy & cirrhosis Yoo SJ, Prsa M, Schantz D, et al. MR assessment of abdominal circulation in Fontan physiology. Int J Cardiovasc Imaging 2014; 30:

23 Protein losing enteropathy Loss of protein at the intestinal mucosa Mechanism hypothesis Chronic low cardiac output vascular redistribution away from gut Systemic venous hypertension vascular congestion Release of inflammatory mediators Breakdown of bowel mucosa Similar mechanism may be responsible for plastic bronchitis Rychik J. Forty years of the Fontan operation: a failed strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2010; 13:

24 Late attrition after Fontan Decline in exercise tolerance Heart failure Arrhythmias Thromboembolic complications All are worse with atrio-pulmonary Fontan palliations Atrio-pulmonary Fontan with dilated right atrium

25 Treatment failing Fontan Fontan take down Fontan revision Heart transplant

26 Fontan take down Usually a bail out in early Fontan failure Reduced due to better patient selection Mortality 30 days post take down 26% Mean follow-up 5.9 yrs 40% Mortality + heart tx 45% Van Melle JP, Wolff D, Horer J, et al. Surgical options after Fontan failure. Heart 2016; 102:

27 Fontan revision & Heart transplant Late Fontan failure No difference in mortality Mortality Fontan Conversion 30 days post conversion 11% Mean follow-up 7.7 yrs 22% Mortality + heart tx 26% Van Melle JP, Wolff D, Horer J, et al. Surgical options after Fontan failure. Heart 2016; 102:

28 Hypoplastic left heart syndrome Norwood procedure: conversion of right ventricle to systemic ventricle by connecting pulmonary artery root and native aorta Bi-direction Glenn palliation Fontan palliation HLHS: Norwood (left) and extracardiac Fontan (right)

29 Long term outcome of HLHS palliation 70% survive to adulthood No long term data on adult mortality Fontan mortality Adults 8.2% over 5 years Univentricular heart patient mean age of death < 30 years Feinstein JA, Benson W, Dubin AM, et al. Hypoplastic left heart syndrome. Current considerations and expectations. JACC 2012; 59: S1-S42.

30 Hypoplastic left heart syndrome morbidity Known risk of Fontan Additional concerns Heart failure from absent left ventricle Neoaortic root abnormalities Aortic arch repair abnormalities Feinstein JA, Benson W, Dubin AM, et al. Hypoplastic left heart syndrome. Current considerations and expectations. JACC 2012; 59: S1-S42.

31 Conclusion adults with complex congenital heart disease Palliation results of Fontan and Norwood procedures Short and intermediate results are good Long term results are indeterminate Multiple organ systems require life-long follow up

S. Bruce Greenberg, MD FNASCI and President, NASCI Professor of Radiology and Pediatrics University of Arkansas for Medical Sciences

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