SHA24/043004

Heart Transplantation in Congenital Heart Disease Kirk R. Kanter MD Professor of Surgery Chief, Pediatric Cardiac Surgery Emory University School of Medicine Children’s Healthcare of Atlanta Atlanta, Georgia

History • First human-to-human heart transplant in December, 1967 • First transplant for congenital heart disease also in December, 1967 • Increasing success with surgical management of congenital heart disease • Seemingly irreducible numbers of “repaired” patients who will need heart transplantation

NUMBER OF HEART TRANSPLANTS

BY YEAR

ISHLT

2012

NOTE: This figure includes only the heart transplants that are reported to the ISHLT Transplant Registry. As such, the presented data may not mirror the changes in the number of heart transplants performed worldwide .

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

DIAGNOSIS IN ADULT HEART TRANSPLANTS

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: < 1 Year)

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: 1-10 Years)

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

DIAGNOSIS IN PEDIATRIC HEART TRANSPLANT RECIPIENTS (Age: 11-17 Years)

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

ADULT HEART TRANSPLANTS Donor and Recipient Characteristics 1992-2000 (N = 36,507)

2001-2005 (N = 16,352)

2006-6/2011 (N = 17,868)

Cardiomyopathy

46.4%

48.5%

53.8%

Coronary artery disease

45.8%

42.6%

37.1%

Valvular

3.8%

3.3%

2.6%

Retransplant

1.9%

2.2%

2.6%

Congenital

1.8%

2.7%

3.0%

Other causes

0.4%

0.6%

0.9%

Head trauma

44.6%

54.5%

46.6%

Stroke

28.2%

33.0%

24.8%

Other

27.2%

12.5%

28.6%

p-value

Diagnosis

<0.0001

Donor cause of death

ISHLT

(Contâ&#x20AC;&#x2122;d)

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

<0.0001

Early Risk Factors • PVR calculation can be problematic • Comorbidities (PLE, hepatic or renal dysfunction) - Cardiac cirrhosis, hepatitis C

• Elevated PRA due to prior blood product exposure • Postop issues (bleeding, AP collaterals, debilitation, pulmonary hypertension) • Technically challenging operation

Technical Considerations • Obtain adequate donor tissue (arteries, veins) • Match donor to recipient - Avoid marginal donor - Consider ischemic time - ?Oversize donor?

• Protect RV (collaterals, venting, PVR calculations) • Beware of pre-sensitization • Meticulous technique (abnormal coags, collaterals)

Transposition of the Great Arteries s/p Arterial Switch Procedure

Congenitally Corrected TGA

Obtain Extra Donor Vessels

Prior Fontan

Situs Inversus, Interrupted IVC with Azygos Continuation to LSVC

Use right atrial cuff to form IVC channel

Open Left Pericardium Donor aorta for SVC extension

Separate pulmonary vein anastomoses IVC Channel

Completed Transplant

ADULT HEART TRANSPLANTS Kaplan-Meier Survival by Diagnosis (Transplants: January 1982 - June 2010)

HALF-LIFE Cardiomyopathy: 11.4 years; CAD: 9.4 years; Congenital: 13.7 years; Retransplant: 6.0 years; Valvular: 10.9 years

All pair-wise comparisons are statistically significant at < 0.001 except cardiomyopathy vs. congenital (p=0.6340).

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

ADULT HEART TRANSPLANTS Kaplan-Meier Survival by Diagnosis Conditional on Survival to 1 Year (Transplants: January 1982 - June 2010) HALF-LIFE Cardiomyopathy: 14.0 years; CAD: 11.7 years; Congenital: 20.4 years; Retransplant: 10.7 years; Valvular: 14.3 years

All pair-wise comparisons are significant at p < 0.05 except cardiomyopathy vs. valvular

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

ADULT HEART TRANSPLANTS Kaplan-Meier Survival by Era (Transplants: January 1982 - June 2010) Diagnosis: Congenital No comparisons are significant at < 0.05 except 1993-2002 vs. 2003-6/2010: p = 0.0489

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

ADULT HEART TRANSPLANTS Kaplan-Meier Survival by Diagnosis Conditional on Survival to 1 Year (Transplants: January 2003 â&#x20AC;&#x201C; June 2010)

No pair-wise comparisons are significant at < 0.05 except cardiomyopathy vs. CAD, cardiomyopathy vs. retransplant, CAD vs. congenital and congenital vs. retransplant

ISHLT

2012

J Heart Lung Transplant. 2012 Oct; 31(10): 1045-1095

Combined CTRD & PHTS Study

Lamour et al, JACC, 2009

Combined CTRD & PHTS Study (1990-2002) • 7,345 tx from Cardiac Transplant Registry Database (CTRD) >18 yo at listing • 121 (1.6%) with congenital heart disease • 923 tx from Pediatric Heart Transplant Study (PHTS)>6 mos and <18 yo at listing • 367 (40%) with congenital heart disease • Total of 488 patients transplanted with congenital heart disease

Diagnoses

Lamour et al, JACC, 2009

Last Major Operation

Lamour et al, JACC, 2009

Overall Survival

Lamour et al, JACC, 2009

Conditional Survival

Lamour et al, JACC, 2009

Multivariable Risk Factors

Lamour et al, JACC, 2009

Effect of Prior Fontan on Survival

Lamour et al, JACC, 2009

Conclusions • Congenital heart disease is a risk factor with cardiac transplantation • Most of the risk is early - Maybe better long-term survival (younger patients?)

• Prior Fontan is a definite risk • Older age at transplant also an early risk factor - Should we transplant sooner?

Patient Survival Fontan

Non-Fontan

1.0

Freedom from Death

P=.4975

0.8

0.6

PATIENTS AT RISK 0 Year 1 3 27 21 17 Fontan Non-Fontan 163 124 97

0.4

5 16 74

0.2

0.0 0

1

2

Years

3

4

5

Observations • Children with a Fontan procedure undergoing heart tx have more complicated operations and longer hospitalizations • Very early rejection is more common • Early and intermediate survival is similar • Ongoing risk of death and retransplantation

Conclusions • Heart transplantation in children after the Fontan procedure can be performed with comparable results to non-Fontan patients • Be careful—there are a lot of Fontan patients out there!!

Future Directions • Improved surgical strategies • Improved medical therapy • Minimize transplant risk - Patient selection - Timing of transplant

• Mechanical circulatory support

Mechanical Support of the Failing Fontan

Rodefeld et al, JTCVS, 2010

Mechanical Support of the Failing Fontan

Rodefeld et al, JTCVS, 2010