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