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Updated: Mar 9, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Increasing Complexity of Heart Transplantation in Patients With Congenital Heart Disease
William Y Shi1, Pankaj Saxena2, Matthew S Yong2
1Department of Cardiac Surgery, Royal Childrens Hospital, University of Melbourne, Melbourne, Australia; Department of Cardiology, Royal Children's Hospital, University of Melbourne, Melbourne, Australia.
Insights
Heart transplantation for congenital heart disease (CHD) patients is increasing. Recent recipients are more complex, requiring longer surgeries and advanced support, but long-term survival remains comparable to non-CHD patients.
Area of Science:
- Cardiology
- Cardiac Surgery
- Transplantation Medicine
Background:
- Improved surgical outcomes have led to a growing population of patients with repaired congenital heart disease (CHD) requiring heart transplantation.
- This study reviews the experience and outcomes of heart transplantation in patients with CHD.
Purpose of the Study:
- To evaluate the outcomes of heart transplantation in patients with congenital heart disease (CHD).
- To compare outcomes between early and late eras of transplantation (1988-1999 vs. 2000-2014).
Main Methods:
- Retrospective review of 77 patients with CHD who underwent heart transplantation between 1988 and 2014.
- Comparison of outcomes between two distinct eras: early (1988-1999) and late (2000-2014).
Main Results:
- Patients in the later era had longer bypass and ischemic times, and more complex vascular reconstructions.
- Despite increased complexity and need for postoperative extracorporeal membrane oxygenation, in-hospital mortality did not differ between eras.
- Patients with prior univentricular palliation in the later era showed increased postoperative renal impairment.
Conclusions:
- Congenital heart disease patients undergoing heart transplantation in the recent era are more complex, with greater prior surgical history and intraoperative challenges.
- While 30-day mortality was higher in CHD patients, 10-year survival rates were similar to non-CHD recipients.
- Increased intraoperative complexity and postoperative support needs highlight the evolving challenges in this patient population.
Abstract:
Owing to improved surgical results, there is a growing population of patients with repaired congenital heart disease (CHD) requiring heart transplantation. The objective of the study was to review our experience in these patients. A retrospective review of the outcomes of heart transplantation in patients with CHD (n = 77) between 1988 and 2014 was performed. Outcomes of early (1988-1999) and late (2000-2014) eras were compared. In results, the mean age was 18 ± 14 years (range: 16 days-58 years). Seventy (91%) patients underwent a mean of 2.6 ± 1.3 (range: 1-6) cardiac operations before transplantation, whereas 7 were primary transplants. Univentricular palliation had been performed in 44 (57%) patients. Patients with CHD in the later era had longer mean cardiopulmonary bypass time (early: 190 ± 70 minute vs late: 271 ± 115 minute; P < 0.001), ischemic times (early: 222 ± 98 minute vs late: 275 ± 102 minute; P = 0.039), and more often required reconstruction of the great arteries at the time of transplantation (8% vs 28%; P = 0.036). In those with prior univentricular palliations, the ratio of ischemic to cardiopulmonary bypass time decreased in the later era (early: 1.41 ± 0.60 vs late: 0.99 ± 0.37; P = 0.016), reflecting increased intraoperative complexity. Following transplantation, hospital mortality was 13% (10/77; 7 due to primary graft failure). There was no difference in inhospital mortality between the 2 eras (P = 0.52); however, patients in the later era more often required postoperative extracorporeal membrane oxygenation (early: 8%, 3/38 vs late: 28%, 11/39; P = 0.036). In patients with prior univentricular palliations, those in the late era were more likely to experience postoperative renal impairment (early: 1/21, 5% vs late: 9/23, 39%; P = 0.01). Patients with CHD had higher 30-day mortality (CHD: n = 8, 10% vs non-CHD: n = 17, 3.8%; P = 0.021), but similar survival at 10 years (67% ± 12% vs 70% ± 4.7%; P = 0.87) compared to those without CHD. In conclusion, patients with CHD undergoing cardiac transplantation in the recent era were more complex. They had a greater number of prior cardiac operations, and more often underwent complex vascular reconstructions and required more prolonged intraoperative preparation.
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