277RESULTS OF HEART TRANSPLANTATION FOLLOWING FAILED STAGED PALLIATION OF HYPOPLASTIC LEFT HEART SYNDROME AND RELATED SINGLE VENTRICLE ANOMALIES

• Published in: Interactive cardiovascular and thoracic surgery Vol. 19; no. suppl_1; p. S83
• Main Authors: Deshpande, S., Alsoufi, B., Kogon, B., Mahle, W., Maher, K., Kanter, K.
• Format: Journal Article
• Language: English
• Published: Oxford University Press 01-10-2014
• ISSN: 1569-9293; 1569-9285
• DOI: 10.1093/icvts/ivu276.277

Objectives: Multistage palliation is the mainstay management strategy of hypoplastic left heart syndrome (HLHS). If this palliation strategy fails, heart transplantation (HT) is required. HT results in those children are reportedly poor due to immunologic, clinical and anatomic risk factors. We report our institutional outcomes following HT in previously palliated HLHS patients.
Methods: From 1994 to 2013, 253 children underwent HT, including 105 with congenital heart disease (CHD). We analysed outcomes in our patient cohort of previously palliated HLHS and variants (n = 23).
Results: There were 14 males. Underlying anomaly was HLHS (n = 21), other (n = 2). HT was performed following Norwood (n = 3), Glenn (n = 16), Fontan (n = 4). Median age at transplantation was 2.8 years (0.6–16.8) and median HT listing duration was 61 days (1–554). Nine patients (39%) had panel reactive antibody >10%. Hospital mortality was 4% while 1- and 8-year survival was 83% and 51% compared to hospital mortality for other HT recipients with CHD of 4% and 1- and 8-year survival of 80% and 54% (P = 0.95). Survival was 67%, 66% and 50% when HT was done following Norwood, Glenn and Fontan, respectively (P = 0.88). During follow-up, 3/23 patients required re-transplantation. The only factor on multivariable analysis affecting survival was later era >2004 (5-year survival 100% vs 42%, P = 0.01).
Conclusion: Children failing multistage palliation of HLHS often require HT, mainly following Glenn operation. HT results in this group are comparable to those in other CHD patients. Improvements in pre-transplant management, immune-suppression and outpatient care in the later era might have specifically benefited this particularly risky group of patients.