Journal
JOURNAL OF HEART AND LUNG TRANSPLANTATION
Volume 31, Issue 5, Pages 509-516Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.healun.2011.12.013
Keywords
pediatric heart transplant; survival; congenital heart disease; mortality risk; hypoplastic left heart syndrome; surgical palliation; cardiomyopathy
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BACKGROUND: Infant heart transplant (HT) recipients have the best long-term survival of any age group, but the small donor pool and high early mortality limit the therapeutic effectiveness. We sought to determine the relationship between pre-HT diagnosis and early HT outcome to better define the mortality risk associated with a diagnosis of congenital heart disease (CHD) and to examine differences between early and current HT eras. METHODS: The Pediatric Heart Transplant Study (PHTS) database was used to identify 739 infant HT recipients at age 6 months between 1993 and 2008 divided into the following etiologic groups: cardiomyopathy (CM), 18%; hypoplastic left heart syndrome (HLHS) without surgery, 41%;HLHS with surgery, 9%; other CHD without surgery, 16%; and other CHD with surgery, 15%. Severity of illness at HT, post-HT survival, and era effects were compared. RESULTS: At 1 year after HT, survival was 89% for the CM group, which was the best, 79% for CHD without surgery, 82% for CHD with surgery, 79% for HLHS without surgery, and 70% for HLHS with surgery, which was the worst outcome. Hazard function analysis demonstrated the difference occurred within the first 3 months after HT. After adjusting for illness severity, differences in mortality risk persisted across etiologic groups. HT survival was similar in the current surgical era for HLHS with surgery, 71% (1993-1998) vs 70% (1999-2008). CONCLUSIONS: Infant HT recipients with different pre-HT diagnoses have significantly different post-HT outcomes. HLHS infants with surgery have the lowest survival and their outcome is unchanged in the current era. J Heart Lung Transplant 2012;31:509-16 (C) 2012 International Society for Heart and Lung Transplantation. All rights reserved.
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