Surgical Palliation or Primary Transplantation for Aortic Valve Atresia

Published on Apr 1, 2020in The Journal of Thoracic and Cardiovascular Surgery5.261
· DOI :10.1016/j.jtcvs.2019.08.104
Kathryn A. Stackhouse4
Estimated H-index: 4
(BIDMC: Beth Israel Deaconess Medical Center),
Brian W. McCrindle88
Estimated H-index: 88
+ 7 AuthorsGösta B. Pettersson42
Estimated H-index: 42
(Cleveland Clinic)
Abstract Objectives To describe surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with either surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life. Methods From 1994–2000, 565 neonates with aortic atresia were admitted to 26 Congenital Heart Surgeons’ Society hospitals and followed annually for vital status. Initial management included surgical palliation (n=453) and primary cardiac transplantation (n=68). PedsQL health-related quality-of-life questionnaires were sent cross-sectionally to a subgroup of 198 patients alive at previous follow-up, with 80 responses. Results Risk of death was initially high for both treatment strategies. However, compared with initial surgical palliation, survival with primary transplantation, including waitlist mortality, was greater and persisted long-term (65% vs 40% at 15 years; P=.002). Survival after secondary transplantation (48% at 9 years) was lower than after primary, 74%. Health-related quality-of-life total score was lower overall than that of the general adolescent population (71±16 vs 84±13; P=.0001; normal=100), but similar to that of adolescents with chronic diseases. It was similar in the surgical palliation and primary transplantation groups (70±16 vs 75±15; P=.3). Surgical palliation patients reported more symptoms (76±15 vs 63±18; P=.02). Conclusions Patients receiving primary heart transplantation for aortic atresia in 1994–2000 experienced better survival, fewer symptoms, and equivalent quality of life compared with those undergoing initial surgical palliation. Notwithstanding limited availability of neonatal and infant donor hearts, primary transplantation may be considered for those neonates with risk factors predictive of exceptionally poor survival after surgical palliation.
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