Abstract

Purpose Over the past 3 decades, palliation of children with congenital heart disease (CHD) has been increasingly successful. This has created an ever-growing cohort of adolescents and young adults with congenital heart disease who unfortunately develop heart failure. Mechanically supporting such patients is possible, however, patients who have residual lesions or require multiple procedures (i.e. conduit exchange, valve repair) in order to place a ventricular assist device have a higher risk of morbidity and mortality. In complex patients, the use of the SynCardia total artificial heart (TAH) to avoid multiple procedures may be beneficial. However, the success of the TAH in the CHD cohort has not been reported. Methods and Materials All patients with congenital heart disease who had the SynCardia TAH implanted from 1986 to 2012 were identified in the Worldwide Syncardia TAH database, which contains 1091 patients. Results TAH was implanted in 24 patients with CHD (2.2% of total cohort). 25% (6/24) of the CHD patients were adolescents (12-18yo). These TAHs were implanted in 16 institutions around the world [US(9), EU(5) and Canada(2)]. Median age was 28 years [13- 56] and 25% were female. Total days of support were 1476 days with a median of 24 [1-359] days. Overall survival was 62% with a 100% survival in adolescents with CDH. Mortalities occurred in the 1st month of support in 89% (8/9) of cases. Cause of death was multisystem organ failure in 5, pulmonary hemorrhage in 1, not reported in 3. Competing outcomes analysis at 2 months demonstrates 67% of patients had a positive outcome: 34% were still on the TAH, 33% were transplanted, and 33% died. Conclusions Although a challenging cohort, the majority of these complex patients were successfully supported, particularly adolescents with congenital heart disease.

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