Abstract

Introduction: Aortic valve replacement (AVR) is required in patients with congenital heart disease who are not candidates for Ross procedure or valve repair. The long-term outcomes of prosthetic AVR in patients with congenital heart disease are unknown. Methods: A single-institution, retrospective review of all patients treated with mechanical (M) and bioprosthetic (BP) AVR between 12/1985 to 2/2020 was undertaken. Results: One-hundred and ninety-three patients underwent BP (n=134) or M (n=59) AVR. Indications for AVR were insufficiency (91/193), stenosis (61/194), mixed (38/193), aneurysm (2/193), and unknown (1/193). Patients receiving M valves were more likely to be younger (18.5 years versus 29.0 years, p < 0.01) and less likely to require subsequent valve reintervention (p < 0.01). Average time to first reintervention was 76 and 71 months in the BP and M groups respectively (p = 0.785). Survival between the M and BP groups was similar (p = 0.120). Twenty-four patients who received a M valve and thirteen patients who received a BP valve died during the study period. Causes of death were cardiac (16/37), stroke (3/37), non-cardiac (4/37), and unknown (14/37). Conclusions: Long-term freedom from death or valve reintervention is poor in congenital heart disease patients requiring AVR. Survival is similar between patients treated with M and BP valves, however, BP valves more frequently required reintervention during the study period. Both BP and M valves are associated with significant long-term mortality. These results highlight the need for the development of robust aortic valve repair techniques so that AVR can be avoided.

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