Abstract

<h3>Introduction</h3> Valvular disease in pediatric donor hearts may be a relative contraindication to graft use. Outcomes following use of donor hearts with milder forms of valvular disease have not been previously reported. We describe 4 cases of pediatric heart transplantation (HTx) in which the donor heart had a bicuspid aortic valve (BAV). <h3>Case Report</h3> Of the 469 HTx performed at our center since 1985, 4 donor hearts had a BAV. All recipients were female; median age 11 years (range 0.3 to 19 years). In all cases, the BAV was not discovered until after HTx. All donors were less than 30 years old. The patients were followed for a median of 6 years (range 2 to 9 years) with all patients alive at last follow up. Two patients transferred to adult care and 2 patients are followed by our clinic. In follow up, no patient required an aortic valve intervention or had infective endocarditis. At last review, no patient had greater than mild aortic insufficiency or more than mild aortic stenosis. Three patients developed mild to moderate left ventricular hypertrophy in the first year post-transplant that improved over time. One patient experienced a peri-operative embolic stroke at time of transplant unrelated to the bicuspid aortic valve. <h3>Summary</h3> On short- and intermediate-term follow up, donor hearts with bicuspid aortic valve demonstrated acceptable graft longevity and valvular function. A functionally normal bicuspid aortic valve in a pediatric heart transplant donor should not be a contraindication to organ acceptance.

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