The incidence of congenital bicuspid or bileaflet and quadricuspid or quadrileaflet arterial valves in 3,861 donor hearts in the European Homograft Bank.

BACKGROUND AND AIM OF THE STUDY: The incidence of bicuspidity and quadricuspidity of arterial valves in donor hearts has been assessed in the European Homograft Bank.
METHODS: Morphologically unacceptable donor valves for clinical use were assessed for cusp or leaflet malformation, identifying the different valve types. The state of the adjacent valve was also evaluated. Histological evaluations of the corresponding conduits were performed, in order to assess the incidence of media degeneration.
RESULTS: Among 3,861 donor hearts, 39 (1.0%) showed congenital cusp or leaflet malformation of one or both arterial valves, 28 (0.7%) showed bicuspid or bileaflet aortic valves (BCAV), four (0.1%) showed bicuspid or bileaflet pulmonary valves (BCPV), and eight (0.2%) showed quadricuspid or quadrileaflet pulmonary valves (QCPV). Only in one case (0.03%) were both arterial valves bicuspid or bileaflet. Among the bicuspid arterial valves, 27 (84%) were isolated aortic valves, three (9%) were isolated pulmonary valves, and in one case (3%) both the aortic and pulmonary valves were bicuspid or bileaflet.
CONCLUSION: The most frequently identified congenital malformation of the arterial valves was aortic bicuspidity, followed by pulmonary quadricuspidity and bicuspidity. If asymptomatic, these were detected during routine echocardiography, or at autopsy. These malformations might be genetically determined, as they are detected more often among different members of the same families, and in those persons of the same gender or blood group. These valves have a predisposition to accelerated calcification, stenosis, endocarditis and dissection of the ascending aorta. Neither quadricuspid/quadrileaflet nor unicuspid/monoleaflet aortic valves were detected in this study.