Prolapse of atrioventricular valve leaflets in the setting of double orifice

Prolapse of the valve leaflets is reported in 2 cases in the setting of double orifice atrioventricular valve. Diagnosis in the first case, an asymptomatic 8-year-old female, was achieved by echocardiography and consisted in prolapsing leaflets of a double orifice ("bridge type") mitral valve. The second patient, a 24-year-old male, died suddenly. Postmortem examination revealed prolapsing atrioventricular valves, with double orifice ("holy type") of the septal leaflet of the tricuspid valve. There was disruption of the penetrating atrioventricular bundle into multiple fragments with fasciculo-ventricular by-pass fibers.