Isolated pulmonary valvular stenosis with equilateral triangle orifice.

Isolated congenital pulmonary valvular stenosis is a relatively common abnormality. A 52-year-old woman with an asymptomatic cardiac murmur since her childhood visited our hospital. The peak pressure gradient across the pulmonary valve (PV) was calculated 25 mmHg by Doppler velocity during the transthoracic echocardiography, the severity was graded into mild. The cardiac computed tomography indicated a dome-shaped PV with an equilateral triangle orifice without commissural fusion. No case has been described in a triangle orifice, it will be necessary to follow up carefully. < Learning objective: Here we report the first case of pulmonary valvular stenosis with an equilateral triangle orifice without commissural fusion of pulmonary valve, which is speculated to be an embryonic remnant during semilunar leaflet formation.>.