Isolated pulmonary valvular stenosis with equilateral triangle orifice

Abstract

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.>.

Keywords: Cardiac computed tomography; Pulmonary valvular stenosis; Triangle orifice.

Fig. 1

Chest radiography demonstrated that the pulmonary artery was dilated markedly (A). Echocardiography showed the high velocity jet across the pulmonary valve and turbulent flow in the pulmonary artery (B). Enhanced computed tomography showed the thickened valve and the secondary dilatation of the main pulmonary artery (C), and the triangle orifice of pulmonary valve seen from the arterial aspect in 3-dimensional view (D). PA, pulmonary artery; RV, right ventricle.

Fig. 2

The systolic phase (upper panels) and the diastolic phase (lower panels) of pulmonary valve on electrocardiogram-gated cardiac computed tomography, with 3-dimensional schema. The long-axis view showed a dome-shaped opening (A), and a closure (B). The dotted line indicates coaptation zone level (C in A, D in B), and the broken line indicates sinotubular (ST) junction level (E in A, F in B). At the coaptation zone level, tricuspid semilunar valves opened excessively (arrows in C), and three leaflets closed (D). At the ST junction level, the triangle was surrounded by a straight line to connect each commissure, fixed through the cardiac cycle (E, F). The schema of pulmonary valve has rendering systolic phase (G), and diastolic phase (H). Inset panel shows schematics of coronal cut end at the section indicated by black solid line (rectangle).