Congenital isolated absence of a single cusp of pulmonary valve.

Abstract

A 25-year-old, previously asymptomatic female, presented to the outpatient clinic with episodic palpitations for past 6 months. She was acyanotic and showed no peripheral stigmata of infective endocarditis. Transthoracic echocardiography revealed dilated right ventricle with severe low-pressure pulmonary regurgitation. A cardiac computed tomography angiography performed for evaluation of pulmonary arterial circulation and intracardiac anatomy revealed isolated absence of posterior pulmonary cusp. The two other (right anterior and left anterior) cusps were normal and covered only part of the valve orifice, resulting in pulmonary insufficiency. The main pulmonary artery showed asymmetric dilatation. No other structural heart defects were noted.