Isolated pulmonary supravalvular stenosis accompanied by pulmonary artery aneurysm

We present a 19-year-old asymptomatic female patient in whom isolated pulmonary supravalvular stenosis and pulmonary artery aneurysm were incidentally detected. On cardiologic examination, a systolic murmur was heard over the lungs, right axis deviation was seen on the electrocardiogram, and marked appearance of the pulmonary artery was noted on the chest X-ray. On transthoracic echocardiography, the pulmonary artery trunk was found to be wider than normal, and there were mild pulmonary and tricuspid regurgitation. Color Doppler examination showed a turbulent flow 2 cm distal to the pulmonary valve. Transesophageal echocardiography showed a supravalvular membranous structure and a proximal pulmonary artery aneurysm. The pulmonary artery trunk was measured as 40 mm at the widest part. Thoracic computed tomography revealed that the proximal pulmonary artery aneurysm was localized to the pulmonary artery trunk and measured 41 mm. As the patient was asymptomatic and there were no signs of aneurysmatic compression or a left-to-right shunt, and due to the presence of a relatively low gradient (16 mmHg) across the stenotic area, she was scheduled to regular radiologic control.