Right Ventricular Outflow Tract:

Abstract

Absent pulmonary valve syndrome is a rare congenital heart disease with absent or rudimentary pulmonary valve leaflets, severe pulmonary regurgitation, and dilatation of the main and branch pulmonary arteries leading to airway compression. Neonates with absent pulmonary valve syndrome develop a varying degree of respiratory distress depending on the degree of airway compression with 40% of the neonates exhibiting respiratory distress at birth. The first-line diagnostic imaging modality for absent pulmonary valve syndrome is transthoracic echocardiogram. However, echocardiography has a limited role in evaluating airways. Cardiac CT provides useful information about the airway compression by dilated or aneurysmal pulmonary arteries. Multidetector CT scan obtains isotropic volume data and allows production of high-quality two-dimensional and three-dimensional multiplanar reformatted images showing degree and location of the airway compression. Surgical repair includes patch closure of the ventricular septal defect and right ventricular to pulmonary artery conduit placement with plication of dilated pulmonary arteries.