pulmonary atresia with intact ventricular septum

Last reviewed 01/2018

In this condition the pulmonary valve leaflets are completely fused and the right ventricular outflow tract is atretic. Consequently right atrial blood is shunted right to left through the foramen ovale and thence to the systemic circulation. Pulmonary blood flow occurs via a patent ductus arteriosus.

In patients with a small right ventricle there are often sinusoidal channels within the right ventricle which communicate with the coronary circulation whilst in patients with medium or large ventricular cavities there is often tricuspid insufficiency.