Paediatric Imaging

Pulmonary atresia

a rare congenital anomaly representing less than 1% of all congenital heart disease. The newborn presents with severe cyanosis at birth.

80% of patients with this condition have a pulmonary valve that is atretic with a diaphragm-like membrane. The infundibulum is atretic in 20% of patients. The pulmonary artery trunk is rarely atretic. The ventricular septum is intact. The right ventricle cavity is always small and often minute.

On a chest radiograph the heart size is either normal or large from the right atrial enlargement. The pulmonary vascular markings are decreased with clear lung fields.

Echocardiography is diagnostic with the demonstration of thickened, immobile, atretic pulmonary valve with no Doppler evidence of blood flow through it. A right to left atrial shunt, when present, is demonstrated with colour-flow and Doppler studies.

LT