Pulmonary atresia

Case contributed by Vincent Tatco
Diagnosis certain


This is a diagnosed case of pulmonary atresia with ventricular septal defect. CT scan was done to assess the status of the pulmonary arteries.

Patient Data

Age: 3
Gender: Male

CT scan shows small but confluent pulmonary arteries, which exhibit a seagull appearance. Dextrocardia and right-sided aorta are also demonstrated.

Case Discussion

Pulmonary atresia with ventricular septal defect (PA-VSD) is a congenital cardiac malformation in which there is lack of continuity between the ventricle and the pulmonary arteries associated with ventricular septal defect. The blood supply to the lungs originates from extracardiac sources, most commonly from a patent ductus arteriosus (PDA) or from major aortopulmonary collateral arteries (MAPCAs).

The most important aspect of cross-sectional imaging in evaluating patients with PA-VSD is the analysis of the pulmonary artery anatomy. Images should be carefully analyzed for the following:

  • length of pulmonary atresia
  • presence of pulmonary artery confluence
  • size of main, right, and left pulmonary arteries at the origin and at the hilum
  • presence of branch pulmonary artery stenosis
  • sources of pulmonary blood flow to each lung, including the number of bronchopulmonary segments supplied by native pulmonary arteries and the distribution of each MAPCA

On computed tomography (CT), the confluent pulmonary arteries, together with the abbreviated main pulmonary trunk, appear as a seagull in flight.

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