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Pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries in a young adult

Pulmonary atresia with ventricular septal defect (PA-VSD) and pulmonary blood supply arising from the aorta in the form of major aortopulmonary collateral circulation (MAPCAs) surviving in the adulthood is a rare occurrence. It is usually fatal in the infancy without surgical repair. We report the case of a 25-year-old female with clinical and imaging findings of PA-VSD. She had previous episodes of cyanosis and exertional breathlessness which she could tolerate without much hampering of day-to-day activity. The current presentation was due to symptoms of bacterial infection which led to the diagnosis of the underlying complex cardiovascular malformation.