CLINICAL PRESENTATION: This report describes a case of a 24-year-old, acyanotic, female patient presenting with mild effort dyspnea, a right ventricular heave, a displaced cardiac apex, a loud single S2, a holosystolic murmur on the left parasternal area, and a continuous murmur at the left posterior chest. Transthoracic and transesophageal echocardiography demonstrated dilated left atrium and ventricle, hypertrophied right ventricle and a large ventricular septal defect with overriding of the aorta. However, both studies failed to visualize the pulmonic valve. The patient underwent cardiac catheterization which revealed the presence of pulmonary atresia. A descending aortogram showed the presence of a single large major aortopulmonary collateral artery (MAPCA) arising from the descending thoracic aor ta which supplies confluent left and right pulmonary arteries. The pulmonary arterial vasculature is enlarged and pruned in appearance. The main pulmonary artery is non-atretic. Mean proximal pulmonary artery pressure was 68 mmHg, indicating severe pulmonary hypertension. Patient wasstarted on digoxin and sildenafil.
SIGNIFICANCE: This case report emphasizes the importance of a well-developed central pulmonary circulation and an adequate but not excessive pulmonary blood supply from an aortopulmonary vascular connection in the survival of patients with pulmonary atresia into adulthood.