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A 5 year old girl presented with mild cyanosis since birth. Systemic oxygen saturation was 80%. Chest radiography revealed a prominent round opacity at the upper right heart border. Transthoracic echocardiography showed a dilated proximal right pulmonary artery. Echocontrast injected into a peripheral vein opacified the right heart, then passed rapidly to the left atrium. Positive pressure ventilation during cardiac catheterisation caused further systemic desaturation to 54%. Pulmonary artery angiography demonstrated a large fistula between the proximal right pulmonary artery and the left atrium. The communication tapered at its midpoint to a minimum diameter of 9.8 mm (below left). The fistula was crossed from the right heart with a long transseptal sheath and a 14 mm Amplatzer muscular ventricular septal defect occluder was deployed at its narrowest point (below centre). Oxygen saturation increased immediately to 100%. Repeat angiography demonstrated complete occlusion of the fistula. The device did not obstruct the right pulmonary artery and remained stable on release.
Direct communication between the right pulmonary artery and the left atrium is a rare congenital cardiac malformation. It may present in the neonatal period with intense cyanosis requiring urgent intervention. If desaturation is less severe, presentation may be delayed to later in childhood or even adult life. Early repair should be performed to avoid complications, particularly systemic emboli, cerebral abscess, and rupture of aneurysmal communications. Although surgical ligation or division is the accepted treatment, transcatheter coil occlusion has been described in one neonate and Amplatzer device occlusion offers an alternative for larger communications.