An uncommon variant of cyanotic congenital heart disease in a young adult female: a rare case of right pulmonary artery to left atrial fistula (PALAF)

Ram Sundar Twayana, Sanjaya Humagain, Rajendra Koju, Kriti Subas Joshi, Robin Man Karmarcharya, Sajana KC, Navaraj Poudel

Cyanotic congenital heart disease is not a rare entity, but fistula between the right pulmonary artery and the left atrium is an uncommon vascular anomaly. Although it is a real challenge to diagnose the case, detailed clinical evaluation and selective investigations are keys for diagnosis, and surgical intervention is still considered the best treatment option. A 19 years old girl from the remote village of Nepal presented with the history of exercise intolerance associated with cyanosis and clubbing of the extremities. We diagnosed her as a case of right pulmonary artery to left atrial fistula, a rare variant of pulmonary arteriovenous malformation. She underwent successful surgical correction of the anomaly under cardiopulmonary bypass surgery. Direct communication between the right pulmonary artery and the left atrium is a rare cyanotic congenital heart disease, which is diagnosed late and often associated with the atrial septal defect. The best treatment available is surgical correction.