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)

• Published in: BMC research notes Vol. 8; no. 1; p. 597
• Main Authors: Twayana, Ram Sundar, Humagain, Sanjaya, Koju, Rajendra, Joshi, Kriti Subas, Karmarcharya, Robin Man, Kc, Sajana, Poudel, Navaraj
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 23.10.2015; BioMed Central
• Subjects: Adult; Body mass index; Cardiovascular disease; Case Report; Communication; Congenital diseases; Congenital heart disease; Coronary artery bypass; Cyanosis - complications; Development and progression; Female; Fistula; Genetic aspects; Genetic disorders; Health aspects; Heart; Heart Atria - pathology; Heart diseases; Heart Diseases - complications; Heart Diseases - congenital; Heart surgery; Hospitals; Humans; Mortality; Pulmonary arteries; Pulmonary Artery - pathology; Risk factors; Teenagers; Tomography; Vascular Fistula - complications; Veins & arteries; Young Adult; Youth
• ISSN: 1756-0500; 1756-0500
• DOI: 10.1186/s13104-015-1593-y

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.