# 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)

## TL;DR

A rare heart condition involving a fistula between the right pulmonary artery and left atrium was successfully surgically corrected in a young woman from Nepal.

## Contribution

Presentation of a rare case of right pulmonary artery to left atrial fistula with successful surgical correction.

## Key findings

- Right pulmonary artery to left atrial fistula is a rare cyanotic congenital heart disease.
- Surgical correction under cardiopulmonary bypass is an effective treatment for this condition.
- The condition is often diagnosed late and may be associated with atrial septal defect.

## Abstract

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.