Objective: This report describes the diagnosis and management of a rare case of pulmonary AV fistula communicating with left atrium and presenting as cyanotic heart disease in a child.

Methods and results: 6 year old boy presented with recurrent respiratory tract infection since 6 months of age, dyspnoea on exertion and cyanosis since last 2 years, without any cyanotic spells. Echocardiography revealed 6 mm osteum secondum ASD with left to right shunt, large AV fistula with flow in Right Pulmonary Artery, contrast echo revealed early appearance of contrast in LA. Angiography showed giant pulmonary AV fistula arising from RPA and appearance of contrast in enlarged LA but the exact site of communication between the two could not be demonstrated. Cardiac catheterization data is shown in table 1.

Trans-thoracic echo done in Operation room after induction of anaesthesia showed a communication between right pulmonary artery and LA, which was entering from posterior wall of LA, also the giant aneurismal sac was lifting the LA from behind producing a camel hump (Fig1. parasternal long axis view). Under standard cardiopulmonary bypass & cardioplegia, aneurismal sac was seen arising from RPA and entering in the posterior wall of LA. LA was opened and the fistula repaired, ASD closed. Patient was weaned off from CPB. SPO2 returned to 100% after surgery. Patient was extubated 6 hrs after the surgery.

Conclusion: Echocardiography in operation room was helpful in localizing the site of fistula so that surgeons could locate it quickly without wasting precious time on CPB.