Hepatic Vein to Atrial Fistula: Rare Cause of Cyanosis in a Post-operative Case of Fontan Surgery

Abstract

This is regarding a 10-year-old boy with a diagnosis of common atrium, single ventricle, common atrio ventricular (AV) canal, severe pulmonary stenosis, no AV valve regurgitation, and good biventricular function. He underwent bidirectional Glenn shunt with MPA ligation at age of 1 year in 2005. In July 2011, extra cardiac fenestrated Fontan operation was done. His saturation after surgery was 92 % on room air and child was doing well after 3 months follow up. He did not come for follow-up for 2 years. After 2 years, he came to us with dyspnoea on exertion and room air saturation of 70 %. After detailed evaluation, we found that his lungs were fairly normal with good sized branch pulmonary arteries (PA), cardiac function was good, and there was no AV valve regurgitation and no signs of Fontan failure. Confused about the cause of cyanosis, we took the patient for cardiac catheterization. We found that Fontan circuit (Fig. 1) was flowing well with no decompressing veins. Mean PA pressure was 12 mm Hg. We decided to balloon occlude the fenestration (Fig. 2), but there was only 2 % increase in saturation with no effect on PA pressures. So, it was concluded that fenestaration was not the cause of this severe cyanosis. Hand injection was done in infra hepatic portion of inferior vena cava (IVC) which showed a huge fistula from left hepatic vein to atrium (Fig. 3). It was tapering distally with 14 mm near origin and 9 mm at distal end. We decided to balloon occlude the fistula. It was occluded with 14 mm 9 4 cm Tyshak II balloon (Fig. 4). After occlusion, his saturation came up to 94 % and mean PA pressure was 14 mm Hg. Decision was taken to close the fistula with atrial septal defect (ASD) occluder. Fistula was successfully closed with the help of 16 mm Lifetech ASD device (Fig. 5). Post-device angiogram showed no residual flow through the fistula, room air saturation of 94 %, and mean PA pressure of 14 mm Hg. He was discharged next day and is doing well on a follow-up period of 6 months.