Reversal of severe hepato-pulmonary syndrome in congenital hepatic fibrosis after living-related liver transplantation

We report a 5-year-old girl with congenital hepatic fibrosis who presented with clubbing and cyanosis. Four weeks after living-related liver transplantation, she had normal blood gases and reduction in shunting to 7% on macroaggregated albumin scan.

Aabha Nagral, Fazal Nabi, Abhi Humar, Sanjay Nagral, Nilesh Doctor, S R Khubchandani, Y K Amdekar
Mar-April 2007, NCBI

Abstract

We report a 5-year-old girl with congenital hepatic fibrosis who presented with clubbing and cyanosis. Partial pressure of oxygen was 40 mmHg with oxy-gen saturation of 70% on room air, which improved to 128 mmHg and 92% on inhalation of 100% oxygen. Macroaggregated albumin scan showed 58% shunting to the brain, suggestive of severe hepatopulmonary syndrome. Echocardiogram and pulmonary angiogram ruled out pulmonary hypertension. Four weeks after living-related liver transplantation, she had normal blood gases and reduction in shunting to 7% on macroaggregated albumin scan.