An unusual case of cyanosis

Hepatopulmonary syndrome is a recognised complication of cirrhosis. However there are only few reports of this condition in patients with Non Cirrhotic Portal Fibrosis (NCPF) and Extra-Hepatic Portal Vein Obstruction (EHPVO). Here we present a 34 year old female with biopsy proven Non Cirrhotic portal fibrosis (NCPF) who presented with cyanosis and shortness of breath. She was thoroughly evaluated for all cardiac and pulmonary causes for her symptoms and was finally diagnosed to have Hepatopulmonary syndrome.