Portopulmonary hypertension in a case of non-cirrhotic portal fibrosis.

Portopulmonary hypertension (PPHT) is the unusual association of portal hypertension (HT) with pulmonary HT. We report a case of noncirrhotic portal fibrosis (NCPF) leading to PPHT which is exceedingly rare with only very few cases reported in the literature. This is an autopsy report of a 30 years old man, a known case of portal HT who died suddenly due to a syncopal attack. Autopsy revealed massive pulmonary thromobembolism with pulmonary HT. Liver showed changes of NCPF. The rarity of NCPF causing PPHT prompted this case report.