Porto-Pulmonary Hypertension in Extrahepatic Portal Vein Obstruction

Abstract

Pulmonary hypertension is defined as an increase in the resting mean pulmonary arterial pressure of more than 25 mmHg and is classified on the basis of the hemodynamics and clinical settings. An important category is pulmonary arterial hypertension, designated as Category 1, which is commonly seen in patients with congenital left-to-right shunts or diagnosed an idiopathic/heritable disorder. Also included in this category is another uncommon condition termed as porto-pulmonary hypertension, which develops as a complication of portal hypertension in patients with or without liver diseases. This is one such example, which developed in an adolescent with extrahepatic portal vein obstruction with an unfortunate fatality.