A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis

Portopulmonary hypertension (PPHTN) is a clinically and pathophysiologically distinct complication of advanced liver disease. PPHTN is characterized by the development of pulmonary arterial hypertension in association with advanced hepatic disease-related portal hypertension. A characteristic feature of PPHTN is an obstruction to the pulmonary artery flow caused by vasoconstriction, the proliferation of the endothelium and smooth muscle components of the vascular wall, as well as in situ thrombosis. This disorder is commonly underdiagnosed but the clinical implications are significant because it has substantial effects on survival and requires special treatment. We report a case of portopulmonary hypertension in a 53-year-old woman with primary biliary cirrhosis who presented with exertional dyspnea. (Tuberc Respir Dis 2007; 62: 421-426)

keywords: Portal hypertension, Primary biliary cirrhosis, Pulmonary hypertension.

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Article Contents

Vol.62 No.5

A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis

Abstract

Reference

Tuberculosis & Respiratory Diseases