Tuberc Respir Dis > Volume 62(5); 2007 > Article
Tuberculosis and Respiratory Diseases 2007;62(5):421-426.
DOI:    Published online May 1, 2007.
A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis.
Se Joong Kim, Eun Ju Lee, Ki Hwan Jung, Eun Hae Kang, Sung Yong Lee, Hong Euy Lim, Hyung Joon Yim, Sang Yeub Lee, Je Hyeong Kim, Chol Shin, Jae Jeong Shim, Kwang Ho In, Kyung Ho Kang, Se Hwa Yoo
Department of Internal Medicine, College of Medicine, Korea University, Seoul, Korea.
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.
Key Words: Portal hypertension, Primary biliary cirrhosis, Pulmonary hypertension

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