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KMID : 0383820070620050421
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Tuberculosis and Respiratory Diseases
2007 Volume.62 No. 5 p.421 ~ p.426
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A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis
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Kim Se-Joong
Lee Eun-Joo
Jung Ki-Hwan
Kang Eun-Hae
Lee Sung-Yong
Lim Hong-Euy
Yim Hyung-Joon
Lee Sang-Yeub
Kim Je-Hyeong
Shin Chol
Shim Jae-Jeong
In Kwang-Ho
Kang Kyung-Ho
Yoo Se-Hwa
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Abstract
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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.
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KEYWORD
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Portal hypertension, Primary biliary cirrhosis, Pulmonary hypertension
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