A case of portopulmonary hypertension associated with primary biliary cirrhosis

Joong Kim Se, Eun Joo Lee, Hwan Jung Ki, Hae Kang Eun, Sung Yong Lee, Hong Euy Lim, Hyung Joon Yim, Sang Yeub Lee, Je Hyeong Kim, Chol Shin, Jae Jeong Shim, Kwang Ho In, Ho Kang Kyung, Hwa Yoo Se

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)421-426
Number of pages6
JournalTuberculosis and Respiratory Diseases
Volume62
Issue number5
Publication statusPublished - 2007 May 1

Fingerprint

Biliary Liver Cirrhosis
Hypertension
Portal Hypertension
Vasoconstriction
Vascular Smooth Muscle
Pulmonary Hypertension
Dyspnea
Pulmonary Artery
Endothelium
Liver Diseases
Thrombosis
Survival
Liver
Therapeutics

Keywords

  • Portal hypertension
  • Primary biliary cirrhosis
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

A case of portopulmonary hypertension associated with primary biliary cirrhosis. / Se, Joong Kim; Lee, Eun Joo; Ki, Hwan Jung; Eun, Hae Kang; Lee, Sung Yong; Lim, Hong Euy; Yim, Hyung Joon; Lee, Sang Yeub; Kim, Je Hyeong; Shin, Chol; Shim, Jae Jeong; In, Kwang Ho; Kyung, Ho Kang; Se, Hwa Yoo.

In: Tuberculosis and Respiratory Diseases, Vol. 62, No. 5, 01.05.2007, p. 421-426.

Research output: Contribution to journalArticle

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AB - 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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