A case of portopulmonary hypertension associated with primary biliary cirrhosis

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

doi:10.4046/trd.2007.62.5.421

A case of portopulmonary hypertension associated with primary biliary cirrhosis

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

Research output: Contribution to journal › Article › peer-review

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 language	English
Pages (from-to)	421-426
Number of pages	6
Journal	Tuberculosis and Respiratory Diseases
Volume	62
Issue number	5
DOIs	https://doi.org/10.4046/trd.2007.62.5.421
Publication status	Published - 2007 May

Keywords

Portal hypertension
Primary biliary cirrhosis
Pulmonary hypertension

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine
Infectious Diseases

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.4046/trd.2007.62.5.421

Cite this

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title = "A case of portopulmonary hypertension associated with primary biliary cirrhosis",

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.",

keywords = "Portal hypertension, Primary biliary cirrhosis, Pulmonary hypertension",

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

year = "2007",

month = may,

doi = "10.4046/trd.2007.62.5.421",

language = "English",

volume = "62",

pages = "421--426",

journal = "Tuberculosis and Respiratory Diseases",

issn = "1738-3536",

publisher = "The Korean Academy of Tuberculosis and Respiratory Diseases",

number = "5",

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TY - JOUR

T1 - A case of portopulmonary hypertension associated with primary biliary cirrhosis

AU - Se, Joong Kim

AU - Eun, Ju Lee

AU - Ki, Hwan Jung

AU - Eun, Hae Kang

AU - Sung, Yong Lee

AU - Hong, Euy Lim

AU - Hyung, Joon Yim

AU - Sang, Yeub Lee

AU - Je, Hyeong Kim

AU - Shin, Chol

AU - Jae, Jeong Shim

AU - Kwang, Ho In

AU - Kyung, Ho Kang

AU - Se, Hwa Yoo

PY - 2007/5

Y1 - 2007/5

N2 - 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.

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.

KW - Portal hypertension

KW - Primary biliary cirrhosis

KW - Pulmonary hypertension

UR - http://www.scopus.com/inward/record.url?scp=34548295574&partnerID=8YFLogxK

U2 - 10.4046/trd.2007.62.5.421

DO - 10.4046/trd.2007.62.5.421

M3 - Article

AN - SCOPUS:34548295574

SN - 1738-3536

VL - 62

SP - 421

EP - 426

JO - Tuberculosis and Respiratory Diseases

JF - Tuberculosis and Respiratory Diseases

IS - 5

ER -

A case of portopulmonary hypertension associated with primary biliary cirrhosis

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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