Journal List > Tuberc Respir Dis > v.62(5) > 1001093

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Kim, Lee, Jung, Kang, Lee, Lim, Yim, Lee, Kim, Shin, Shim, In, Kang, and Yoo: A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis
Case Report
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2007; 62(5): 421-426.

Published online: 31 May 2007

DOI: https://doi.org/10.4046/trd.2007.62.5.421

A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis

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

Department of Internal Medicine, College of Medicine, Korea University, Seoul, Korea.

Address for correspondence: Je Hyeong Kim, M.D. Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Ansan Hospital, 516 Gojan-1-dong, Danwon-gu, Ansan, 425-707, Korea. Phone: 82-31-412-5950, Fax: 82-31-413-5950, chepraxis@korea.com

Received 9 March 2007       Accepted 2 May 2007

Copyright © 2007 The Korean Academy of Tuberculosis and Respiratory Diseases

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

Figures and Tables

Figure 1
The simple chest radiography on admission (A) showed cardiomegaly, prominent pulmonary trunk, and pneumonic infiltration in right lower lung field, which improved two months later (B).
trd-62-421-g001
Figure 2
Chest computed tomography scan on admission showed the consolidative pneumonic infiltration in right lower lobe (A) and dilatation of pulmonary trunk without evidences of pulmonary thromboembolism (B).
trd-62-421-g002

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