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A Case of Portopulmonary Hypertension with the Right-to-left Shunt Natsuko Miyamoto 1 , Mitsuhiro Sumitani 1 , Manabu Tsuda 1 , Masayoshi Nishijima 1 , Seiichi Shoji 1 1Department of Respiratory Medicine, Osaka City General Hospital Keyword: 門脈肺高血圧症 , 肝肺症候群 , エンドセリン , portopulmonary hypertension , hepatopulmonary syndrome , endothelin pp.1286-1290
Published Date 2012/12/15
DOI https://doi.org/10.11477/mf.1404102116
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 A 73-year-old female visited our hospital because of progressive dyspnea on exertion. She had autoimmune hepatitis but she did not receive steroid therapy for her request. She had a feeling of dyspnea from five years ago, and her symptom gradually worsened. She was never a smoker. When she visited our hospital, PaO2 level was 60.3torr and PaCO2 level was 35.5torr in room air. There was evidence of hepatic cirrhosis and splenomegaly in abdominal echography, but no evidence of ascites. Additionally, the right-to-left shunt rate by (99m) Tc-MAA scanning was found in 16.7%. Therefore we considered the possibility of hepatopulmonary syndrome. However, estimated right ventricular systolic pressure level by echocardiogram showed 60torr, and right heart catheterization confirmed elevation of pulmonary vascular resistance and pulmonary arterial pressure. We finally diagnosed her as having portpulmonary hypertension. By treatment using Ambrisentan, the patient's clinical status improved in its symptoms, echocardiographic findings, and six minutes walk distance. The side effect by Ambrisentan showed mild eruption and headache, but did not cause an aggravation of liver function. This case report illustrates one possible safety neasure and the effect in Ambrisentan for portopulmonary hypertension.


Copyright © 2012, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院

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