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고경행 ( Kyung Haeng Ko ),박창민 ( Chang Min Park ),임명수 ( Myung Soo Rim ),김유일 ( Yoo Il Kim ),장일권 ( Il Gweon Jang ),황준화 ( Joon Hwa Hwang ),임성철 ( Sung Chul Lim ),김영철 ( Young Chul Kim ),박경옥 ( Kyung Ok Park ),박 대한결핵 및 호흡기학회 1999 Tuberculosis and Respiratory Diseases Vol.47 No.4
김영철,박경옥,이정훈,박종춘,강광원,김준우,황준화,장일권,박형관,박창환 대한내과학회 1997 대한내과학회지 Vol.53 No.4
Hepatopulmonary syndrome consists of a triad of liver dysfunction, intrapulmonary vascular dilatation, and hypoxemia. This is one of the main causes of arterial hypoxemia in patients with chronic liver disease. The vascular abnormalities are precapillary dilatation, direct arterial-venous communication, and dilated pleural vessels. In this article, we report a case of hepatopulmonary syndrome in a 62-year-old woman who had complained progressively worsening dyspnea, platypnea, and orthodeoxia. She had huge splenomegaly, clubbing fingers and cyanosis of lip and fingers. Arterial blood gas analysis showed refractory arterial hypoxemia and orthodeoxia suggesting right-to-left $quot;shunting$quot;. Chest X-ray showed increased interstitial markings on the lower part of right lung, In ^(99m)Tc-labeled macroaggregated albumin (MAA) lung perfusion scan, there was no perfusion defect in the lung, but labeled radionuclide were taken up in the intraabdominal organs, kidney, liver and spleen. The amount of shunted radionuclide were about 58 percent. In contrast echocardiography, microbubbles which were injected via cephalic vein were visualized in the left atrium at 4 cardiac cycles after leaving the right ventricle indicating intrapulmonary right-to-left $quot;shunting$quot; rather than intracardiac shunt. Pulmonary angiographic finding revealed diffuse blotchy arterial dilatation on both lung fields, especially lower lobes of both lungs. Current modalities of treatment of hepatopulmonary syndrome are the therapeutic embolization of direct arterial-venous communication for focal vascular dilatations, and TIPSS (Transjugular intrahepatic porto-systemic shunt) or liver transplantation for diffuse intrapulmonary vascular dilatations. Despite our recommendation of TIPSS, she refused the procedure and is under home oxygen therapy.