Jan J S, Chiu C T, Lin F C, Sheen I S, Lin D Y, Chen T C
Department of Hepato-Gastroenterology, Chang Gung Memorial Hospital, Taoyuan Hsien, Taiwan, ROC.
Changgeng Yi Xue Za Zhi. 1996 Jun;19(2):160-5.
Hepatopulmonary syndrome (HPS) is the triad of liver disease, pulmonary vascular dilatation, and abnormal arterial oxygenation. We report a case with progressive orthodeoxia and platypnea who was proven to have HPS after serial investigations, including autopsy. This 61-year-old male is a case of alcoholic liver cirrhosis without any documented cardiopulmonary disorders before. However, he suffered from progressive dyspnea in his last one-year life. During that period, progressive severe hypoxemia, orthodeoxia and platypnea were detected. Serial non-invasive diagnostic approaches, including two-dimensional contrast enhanced echocardiography and technetium-99m labeled macroaggregated albumin (MAA) lung perfusion scanning, were performed and showed positive evidence of intrapulmonary shunting. Direct evidence of intrapulmonary vascular dilatation was finally proven by autopsy. Although this syndrome is not rare, clinical awareness of the association between liver disease and arterial oxygen desaturation is still inadequate. We report a case proved by typical clinical manifestation and complete investigation and review the literature to emphasize this important disorder.
