Hepatopulmonary Syndrome-A Case Report and Literature Review

肝肺症候群－病例報告和文獻回顧

何承航(Cheng-Hang Ho) ； 邱銘煌(Ming-Huang Chiu)

《胸腔醫學》 23卷4期 (2008/08) Pp. 266-272

https://doi.org/10.29806/TM.200808.0005

摘要

肝肺症候群(hepatopulmonary syndrome)臨床上包含三項症候：1.慢性肝病2.低動脈血氧.3肺內血管擴張。它的致病原因並不完全了解，過多的一氧化氮產生似乎是造成肺內血管擴張的其中一項因素。而防止革蘭氏陰性菌轉移可以減少肝肺症候群的嚴重度。它臨床上主要的表現有低動脈血氧、杵狀指和蜘蛛狀血管瘤。本文報導62歲男性感染C型肝炎合併肝硬化診斷5年後，抱怨漸進性費力呼吸不順2年之久。病人本次呈現革蘭氏陰性菌尿症，併發燒、全身不適，伴隨著呼吸困難，特別是platypnea（坐時較喘、躺下改善）和orthodeoxia（動脈缺氧情形，坐時嚴重，躺下改善）。在擾動生理食鹽水微泡顯影劑超音波心圖檢查下，可見右心顯影後，約6次心搏後，在左心出現生理食鹽水微泡的超音波顯影。鎝-99m大聚合白蛋白肺灌注掃描證明擴散性肺外攝入，特別是腦、腎臟、甲狀腺和脾臟。當菌尿症獲得控制後，病人對氧氣的治療反應良好。

關鍵字

肝肺症候群hepatopulmonary syndrome ； platypnea ； orthodeoxia

並列摘要

Hepatopulmonary syndrome (HPS) is defined as the clinical triad of advanced liver disease, arterial deoxygenation and intrapulmonary vascular dilatation. Its pathogenesis is not completely understood. Excessive pulmonary nitric oxide production seems to be 1 of the factors that contribute to the intrapulmonary vascular dilatation. Prevention of Gram-negative bacilli translocation reduces the severity of HPS. The major clinical manifestations are arterial hypoxemia, clubbing of the fingers and spider nevi. We report a 62-year-old male with chronic hepatitis C-related liver cirrhosis diagnosed for 5 years, who complained of progressive exertional dsypnea for more than 2 years. This patient presented with Gram-negative bacteriuria with symptoms of fever and general malaise, followed by severe dyspnea; especially platypnea and orthodeoxia. Contrast-enhanced echocardiography with agitated saline showed microbubble opacification of the left heart within 6 heartbeats after it appeared in the right heart. Technetium-99m macroaggregated albumin (Tc-99m MAA) lung perfusion scan demonstrated diffuse uptake outside the lung fields, especially in the brain, kidneys, thyroid and spleen. The patient responded well to oxygen therapy after urosepsis had been controlled.

並列關鍵字

hepatopulmonary syndrome HPS ； platypnea ； orthodeoxia