Daimaru N, Okamura T, Nagano H, Shigematsu N, Yasunaga C, Sueishi K
Department of Respiratory Medicine, National Minamifukuoka Chest Hospital, Fukuoka, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1990 Nov;28(11):1504-10.
41 year-old male with liver cirrhosis accompanying severe hypoxemia was presented. Shortly after the diagnosis of liver cirrhosis, he suffered from exertional dyspnea and cyanosis. Though home oxygen therapy had been prescribed for 2 years, hypoxemia gradually progressed accompanied by persistent cough, mucous sputa and intermittent fever. The chest X-ray revealed bilateral interstitial shadow particularly localized in lower lung fields. The arteriovenous shunt ratio was shown to be 24% by oxygen method. Perfusion lung scan using 99mTc-labeled MAA revealed perfusion defects in bilateral lung fields and radionuclide uptake was strongly demonstrated in the kidneys. These clinical data suggested that severe hypoxemia was probably due to multiple arteriovenous shunt. With further progression of hypoxemia for 4 months, he died of hepatic failure and pulmonary infection. Autopsy showed Miyake's type B cirrhosis. Multiple pleural and subpleural arteriolar nevi were demonstrated grossly and microscopically. There were no arteriovenous malformations demonstrated after injection of barium-gelatin solution into the pulmonary artery. Histologically, irregularly dilated vessels were found in the lung parenchyma beneath the pleura and filled with blood and injection material. These clinical and pathological findings provided evidence that the mechanism of arterial desaturation was pulmonary arteriovenous shunting due to liver cirrhosis.
41岁男性，患有肝硬化并伴有严重低氧血症。在诊断为肝硬化后不久，他出现劳力性呼吸困难和发绀。尽管家庭氧疗已进行2年，但低氧血症仍逐渐进展，并伴有持续咳嗽、咳痰和间歇性发热。胸部X线显示双侧间质阴影，尤其在下肺野局部出现。通过氧法测得动静脉分流率为24%。使用99mTc标记的MAA进行肺灌注扫描显示双侧肺野灌注缺损，肾脏有强烈的放射性核素摄取。这些临床资料提示严重低氧血症可能是由于多处动静脉分流所致。在低氧血症进一步进展4个月后，他死于肝衰竭和肺部感染。尸检显示为三宅B型肝硬化。大体及显微镜检查可见多处胸膜及胸膜下小动脉痣。向肺动脉内注入钡明胶溶液后未发现动静脉畸形。组织学检查发现，胸膜下肺实质内有不规则扩张的血管，充满血液和注入物质。这些临床和病理发现证明，动脉血氧饱和度降低的机制是肝硬化导致的肺动静脉分流。
