Matsuura Keiko, Akizuki Shinichiro, Nakamura Natsuki, Ishibashi-Ueda Hatsue, Moriyama Masatsugu
Department of Molecular Pathology, Faculty of Medicine, Oita University, Yufu-city, Oita, Japan.
South Med J. 2007 Feb;100(2):218-21. doi: 10.1097/01.smj.0000242349.55168.a7.
We report an autopsy case of right isomerism in a 42-year-old Japanese man. Although he had not undergone any surgical intervention, he was healthy and did not exhibit any symptoms of heart failure. The cardiac findings indicated right isomerism with complex and severe cardiac malformations, including a morphologically common atrium, common atrioventricular valve, double inlet right ventricle, total anomalous pulmonary venous drainage, pulmonary outflow obstruction, pulmonary atresia, and major aortopulmonary collaterals from the descending aorta. He had no spleen, and exhibited some gastrointestinal malformations. The absence of risk factors for mortality from right isomerism, such as pulmonary venous obstruction, AV valve regurgitation, sepsis or severe arrhythmia, might have contributed to the longevity of this patient. The presence of pulmonary outflow obstruction that is reported to confer a better prognosis might also have contributed to his longevity.
我们报告一例42岁日本男性右位异构的尸检病例。尽管他未接受任何手术干预，但身体健康，未表现出任何心力衰竭症状。心脏检查结果显示为右位异构，伴有复杂且严重的心脏畸形，包括形态学上的共同心房、共同房室瓣、右心室双入口、完全性肺静脉异位引流、肺流出道梗阻、肺动脉闭锁以及降主动脉发出的主要主肺动脉侧支。他没有脾脏，并表现出一些胃肠道畸形。缺乏右位异构相关的死亡风险因素，如肺静脉梗阻、房室瓣反流、败血症或严重心律失常，可能是该患者长寿的原因。据报道存在的肺流出道梗阻具有较好的预后，这也可能是他长寿的原因。
