Harada Y, Fuseno H, Tomii T, Yamahara Y, Kuwahara T, Ohtomo T, Nakamura M, Kitamura T
Department of Internal Medicine, Shakaihoken Kobe Central Hospital.
Kokyu To Junkan. 1990 Oct;38(10):1049-52.
A 62-year-old women had a history of cyanosis and easy fatigability from school age but had refused medical examinations. She was admitted to our department with exacerbation of dyspnea at the age of 59. Laboratory tests showed hypoxia complicated with polycythemia with 35 mmHg of PaO2, 8.5 x 10(6) erythrocytes per mm3. The systolic pressure of the right ventricle was 155 mmHg. RI angio demonstrated a high degree of the right-to-left shunt at the ventricular level. The patient was given oxygen therapy at home without diagnosis of tetralogy of Fallot (TOF) and eventually died at the age of 62. Tetralogy of Fallot was diagnosed at autopsy. Pulmonary stenosis of this case was located in infundibulum. The lining of the infundibular inlet was constituted of the thick fibrous tissue which remarkably augmented the pulmonic stenosis. Although the autopsy findings were considered to be relatively mild TOF, the death was presumed to be caused by development of the infundibular stenosis due to formation of fibrous tissue. This is the oldest patient in Japanese medical literature to be identified by cardiac catheterization and autopsy finding as a case of TOF.
一名62岁女性自学龄起即有发绀和易疲劳史，但拒绝接受医学检查。59岁时因呼吸困难加重入住我科。实验室检查显示低氧血症合并红细胞增多症，动脉血氧分压（PaO2）为35 mmHg，红细胞计数为每立方毫米850万。右心室收缩压为155 mmHg。右心造影显示心室水平存在高度右向左分流。该患者在家接受氧疗，未诊断为法洛四联症（TOF），最终于62岁死亡。尸检诊断为法洛四联症。该病例的肺动脉狭窄位于漏斗部。漏斗部入口内衬由增厚的纤维组织构成，显著加重了肺动脉狭窄。尽管尸检结果被认为是相对轻度的法洛四联症，但推测死亡原因是由于纤维组织形成导致漏斗部狭窄进展。这是日本医学文献中经心导管检查和尸检结果确诊为法洛四联症的年龄最大的患者。
