高齢者ファロー四徴症の一剖検例

Tetralogy of Fallot revealed by autopsy in an elderly subject

　症例は62歳女性。学童期から易疲労感とチアノーゼを認めていたが加療せず。59歳時，呼吸困難増悪のため当科入院。赤血球850万／mm³，PaO₂35mmHgの多血症を伴う低酸素血症を認め，右室収縮期圧は155mmHgであった。RIアンギオでは心室レベルにおける高度の右左短絡を認めた。ファロー四徴症（TOF）の診断を得ぬまま在宅酸素療法を行っていたが62歳で死亡。剖検でファロー四徴症と診断された。本例の肺動脈狭窄は円錐狭窄であり，その心腔側は厚い線維組織に覆われていた。そしてこの組織は流出路方向ヘロート状に発育して狭窄を著しく増強していた。本例の剖検所見は比較的軽症のTOFであったが，この線維組織形成により肺動脈円錐狭窄が進行して死亡したと考えられた。

　本例は心臓カテーテル検査成績と剖検所見のそろっている報告としては国内で最も高齢のTOFである。

A 62-year-old woman had a history of cyanosis and easy fatigability from school age but had re-fused 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, 8.5×10⁶ 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 infun-dibular inlet was constituted of the thick fibrous tissue which remarkably augmented the pulmonic stenosis. Although the autopsy findings were con-sidered to be relatively mild TOF, the death was presumed to be caused by development of the in-fundibular 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.