法洛氏四重症合併肺動脈瓣缺損症候群的麻醉－病例報告

Anesthetic Management of Tetralogy of Fallot with Absent Pulmonary Valve - A Case Report

李毅(Yi Lee) ； 魏志濤(Tze-Taur Wei) ； 邱英世(Ing-Sh Chiu)

《Acta Anaesthesiologica Sinica》 32卷1期 (1994/03) Pp. 69-72

摘要

法洛氏四重症之解剖構造為（1）心室中膈缺損，（2）主動脈跨騎於中膈缺損，（3）右心室肥大，（4）肺動脈狹窄或阻塞－此狹窄可位於圓椎部（infundi-bulum），瓣膜部位或瓣膜之後，或合併存在。自1964年Lev等人發表有關法洛氏四重症之病理解剖及其變異型以來，迄今發表的文獻統計法洛氏四重症有2.6－6.0%合併肺動脈瓣缺損，血流動力的機轉則迥異於傳統的法洛氏四重症候群。在肺動脈瓣缺損的病人，肺動脈血隨左右心室收縮及舒張而反覆回流（pulmonary regurgitation ＆ turbulent flow）造成肺主動脈嚴重擴大；而肺主動脈由於不正常血流的反覆衝擊形成動脈瘤樣擴張（aneurysmal dilatation），進而在平躺時嚴重壓迫主支氣管或分支氣管，因而病人會呈現重度呼吸障礙甚至須賴俯臥姿勢以維持氣道通暢。本病例報告即為敍述一名法洛氏四重症合併肺動脈瓣缺損症候群病人之麻醉考慮及其處理步驟。

並列摘要

Tetralogy of Fallot is characterized by a VSD, overriding of the aorta, right ventricular hypertrophy, and pulmonary stenosis. Absence of the pulmonary valve occurs in 2.6-6% of patients with tetralogy of Fallot. Operative procedures to relieve respiratory symptoms have been described with an mortality of 35-100%. Respiratory compromise may be severe in these infants secondary to bronchial compression by the aneurysmally dilated pulmonary arteries and present a different hemodynamic profile than those with classic tetralogy of Fallot. An understanding of the anatomic and physiologic principles and their anesthetic implications will lead to improved management of these extremely ill infants. We reported the anesthetic considerations and management of a case of tetralogy of Fallot with absent pulmonary valve of an infant who experienced severe respiratory distress on the supine position.