先天性主動脈狹窄症

Congenital Aortic Stenosis

方鴻揚(H. Y. Fang) ； 呂鴻基(H. C. Lue) ； 陳烱霖(C. L. Chen)

《Acta Paediatrica Sinica》 14卷3期 (1973/09) Pp. 109-117

摘要

臺大醫院小兒科近十年來診斷爲先天性主動脈狹窄症之病例共六名，佔先天性心臓病之0.8%，六例爲五男一女，依解剖上狹窄分類爲主動靜瓣膜狹窄二名，主動脈瓣膜上部狹窄一名，合併主動脈瓣膜及其上部狹窄一名，特發性主動脈下部肥厚性狹窄二名。六例中三例有臨床症狀，諸如疲倦，呼吸短促，及心絞痛等。一例發生心臓衰竭而死亡。發育大抵正常，最重要之臨床體檢所見爲收縮期心雜音，位於主動脈區且皆有頸部傳遞。但此心雜音位置因狹窄類型而不同，如特發性主動脈下部肥厚性狹窄，位於左胸骨緣或心尖部。心電圖皆有左心室肥厚，勞損變化僅見於二例。胸部X光皆有心臓擴大，但不一定非常明顯。主動脈弓頸著擴大者有二例。作心臓血管攝影術者一例，其狹窄位置及瓣膜變化清晰可見。心臓導管檢查或及心造影檢查可檢出狹窄位置及程度，爲本病症診斷之最主要檢查。特發性主動脈下部肥厚性狹窄例之狹窄程度，依左心室擴張期之長短而加重或減輕，故可觀察心室外收縮前發之主動脈及左心室壓力變化以助診斷。此種狹窄可經外科手術切除心室中膈部之肥厚性心肌而得解除。

並列摘要

Six cases (five boys and one girl) of congenital aortic stenosis encountered at the Department of Pediatrics of the National Taiwan University during the past 10 years are presented consitituting 0.8% of all cases of congential heart disease. They consist of 4 different types: valvular type, 2; supravalvular type, 1; combined valvular and supravavular type, 1; idiopathic hypertrophic subaortic stenosis (IHSS), 2 cases.
All except 2 cases developed normally and had few, if any symptoms. The most important signs were systolic murmurs. Their locations: varied most often at the aortic area with carotid transmission; in 2 cases of IHSS murmurs located over the apex and/or left sternal border.
In all cases were there ECG evidences of left ventricular hypertrolphy, and in two signs of left ventricular strain patterns were also noted. Roentogenographic evidences of cardiomegaly, though sometimes indistinct, were detected in each case. Prominent aortic arch was found only in two cases. The location of the stenosis and the valvular changes were seen clearly in one case subjected to the angiocardiographic study.
Cardiac catheterization studies were performed in 4 cases. The diagnosis of IHSS was made or confirmed in one case by characteristic hemodynamic changes following ventricular premature contraction and surgery, and in another case by the demonstration of the characteristic carotid tractings in phonocardiography.