Maizza A F, Ho S Y, Anderson R H
Department of Paediatrics, National Heart and Lung Institute, London.
J Heart Valve Dis. 1993 Jan;2(1):66-79.
Obstruction within the outflow tract from the left ventricle can take several anatomic forms. To understand better these substrates, we reviewed the anatomy in the normal heart and, with this background, focused on the malformations observed at each level of the outflow tract which produce stenosis. We examined 75 malformed hearts and reviewed the literature pertinent to surgical aspects of obstruction. Close study of the normal aortic valve shows the lack of any discrete fibrous structure which can be described in terms of a ring-like annulus. The semilunar attachment of the leaflets makes a crown-shaped arrangement at the ventriculo-arterial junction rather than a strict ring. In the pathological study, valvar abnormalities were found in 45 cases. The valve had three leaflets in 20 cases, two leaflets in 24 cases and one leaflet in to the other. In two cases, additional obstruction was found at the sinutubular junction. Subvalvar stenosis was found in 30 hearts, the aortic valve being trifoliate in 28 and bifoliate in 2. The main substrate of subvalvar obstruction was posterior malalignament of the outlet septum (40%), followed by muscular septal hypertrophy (23%); obstruction was also an intrinsic feature in association with atrioventricular septal defects (14%). Although rare in our material, it can also be due to a fibrous or fibromuscular shelf. In our review of the literature, we have highlighted the various forms of valvar malformation and commented on the surgical options. Clear analysis of both the level of obstruction, and its precise anatomic substrate are essential for optimal surgical intervention.
左心室流出道梗阻可有几种解剖学形式。为了更好地理解这些病变基础,我们回顾了正常心脏的解剖结构,并在此背景下,重点关注流出道各水平出现的导致狭窄的畸形。我们检查了75例畸形心脏,并回顾了与梗阻手术方面相关的文献。对正常主动脉瓣的仔细研究表明,不存在任何可描述为环状瓣环的离散纤维结构。瓣叶的半月形附着在心室动脉交界处形成冠状排列,而非严格的环状。在病理研究中,45例发现瓣膜异常。瓣膜有三叶的20例,两叶的24例,一叶的1例。2例在窦管交界处发现额外梗阻。30例心脏发现瓣下狭窄,主动脉瓣三叶的28例,两叶的2例。瓣下梗阻的主要病变基础是流出道间隔后移(40%),其次是肌性间隔肥厚(23%);梗阻也是房室间隔缺损相关的固有特征(14%)。虽然在我们的资料中少见,但也可由于纤维性或纤维肌性嵴所致。在我们对文献的回顾中,我们强调了瓣膜畸形的各种形式,并对手术选择进行了评论。对梗阻水平及其精确的解剖学病变基础进行清晰分析对于最佳手术干预至关重要。