Stamm C, Anderson R H, Ho S Y
Paediatrics, Imperial College School of Medicine, National Heart and Lung Institute, London, UK.
Eur J Cardiothorac Surg. 1997 Oct;12(4):587-92. doi: 10.1016/s1010-7940(97)00184-x.
Competence of the tricuspid valve is crucial for survival of children with hypoplastic left heart syndrome. We studied the morphology and topology of the valvar and subvalvar structures, trying to identify abnormalities which could impair valvar function.
A total of 82 specimens with hypoplastic left heart syndrome were examined pathologically. Measurements of valvar dimensions were taken, significant dysplasia of the valvar leaflets was noted and the muscular and tendinous supporting structures determined. The findings were correlated to the subgroups of hypoplastic left heart syndrome.
Of the hearts, 10 (12%) showed a bileaflet right atrioventricular valve, 27 (33%) a moderately and 2 (2%) a severely dysplastic tricuspid valve. The majority of the abnormalities was found in hearts with a patent mitral valve. In 79% of the hearts with mitral atresia, the septal surface was concave instead of convex to the right ventricular lumen and the direct tendinous attachments of the septal leaflet replaced by a multitude of freestanding papillary muscles. The number of direct septal attachments was significantly higher in hearts with a patent mitral valve.
The tricuspid valve in hypoplastic left heart syndrome can differ from the valve seen in normal patients. The subvalvar apparatus is different in hearts with mitral atresia, whereas dysplasia of the leaflets occurs more often together with mitral stenosis. These features should be considered in reconstructive operations as well as during diagnostic procedures.
三尖瓣功能对于左心发育不全综合征患儿的存活至关重要。我们研究了瓣膜及瓣膜下结构的形态和拓扑结构，试图找出可能损害瓣膜功能的异常情况。
对82例左心发育不全综合征标本进行了病理检查。测量了瓣膜尺寸，记录了瓣膜小叶的明显发育异常情况，并确定了肌肉和腱性支撑结构。研究结果与左心发育不全综合征的亚组相关。
在这些心脏中，10例（12%）显示为双叶右房室瓣，27例（33%）为中度发育异常，2例（2%）为重度发育异常的三尖瓣。大多数异常情况见于二尖瓣开放的心脏。在79%的二尖瓣闭锁心脏中，隔膜表面向右心室腔呈凹形而非凸形，隔膜小叶的直接腱性附着被大量独立的乳头肌所取代。二尖瓣开放的心脏中隔膜直接附着的数量明显更多。
左心发育不全综合征中的三尖瓣可能与正常患者的瓣膜不同。二尖瓣闭锁的心脏中瓣膜下装置不同，而小叶发育异常更常与二尖瓣狭窄同时出现。在重建手术以及诊断过程中都应考虑这些特征。
