Macedo A, Pinto E, Ramos S, Brito M J, Costa M M, Serrano I, Torrezão I, Lima M, Silva M E, Roriz M L
Serviços de Cardiologia Pediátrica e de Anatomia Patológica, Hospital de Santa Marta, Lisboa.
Acta Med Port. 1991 Sep-Oct;4(5):253-6.
In order to correlate gross morphological features with pulmonary and coronary vasculature changes in hypoplastic left heart syndrome (HLHS) 15 heart-lung necropsy specimens from neonates were analysed. Histology of left atrium wall, lung vessels with pulmonary arteriolar disease morphometric grading, study of intra and extra pulmonary wall veins and coronary arteries were recorded. Classic HLHS was observed in 11, and DORV with mitral atresia in 4, the foramen ovale (FO) being the only left atrium outflow tract in all. All specimens had increased % of thickness and muscular extension of arterioles and increased wall thickness of pulmonary veins, but severer arteriolar and venous changes with left atrium fibrosis were noticed in 8 specimens with either closed FO (3 cases) or mean FO diameter (d) of 5 mm when compared with specimens with mean FOd of 9 mm. Coronary arteries histology disclosed in 4 of 8 specimens with opened but hypoplastic mitral valve, intima proliferative changes with lumen narrowing, not observed in 7 specimens with mitral valve atresia. We concluded that in HLHS closed or restrictive FO and permeable mitral valve may predispose neonates to respiratory complications, right ventricle abnormal function and arrhythmias, specially after cardiac surgery.
为了将大体形态特征与左心发育不全综合征（HLHS）的肺和冠状动脉血管变化相关联，分析了15例新生儿心肺尸检标本。记录了左心房壁的组织学、伴有肺小动脉疾病形态计量分级的肺血管、肺壁内和肺壁外静脉以及冠状动脉的研究情况。观察到11例典型HLHS，4例为法洛四联症合并二尖瓣闭锁，卵圆孔（FO）是所有病例中唯一的左心房流出道。所有标本的小动脉厚度百分比和肌肉延伸增加，肺静脉壁厚度增加，但与平均FO直径为9mm的标本相比，8例FO闭合（3例）或平均FO直径（d）为5mm的标本中，小动脉和静脉变化更严重，伴有左心房纤维化。8例二尖瓣开放但发育不全的标本中有4例冠状动脉组织学显示内膜增生性改变伴管腔狭窄，7例二尖瓣闭锁的标本中未观察到这种情况。我们得出结论，在HLHS中，闭合或限制性FO以及可渗透的二尖瓣可能使新生儿易患呼吸并发症、右心室功能异常和心律失常，特别是在心脏手术后。
