Zuberbuhler J R, Anderson R H
Br Heart J. 1979 Mar;41(3):281-8. doi: 10.1136/hrt.41.3.281.
The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle. Successful pulmonary valvotomy with subsequent adequate right ventricular function would have been precluded by a tiny right ventricular cavity or infundibular atresia in 14 specimens and by severe tricuspid stenosis or regurgitation in an additional 4. Severe right or left ventricular endocardial fibroelastosis may have adversely affected ventricular function in several others. Ten specimens displayed convex bulging of the left ventricular septal surface. If these these anatomical findings are representative for the condition as a whole, they provide a good explanation for its disastrous prognosis.
回顾了37例室间隔完整的肺动脉闭锁标本的形态学特征，特别强调了可能影响肺动脉瓣切开术结果的特征。通过测量左右心腔尺寸并与20例正常婴儿心脏进行比较，对右心发育不全的程度进行了量化。右心室腔大小通常小于正常，但构成了一个范围，从极小到大于正常的扩张心室。三尖瓣环大小与右心室大小呈正相关，但肺动脉大小与右心室大小之间无相关性。14例标本中，微小的右心室腔或漏斗部闭锁，以及另外4例严重的三尖瓣狭窄或反流，会使随后无法成功进行肺动脉瓣切开术并获得足够的右心室功能。其他几例中，严重的右心室或左心室心内膜弹力纤维增生可能对心室功能产生了不利影响。10例标本显示左心室间隔表面呈凸状隆起。如果这些解剖学发现代表了整体情况，那么它们很好地解释了该病的灾难性预后。
