Duran A C, Frescura C, Sans-Coma V, Angelini A, Basso C, Thiene G
Institute of Pathological Anatomy, University of Padua Medical School, Italy.
J Heart Valve Dis. 1995 Nov;4(6):581-90.
The bicuspid aortic valve is the most frequent congenital cardiac malformation; it may be isolated or associated with other congenital heart disease. The present investigation consists of a study of bicuspid aortic valves in 1022 heart specimens belonging to the anatomical collection of the Institute of Pathological Anatomy of the University of Padua. A bicuspid aortic valve was observed in 95 specimens. It occurred as an isolated congenital cardiac defect in 28 cases, seven of which had spontaneous laceration of the aortic valve (aortic dissection). It was associated with other congenital cardiac malformations in 67 out of the remaining 994 specimens (6.7%), 41 of which (61.2%) showed obstruction of the aortic arch. The frequency of bicuspid aortic valve in specimens with complete transposition of great arteries was only 1%. Bicuspid aortic valve was particularly frequent in association with ventricular septal defect and was significantly more frequent in cases with (51.1%) than in cases without (20.5%) aortic arch obstruction (p < 0.001). There was no significant relationship between the occurrence of bicuspid aortic valves and left ventricular outflow tract obstructions or mitral valve malformations. The morphology of the pulmonary valve was also examined. Concurrence of a bicuspid aortic and pulmonary valve was detected in 11 specimens, five of these had trisomy-18. Our findings cast doubt on the assumption that altered fetal blood flow through the aortic valve may be the main factor producing the bicuspid condition. Indeed, they rather support the hypothesis that most bicuspid aortic valves are expressions of a developmental complex that affects the aortic arch and the wall of the ascending aorta as well as the aorta valve.
二叶式主动脉瓣是最常见的先天性心脏畸形；它可能是孤立存在的，也可能与其他先天性心脏病相关。本研究对帕多瓦大学病理解剖研究所解剖收藏的1022份心脏标本中的二叶式主动脉瓣进行了研究。在95份标本中观察到了二叶式主动脉瓣。它作为孤立的先天性心脏缺陷出现28例，其中7例发生主动脉瓣自发性撕裂（主动脉夹层）。在其余994份标本中，有67例（6.7%）与其他先天性心脏畸形相关，其中41例（61.2%）表现为主动脉弓梗阻。在大动脉完全转位的标本中，二叶式主动脉瓣的发生率仅为1%。二叶式主动脉瓣在合并室间隔缺损时尤为常见，在有主动脉弓梗阻的病例中（51.1%）比无主动脉弓梗阻的病例中（20.5%）明显更常见（p<0.001）。二叶式主动脉瓣的发生与左心室流出道梗阻或二尖瓣畸形之间无显著关系。还对肺动脉瓣的形态进行了检查。在11份标本中检测到二叶式主动脉瓣和肺动脉瓣并存，其中5例患有18三体综合征。我们的研究结果对胎儿血流通过主动脉瓣改变可能是导致二叶式病变的主要因素这一假设提出了质疑。实际上，它们更支持这样一种假说，即大多数二叶式主动脉瓣是一种发育复合体的表现，该复合体影响主动脉弓、升主动脉壁以及主动脉瓣。
