Blom N A, Ottenkamp J, Jongeneel T H, DeRuiter M C, Gittenberger-de Groot A C
Department of Pediatric Cardiology, Leiden University Medical Center, P.O. Box 9602, The Netherlands.
Pediatr Cardiol. 2005 Jul-Aug;26(4):338-43. doi: 10.1007/s00246-004-0711-8.
It is generally considered that the development of secundum atrial septal defect (ASDII) or oval fossa defect is the result of excessive resorption of the embryological atrial septum primum, but this does not seem to explain all defects. We investigated 58 postmortem hearts with an ASDII and 22 normal hearts from patients ranging in age from 1 day to 49 years. The different structures of the oval fossa were examined. In 86% of the specimens, the defects were the result of a malformation of the valvula foraminis ovalis or embryological atrial septum primum, and in 14% an absent superior limbus (septum secundum) was the cause of the interatrial communication. The "septum primum" ASDs were divided into four subgroups based on the degree of deficiency of the septum primum and position of the ostium secundum within the septum primum. We conclude that the morphogenesis of ASDII is variable and both septum primum and septum secundum defects occur, which may be relevant in view of genetic studies that may lead to further differentiation of patients with and without genetically determined ASDIIs.
一般认为，继发孔型房间隔缺损（ASDII）或卵圆窝缺损的发生是胚胎期原发房间隔过度吸收的结果，但这似乎并不能解释所有的缺损情况。我们研究了58例患有ASDII的尸检心脏以及22例年龄从1天至49岁患者的正常心脏。对卵圆窝的不同结构进行了检查。在86%的标本中，缺损是卵圆孔瓣或胚胎期原发房间隔畸形的结果，而在14%的标本中，房间隔交通的原因是上缘（继发隔）缺失。“原发隔”型ASD根据原发隔的缺损程度以及继发孔在原发隔内的位置分为四个亚组。我们得出结论，ASDII的形态发生是可变的，原发隔和继发隔缺损均会出现，鉴于基因研究可能会导致对有或无基因决定的ASDII患者的进一步区分，这可能具有相关性。
