Zamora C, Somerville J
Arch Inst Cardiol Mex. 1978 May-Jun;48(3):588-602.
Ten patients with duplication of the atrioventricular valves were studied, nine of them with the double orifice located in the mitral valve and the other one in the tricuspid. In two cases the diameter of the secondary valve was the same as that of the normal valve, and in the other 8 cases, it was smaller; each had its own valvular apparatus with chordae tendinae attached to papillary muscles located in different sites. The principal malformation in nine was ostium primum. One of the cases with mitral duplication had pulmonary atresia, an association which has never before been published. In five patients regurgitation of the secondary orifice was present; the diagnosis was made during surgery in nine cases and at necropsy in one. The orifice was sutured in three cases because of regurgitation. Four patients died in the immediate post-operative period, including the one with pulmonary atresia. The other three had pulmonary hypertension. These findings agree with the literature. The etiological hypotheses are discussed and emphasis is given to the importance of the finding of a narrow jet of contrast material directed upward and to the right in the left angiocardiogram in frontal plane. The authors believe that this may be an important sign in diagnosing cases with ostium primum septal defect; taking into account the fact that double mitral orifice is the cause of valvular insufficiency, this can be the site of bacterial endocarditis and usually needs to be surgically closed.
对10例房室瓣重复畸形患者进行了研究，其中9例双孔位于二尖瓣，另1例位于三尖瓣。2例患者的副瓣直径与正常瓣膜相同，其余8例副瓣直径较小；每个副瓣都有自己的瓣膜装置，腱索附着于位于不同部位的乳头肌。9例患者的主要畸形为原发孔型房间隔缺损。1例二尖瓣重复畸形患者合并肺动脉闭锁，这种关联此前从未有过报道。5例患者存在副瓣口反流；9例在手术时确诊，1例在尸检时确诊。3例因反流对瓣口进行了缝合。4例患者在术后即刻死亡，包括1例合并肺动脉闭锁的患者。另外3例有肺动脉高压。这些发现与文献报道相符。文中讨论了病因假说，并强调了在左前斜位左心造影中发现造影剂狭窄喷射向上并向右的重要性。作者认为，这可能是诊断原发孔型房间隔缺损病例的一个重要征象；考虑到双二尖瓣口是瓣膜关闭不全的原因，这里可能是细菌性心内膜炎的发病部位，通常需要手术关闭。
