Muñoz-Castellanos L, Kuri Nivon M, Vázquez Antona C A, Salinas Sánchez H C
Departamento de embriología, Instituto Nacional de Cardiología, Ignacio Chávez, INCICH, Juan Badiano No. 1, 14080 México D.F.
Arch Inst Cardiol Mex. 2000 Nov-Dec;70(6):536-51.
Fifty seven hearts with absence of atrioventricular (A-V) connection were studied morphologically to specify their types of ventriculoarterial connection and their associated anomalies; the anatomic features of the hearts were correlated with their echocardiographic and cardioangiographic images in order to establish their mutual correspondence. Fifty six hearts had situs solitus; fifty specimens had right absent A-V connection and six had left absent A-V connection. One had situs inversus. All the specimens had: A deep A-V sulcus at the site of the absent A-V connection, a dimple in the muscular floor of the involved atrium connected with the dilated and hypertrophic left ventricle, incomplete right ventricle without inlet portion, ventricular septal defect of variable dimensions (it was obliterated in two), atrial septal defect, the ventricular septum deviated from the crux cordis. The left absent A-V connection had ventricular inversion and discordant ventriculoarterial connection. In the right absent A-V connection the ventriculoarterial connections were concordant in thirty eight hearts, from which thirty four had pulmonary stenosis both infundibular and valvular (five had the tetrad of Fallot), two had pulmonary valve atresia and two had a dilated pulmonary artery; discordant in nine hearts, one with aortic atresia; double outlet, from the right ventricle in two, (one with the tetrad of Fallot) and from the left ventricle in one. The heart in situs inversus had ventricular inversion, right absent A-V connection (left-sided), single (right) ventricle and atresia of the left ventricle. The correlations between cardiac morphology and imaging were precise. Developmentally, this cardiopathy is the result of an ectopic unequally lateralized septation of the common atrioventricular canal, which separates two canals, one stenotic leading to atresia and the other which develops too wide.
对57例无房室连接的心脏进行了形态学研究，以明确其心室动脉连接类型及相关异常；将心脏的解剖特征与其超声心动图和心血管造影图像进行关联，以建立它们之间的对应关系。56例心脏为正常位；50例为右型无房室连接，6例为左型无房室连接。1例为镜面右位心。所有标本均有：在无房室连接部位有一条深的房室沟，受累心房肌性底部有一个凹陷与扩张肥厚的左心室相连，右心室不完整且无流入道部分，室间隔缺损大小不一（2例缺损已闭塞），房间隔缺损，室间隔偏离心脏十字交叉。左型无房室连接有心室反位和不一致的心室动脉连接。在右型无房室连接中，38例心脏的心室动脉连接一致，其中34例有漏斗部和瓣膜部肺动脉狭窄（5例有法洛四联症），2例有肺动脉瓣闭锁，2例有肺动脉扩张；9例不一致，1例有主动脉闭锁；双出口，2例起源于右心室（1例有法洛四联症），1例起源于左心室。镜面右位心的心脏有心室反位、右型无房室连接（左侧）、单心室（右侧）和左心室闭锁。心脏形态与影像学之间的相关性精确。从发育角度来看，这种心脏病是共同房室管异位且不均衡侧向分隔的结果，该分隔将两个通道分开，一个狭窄导致闭锁，另一个发育过宽。
