Vázquez-Antona Clara A, Muñoz-Castellanos Luis, Roldan G F Javier, Orellana Julio Erdmenger, Cárdenas Angel Romero, Vargas-Barrón Jesús
Departamento de Ecocardiografía, Instituto Nacional de Cardiología Ignacio Chávez, Mexico.
Arch Cardiol Mex. 2008 Jan-Mar;78(1):40-51.
It has been postulated that there is a morphogenetic relation between the atrioventricular septal defect (AVSD) type A of Rastelli and the type of two separated orifices, this so called partial forms, existent between both types a spectrum of anatomical forms in which interchordal spaces determinate the ventricular septal defects (VSD) size to forms in which the VSD is closed by fusion of the left septal valves to the crest of ventricular septum.
We present five patients which illustrates the variability of the atrioventricular defect by means of two dimensional and three-dimensional echocardiography. In each case was made a transesophagic echocardiogram using three-dimensional reconstruction with an Echo-Scan system (4.0 TomTec Gmb version, Munich, Germany).
It was observed the following spectrum of atrioventricular defect: one patient had a complete closure of the VSD by the insertion of the left septal valves to the interventricular septal crest. One patient has a partially closed VSD. The last 3 patients had a large VSD with a large shunt and high pulmonary pressure. In those patients in whom the VSD was completely or partially closed, the hemodynamic behavior depended of the interatrial shunt and the regurgitation of the atrioventricular valve. They didn't present pulmonary hypertension, what allowed them to be less symptomatic.
The three-dimensional echocardiographic study of the spectrum of AVSD type A of Rastelli, defines accurately the valve components and septal structures, so we can understand the transition between complete and partial forms. This difference determines the clinical evolution of the patients.
据推测,Rastelli A型房室间隔缺损(AVSD)与两个分离孔型之间存在形态发生关系,即所谓的部分型,这两种类型之间存在一系列解剖形式,其中腱索间隙决定室间隔缺损(VSD)的大小,到室间隔缺损通过左间隔瓣与室间隔嵴融合而闭合的形式。
我们展示了5例患者,通过二维和三维超声心动图说明了房室缺损的变异性。在每种情况下,均使用Echo-Scan系统(德国慕尼黑4.0 TomTec Gmb版本)进行三维重建的经食管超声心动图检查。
观察到以下房室缺损范围:1例患者通过左间隔瓣插入室间隔嵴实现室间隔缺损完全闭合。1例患者室间隔缺损部分闭合。最后3例患者有大的室间隔缺损,伴有大量分流和高肺压。在室间隔缺损完全或部分闭合的患者中,血流动力学行为取决于心房分流和房室瓣反流。他们未出现肺动脉高压,因此症状较轻。
对Rastelli A型AVSD范围进行三维超声心动图研究,可准确界定瓣膜成分和间隔结构,从而我们能够理解完全型和部分型之间的转变。这种差异决定了患者的临床病程。