Preminger T J, Sanders S P, van der Velde M E, Castañeda A R, Lock J E
Department of Cardiology, Children's Hospital, Boston, MA 02115.
Circulation. 1994 Jan;89(1):236-42. doi: 10.1161/01.cir.89.1.236.
We report an unusual type of residual interventricular communication in patients with conotruncal malformations in which the aorta is completely or partly aligned with the right ventricle (RV). Interventricular communications after surgical repair usually result from additional defects, patch dehiscence, or incomplete closure and lie in the septal plane. However, after a right ventricular aorta is baffled to the left ventricle, the ventricular septal defect (VSD) patch and RV free wall form part of the systemic outflow tract. This "neo-left ventricular" outflow tract may provide a location for residual interventricular communications out of the septal plane.
We reviewed echocardiographic, angiographic, and clinical records of patients who had one or more residual interventricular communications out of the plane of the ventricular septum after repair of a conotruncal anomaly. Between June 1990 and October 1992, we observed such defects in eight patients, 5 to 26 years old, after repair of double-outlet right ventricle (n = 6), tetralogy of Fallot (n = 1), or truncus arteriosus (n = 1). In each, the VSD patch was anchored to the RV free wall near the aortic root. Nonetheless, channels were observed around the edge of the patch, between the neo-systemic outflow tract and the right ventricle. All patients had right ventricular hypertension; in seven, the pulmonary-to-systemic flow ratio (Qp:Qs) was > or = 2. At multiple unsuccessful reoperations (two to four per patient), the patch edges appeared securely attached to myocardium. Angiographic views profiling the septum failed to localize these defects, since they are not in the native septum. Echocardiographic detection of such anterior defects can be difficult. Transcatheter umbrella closure was attempted in the seven patients with large shunts; success was limited by the multiplicity of flow channels. Umbrella closure eliminated the need for further reoperation in four of seven patients, one patient died suddenly awaiting reoperation, and two deaths followed reoperation.
"Intramural" residual interventricular defects are difficult to diagnose by all modalities. Umbrella placement may reduce the left-to-right shunt. Successful surgical closure may require removal and reattachment of the anterior portion of the patch.
我们报告了一种在圆锥干畸形患者中不常见的残余心室间交通类型，其中主动脉完全或部分与右心室（RV）对齐。手术修复后的心室间交通通常由额外的缺损、补片裂开或不完全闭合引起，且位于间隔平面。然而，在右心室主动脉被改道至左心室后，室间隔缺损（VSD）补片和右心室游离壁构成了体循环流出道的一部分。这个“新左心室”流出道可能为间隔平面外的残余心室间交通提供一个位置。
我们回顾了圆锥干畸形修复后在室间隔平面外有一个或多个残余心室间交通的患者的超声心动图、血管造影和临床记录。在1990年6月至1992年10月期间，我们在8例年龄5至26岁的患者中观察到了此类缺损，这些患者分别为双出口右心室修复术后（n = 6）、法洛四联症修复术后（n = 1）或动脉干修复术后（n = 1）。在每例患者中，VSD补片固定在主动脉根部附近的右心室游离壁上。尽管如此，在补片边缘、新体循环流出道和右心室之间观察到了通道。所有患者均有右心室高压；7例患者的肺循环与体循环血流量比值（Qp:Qs）≥2。在多次（每位患者2至4次）不成功的再次手术中，补片边缘似乎牢固地附着于心肌。描绘间隔的血管造影视图未能定位这些缺损，因为它们不在原始间隔中。超声心动图检测此类前部缺损可能困难。对7例大分流患者尝试了经导管伞封堵；封堵成功率受血流通道多样性的限制。伞封堵使7例患者中的4例无需进一步再次手术，1例患者在等待再次手术时突然死亡，2例患者在再次手术后死亡。
“壁内”残余心室间缺损难以通过所有检查手段诊断。放置伞可能会减少左向右分流。成功的手术闭合可能需要移除并重新附着补片的前部。
