Apical Muscular Ventricular Septal Defects Between the Left Ventricle and the Right Ventricular Infundibulum

Abstract

Background Effective transcatheter or surgical closure of apical muscular ventricular septal defects (VSDs) requires accurate delineation of variable and often complex anatomy. These defects have generally been considered as communications between the apexes of both left and right ventricles. Methods and Results Among 50 consecutive patients with multiple muscular VSDs referred for transcatheter device closure between October 1987 and April 1993, a subset of 10 patients (aged 7 days to 28 years) with apical muscular VSDs shared a unique set of anatomic characteristics: (1) large and often single opening in the left ventricle; (2) multiple right ventricular openings in the anterior aspect of the apical septum; and (3) separation of the right ventricular apical region into which the VSDs open from the rest of the right ventricular inflow and outflow by prominent muscle bundles. Additional analysis of the anatomy by use of echocardiography and cineangiography showed that these muscular defects were between the left ventricular apex and right ventricular infundibular apex. In 6 patients, the transcatheter devices used to create a septum in these hearts were placed in the right ventricle, straddling muscle bundles that separated the apical VSD from the rest of the right ventricular inflow and outflow, resulting in incorporation of a portion of the right ventricular infundibular apex into the physiological left ventricle. Three patients had devices placed between the apexes of the left ventricle and the infundibulum. The defect closed spontaneously within the right ventricle in 1 patient. One patient died after surgery for tetralogy of Fallot in situs inversus. The remaining 9 patients were all clinically well at the time of their most recent follow-up visit (follow-up duration, 32±11 months). This distinct type of apical VSD was identified by echocardiography in 20 of 274 patients who were followed up clinically for muscular VSDs. Conclusions Left ventricular–infundibular apical VSDs constitute a distinct morphological type of muscular VSD that can be distinguished by echocardiography and cineangiography. In selected cases, the infundibular apex can be separated from the rest of the right ventricular inflow and outflow to eliminate flow across these defects.