"Intramural" residual interventricular defects after repair of conotruncal malformations

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.