The direct congenital Gerbode defect was a rare type of ventricular septal defect (VSD) causing a communication between the left ventricle (LV) and right atrium (RA). In this case, only LV-to-right ventricle (RV)shunt was found preoperatively. But in operation, the defect was located at the interventricular septum between the tricuspid (TV) and mitral valves (MV) where the shunt was from LV to right atrium (RA) and septal TV dysplasia was found. The shunt might be LV-to-RA and the blood flow into the RV through the defect of TV. This article discussed why the preoperative ultrasound misdiagnosed this type of shunt and reviewed the literature of Gerbode defect.

The Gerbode defect is a special type of ventricle septum defect causing a communication between the left ventricle and right atrium. In this article, we present a rare case of 2-year-old congenital Gerbode defect. The location of this defect was special, just at the interventricular septum between the tricuspid and mitral valve. She underwent the closure of the defect with an autologous pericardial patch in station of mild hypothermic cardiopulmonary bypass.