A Rare Case of Gerbode Defect and Review of Literature

Abstract

A Gerbode defect is a very rare type of congenital anomaly with a direct communication between left ventricle and right atrium leading to Left to Right shunt. This defect was first reported by Meyer in 1857. First successful closure was done by Kerby et al using hypothermia and inflow occlusion technique. It may present as a direct or indirect type. With improvements in nomenclature and taxonomy that expanded the classification until the current modifications were in place that accounted for defect type and position with respect to the Tricuspid Valve. Now, it is broadly described as Supravalvular type and infravalvular type. The etiology is typically congenital with irregularities emerging by perforation of anterior intraventricular septum, malformation of leaflets, or widening of the commissural space. These embryological deviations subsequently permit an abnormal communication that begins the physiological processes leading to the pathology. Shunt depends on the size of the defect and PVR. Shunt flow begins inutero because of the obligatory difference in the systolic pressure between LV and RA. RA accepts shunt with less or no elevation of pressures due to distensibility. We report a 8 year old female child who presented with dyspnea on exertion and recurrent respiratory infections whose echocardiography confirmed a direct type (supravalvular) Gerbode defect. This was confirmed by cath study and during surgery. She underwent successful suture closure of the shunt using a Goretex patch. This is a case of direct type Gerbode defect doing well after surgery.