Double orifice left AV valve with partial atrio-ventricular septal defect

Partial atrioventricular septal defects (PAVSD) account for three percent of all major congenital cardiac anomalies. It is surgically managed by patch closure of the primum atrial septal defect (ASD) and repair of the cleft left atrioventricular (AV) valve. Double orifice left AV valve, however, occurs in five percent of PAVSD and constitutes a management problem. We report here the echocardiographic presentation, illustrate the surgical findings and suggest technical tips for management of this rare condition.