Introduction:
Ellis–van Creveld (EVC) syndrome is a rare skeletal and ectodermal dysplasia, with a prevalence of about 1 in 20,000 births, and is common among the Old Order Amish of Lancaster County, Pennsylvania (1). The skeletal dysplasia presents at birth with short limbs, and affected individuals have postaxial polydactyly of the hands and sometimes of the feet, and dysplastic teeth and nails (1). This condition was first described by Richard Ellis and Simon Van Creveld in 1940 (2). Congenital heart defects (CHD) are encountered in about 50-60 % of the cases, and include common atrium, atrioventricular septal defect (AVSD) with anomalies of the mitral and tricuspid valves, patent ductus arteriosus, ventricular septal defect, atrial septal defect, and hypoplastic left heart syndrome (3). Moreover, systemic and/or pulmonary venous abnormalities are common as well (4). Double orifice mitral valve (DOMV) is a rare congenital anomaly of the subvalvar mitral valve apparatus in which there is an accessory bridge of fibrous tissue that partially or completely divides the mitral valve into two orifices which functionally may result in regurgitation or stenosis of the mitral valve. It has been reported that AVSD was the most common CHD associated with DOMV (5). Herein, we introduce the case of a 7-year-old girl with EVC Syndrome who underwent open heart surgery for repair of partial atrioventricular septal defect (PAVSD) with DOMV and common atrium.