Chondroectodermal Dysplasia (Ellis—van Creveld Syndrome) With a Complex Cardiac Malformation

A 4-month-old girl with chondroectodermal dysplasia (dwarfism, bimanual polydactyly and ectodermal dysplasia) (Ellisvan Creveld syndrome) and congenital heart disease, consisting of anamalous pulmonary venous return, single atrium and a persistent left superior vena cava, is described. A review of the literature concerning chondroectodermal dysplasia reveals an incidence of congenital heart disease in more than 50% of patients.