Abstract

Ellis-van Creveld (EVC) syndrome is a rare genetic abnormality characterized by a tetrad of chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital heart disease. Common atrium is an uncommon cardiac malformation, and yet it is commonly found in patients with EVC syndrome. This rare condition is inherited as an autosomal recessive trait. This report describes a case of common atrium with unusual electrocardiographic findings in a patient with EVC syndrome. This 17-year-old male of Indian origin born of a consanguineous marriage had short stature, genu valgum, polydactyly, gingival hypertrophy, multiple frenula, common atrium, and right axis deviation of QRS with clockwise depolarization in electrocardiogram.

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