%0 Case Reports
%T Ellis van creveld syndrome: Cardiac anomalies and anesthetic implications.
%A Guha A
%A Malhotra R
%A Saxena R
%A Keshri VK
%J Ann Card Anaesth
%V 26
%N 3
%D 2023 Jul-Sep
%M 37470539
暂无%R 10.4103/aca.aca_166_22
%X Ellis-Van Creveld syndrome (EVC), also known as mesoectodermal dysplasia, is a rare autosomal recessive disorder with a tetrad of clinical features, comprising dwarfism, polydactyly, ectodermal dysplasia with sparse hair, hypoplastic nails and enamel, hypodontia and conical teeth and congenital heart disease (CHD). We report an 18-year-old girl with short stature and polydactyly, who got admitted to our hospital with shortness of breath on exertion for the last 2 years. On echocardiography, a partial atrioventricular canal (AV canal) defect was diagnosed, which was repaired surgically. The patient had an uneventful perioperative period.