Cardiac transplantation in twins with autosomal dominant Emery-Dreifuss muscular dystrophy.
Emery-Dreifuss muscular dystrophy (EDMD) is a neuromuscular disorder resulting in progressive muscle weakness, contractures, and cardiomyopathy. We report successful cardiac transplantation in identical twin brothers with autosomal dominant EDMD, complicated by ventricular arrhythmias and end-stage cardiomyopathy. Early recognition of progressive cardiac disease and subsequent cardiac transplantation are lifesaving in children with EDMD.
Kichuk Chrisant
Maryanne R
MR
Department of Pediatric Cardiology, Children's Hospital of Cleveland Clinic Foundation, Cleveland, Ohio, USA. chrisant@email.chop.edu
Drummond-Webb
Jonathan
J
Hallowell
Sara
S
Friedman
Neil R
NR
D002311:Cardiomyopathy, Dilated / Q000209:etiology / Q000601:surgery*; D002648:Child; D004200:Diseases in Twins / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000150:complications*; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000601:surgery; D014430:Twins, Monozygotic