title: Primary endocardial fibroelastosis presenting in a young child as incessant ventricular tachycardia and dilated cardiomyopathy.
abstract: Endocardial fibroelastosis is a cardiomyopathy not commonly seen in the present age. We describe the case of a 4-year-old girl who presented with sustained incessant ventricular tachycardia, a severely dilated left ventricle, and cardiac dysfunction refractory to all medical management and even to ablation; she eventually underwent cardiac transplantation. The diagnosis was made only after histopathologic examination of the explanted heart showed clear evidence of endocardial fibroelastosis. Through this report, we would like to highlight the fact that primary endocardial fibroelastosis can masquerade as idiopathic dilated cardiomyopathy and that associated frequent premature ventricular contractions and nonsustained ventricular tachycardia require close monitoring. Progressive ventricular dilation and ventricular dysfunction can convey a poor prognosis. Sustained recalcitrant ventricular tachycardia in these patients can be a life-threatening event that requires emergent mechanical support and heart transplantation.
authors.affiliation: Section of Pediatric Cardiology, Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Detroit, Michigan 48201, USA.
authors.forename: Swati O
authors.initials: SO
authors.lastname: Arya
authors.forename: Peter P
authors.initials: PP
authors.lastname: Karpawich
authors.forename: Pooja
authors.initials: P
authors.lastname: Gupta
authors.forename: Sujatha
authors.initials: S
authors.lastname: Buddhe
authors.forename: Harinder R
authors.initials: HR
authors.lastname: Singh
authors.forename: Yaser
authors.initials: Y
authors.lastname: Hussein
authors.forename: Srinath T
authors.initials: ST
authors.lastname: Gowda
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D017115:Catheter Ablation; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D004695:Endocardial Fibroelastosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D000889:Anti-Arrhythmia Agents
keywords: Cardiomegaly; cardiomyopathy, dilated/etiology; endocardial fibroelastosis, primary/complications/diagnosis/etiology/pathology/virology; heart transplantation; mumps/complications; myocarditis/pathology; myocardium/pathology; premature ventricular contractions; prenatal exposure/delayed effects; ventricular tachycardia; verapamil