title: [Arrhythmogenic right ventricular dysplasia/cardiomyopathy - diagnosis in childhood].
abstract: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare, but important cause for sudden death in adolescents and young adults. Part of the patients affected show the pattern of autosomal-dominant inheritance. Two pediatric patients with ARVD/C are presented who may reflect the spectrum of clinical presentation of ARVD/C in childhood resulting in difficulties or even delay to establish the correct diagnosis. One patient with a sporadic form of ARVD/C presented with a syncope and spontaneous as well as inducible ventricular tachycardia. On the ECG, an epsilon wave could be identified. An automatic cardioverter defibrillator was implanted. The second patient had a familiar form of ARVD/C with no symptoms. There was a history of frequent sudden deaths in this family. Biopsies of the right ventricular myocardium showed fibrosis with deposition of fatty tissue. There was clear evidence of ARVD/C in the necropsy of the patient's aunt. Therapy with propanolol was started in this patient.
authors.affiliation: Abteilung Kinderheilkunde III Pädiatrische Kardiologie und Pädiatrische Intensivmedizin Medizinische Hochschule Hannover Carl-Neuberg-Strasse 1 30625 Hannover, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Kriebel
authors.forename: T
authors.initials: T
authors.lastname: Korte
authors.forename: R
authors.initials: R
authors.lastname: Kandolf
authors.forename: C
authors.initials: C
authors.lastname: Jux
authors.forename: B
authors.initials: B
authors.lastname: Windhagen-Mahnert
authors.forename: R
authors.initials: R
authors.lastname: Bökenkamp
authors.forename: H
authors.initials: H
authors.lastname: Bertram
authors.forename: T
authors.initials: T
authors.lastname: Paul
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000628:therapy; D001344:Autopsy; D001706:Biopsy; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005260:Female; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D010375:Pedigree; D011433:Propranolol / Q000627:therapeutic use; D012737:Sex Factors; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000889:Anti-Arrhythmia Agents; D014665:Vasodilator Agents; D011433:Propranolol
vernacular_title: Arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie. Diagnostik im Kindesalter.