title: Symptomatic arrhythmogenic right ventricular dysplasia/cardiomyopathy. A two-centre retrospective study of 15 symptomatic ARVD/C cases and focus on the diagnostic value of MRI in symptomatic ARVD/C patients.
abstract: BACKGROUND
  Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is associated with sudden death in the young and heart failure in the elderly. The purpose is to review 15 symptomatic ARVD/C cases and also to describe the use of MRI as a diagnostic tool.
  
  
  RESULTS
  This retrospective analysis includes 15 patients who presented with symptomatic ARVD/C. Diagnosis was made upon the criteria proposed by the European Society of Cardiology. In all patients there was at least 1 or more abnormal MRI sign. The most frequent abnormalities were focal right ventricular dyskinesia (64%), MRI fatty infiltration (57%) and right ventricular aneurysm or right ventricular outflow tract microaneurysms (57%). Presenting symptoms were palpitations (60%), atypical chest pain (46%), syncope (40%), and aborted sudden death (26%). T-inversion in V2-V3 was seen in 60% of the patients. Thirteen patients (86%) received an ICD implantation. The mean follow-up per patient was 89 months, which resulted in a total follow-up of 111 patient years. Forty-six percent of the patients with an ICD had one or more appropriate shocks during follow-up. To this date no mortality was reported.
  
  
  CONCLUSIONS
  This retrospective study demonstrates that symptomatic ARVD/C patients typically present with symptoms of syncope, palpitations in association with ventricular tachycardia and in a quarter of the cases with aborted sudden cardiac death. The electrocardiogram mostly shows T inversion in the anterior leads. All patients were treated with medication and ICD-implantation or VT-ablation. The malignant nature of the disease in symptomatic ARVD/C patients is stressed by the fact that the presenting symptom is aborted sudden death in a quarter of the cases and the fact that nearly half of the patients with an ICD had at least one appropriate shock during follow-up. There was an abnormal MRI in 100% of the investigated patients. In 20% (3 patients), the MRI criterion (right ventricular dilatation/bulging/aneurysm) was necessary to meet the ESC criteria. Therefore it has become an important tool in our diagnostic work-up when ARVD/C is suspected. We also suggest a change in the diagnostic criteria of ARVD/C. Whereas fatty infiltration seen on RV biopsy is a major criterion, MRI fatty infiltration is not regarded as a diagnostic criterion by the task force to this day.
authors.affiliation: Dept. of Cardiology, AZ Sint Antonius Nieuwegein, The Netherlands. willemdewilde@yahoo.com
authors.forename: Willem
authors.initials: W
authors.lastname: Dewilde
authors.forename: Lucas
authors.initials: L
authors.lastname: Boersma
authors.forename: Joost
authors.initials: J
authors.lastname: Delanote
authors.forename: Peter
authors.initials: P
authors.lastname: Pollet
authors.forename: Bastiaan
authors.initials: B
authors.lastname: Scholzel
authors.forename: Eric
authors.initials: E
authors.lastname: Wever
authors.forename: Yves
authors.initials: Y
authors.lastname: Vandekerckhove
mesh_terms: D000293:Adolescent; D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control; D017147:Defibrillators, Implantable; D003937:Diagnosis, Differential; D004554:Electric Countershock / Q000295:instrumentation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors