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Document Details : Title: Symptomatic arrhytmogenic right ventricular dysplasia/cardiomyopathy Subtitle: A two-centre retrospective study of 15 symptomatic ARVD/C cases and focus on the diagnostic value of MRI in symptomatic ARVD/C patients Author(s): W. Dewilde , L. Boersma , J. Delanote , P. Pollet , B. Scholzel , E. Wever , Y. Vandekerckhove Journal: Acta Cardiologica Volume: 63 Issue: 2 Date: 2008 Pages: 181-189 DOI: 10.2143/AC.63.2.2029526 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. Methods and 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. Conclusion — 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. |
