Abstract 18417: Clinical Presentation, Cardiac Phenotype and Long Term Prognosis of Patients With Late Onset Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Introduction: The phenotypic features and clinical course of elderly Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) patients (age> 50 years at diagnosis) is unknown.
Methods: Demographic, phenotypic and genotypic data of 502 patients with ARVC diagnosis (2010 Task Force Criteria) from Johns Hopkins and Utrecht (NL) ARVD/C registries was obtained and long term clinical outcomes ascertained.
Results: Late presentation was seen in 104 (21%) patients (38% with PKP2 mutation; no DSP or DSG2 mutation carrier) with 3% being ≥ 65 years at diagnosis. Sustained ventricular tachycardia (VT) is the major (43%) mode of presentation in the elderly while cardiac syncope is infrequent (p<0.001). The elderly are significantly less likely to harbor a pathogenic mutation (53%; p=0.005), have precordial T wave repolarization changes (p<0.001) and have lower ventricular ectopy burden (median count 1503 vs 2497; p=0.026) compared to younger patients. In the elderly, over 7±5 years of follow up, 68(65%) patients experienced sustained ventricular arrhythmias with similar (p=0.08) arrhythmia free survival at 5 years of follow up (73% vs.60% in younger). LV dysfunction and heart failure was seen in 24(32%) and 15(14%) patients respectively without need for cardiac transplantation. Male gender (OR 2.12, 95%CI (1.3-3.45); p=0.002), absence of a pathogenic mutation (OR 1.94, 95%CI (1.20, 3.12); p=0.006) and family member status (OR 2.09, 95%CI (1.24, 3.51); P=0.006) were independently associated with late presentation. In the elderly, EP study inducibility (<0.001), major RV structural disease (p=0.022), absent family history (p<0.001), male gender (p=0.002) and pathogenic mutation (p=0.001) were associated with increased risk of ventricular arrhythmias while ECG abnormalities and ventricular ectopy (p=0.489) were not.
Conclusion: One fifth of all ARVD/C patients present after 50 years of age, often with sustained VT and are less likely to have prior syncope or identifiable pathogenic mutation. In ARVD/C, late presentation does not confer a benign prognosis and is associated with high arrhythmic risk. Electrocardiographic changes and ventricular ectopy are significantly less common in the elderly and are not associated with arrhythmic events.
Author Disclosures: A. Bhonsale: None. A.S. te Riele: None. A. Sawant: None. J. Groeneweg: None. C.A. James: None. B. Murray: None. C. Tichnel: None. D. Dooijes: None. J.V. Heijden: None. H. Tandri: None. P.V. Tintelen: None. D.P. Judge: None. R. Hauer: None. H. Calkins: Other Research Support; Modest; Medtronic Inc., St. Jude Medical. Consultant/Advisory Board; Modest; Medtronic Inc., St. Jude Medical..
- © 2015 by American Heart Association, Inc.