Abstract 13457: Electrocardiographic and Imaging Diagnostic Criteria are Predictive of Syncope, Ventricular Tachycardia or Aborted Cardiac Arrest in the Nordic Arrhythmogenic Right Ventricular Cardiomyopathy Registry
Background: Revision of arrhythmogenic right ventricular cardiomyopathy (ARVC) Task Force diagnostic criteria in 2010 (TF2010) increased the sensitivity for detection of patients at early stages of the disease. Whether this is associated with increased detection of patients at risk of severe manifestations of the disease, has not been fully clarified. Our aim was to assess the relation between baseline diagnostic criteria and severe debut of the disease in patients enrolled in the Nordic ARVC Registry.
Methods: Patients with definite ARVC by TF2010 enrolled in the registry in Denmark, Norway and Sweden were included in the analysis: n=139 (102 families), age 48±15 years, 57% male. Patients were defined as symptomatic based on the occurrence of syncope, documented ventricular tachycardia (VT) or aborted cardiac arrest (ACA) by enrolment. Using this definition, the performance of TF2010 criteria was tested for prediction of symptoms.
Results: The study population comprised 102 probands and 37 family members, of whom 24 were identified via family screening (17%). Initial disease manifestations were VT (n=43, 31%), syncope (n=16, 12%) or ACA (n=12, 9%), while 68 (49%) patients had not experienced any of these symptoms at baseline. Median age at first symptom was 40 [range 17-75] years. ACA occurred earlier (28 [range 18-50] years) than syncope (41[18-75] years) or VT (42[17-75] years) as the first symptom (p=0.013). Syncope, VT or ACA as an initial manifestation of ARVC were independently associated with the presence of major depolarisation (OR=2.54 95%CI 1.03-6.31, p=0.044), repolarisation (OR=2.34 95%CI 1.11-4.93, p=0.025) or imaging (OR=5.45 95%CI 2.32-12.78, p<0.001) criteria. The family history of sudden death or ARVC in a 1st degree relative did not predict symptoms. The freedom from any imaging criteria was strongly associated with freedom from symptoms (OR=0.18 95%CI 0.08-0.43, p<0.001).
Conclusion: In patients with definite ARVC enrolled in the Nordic ARVC Registry the presence of major imaging, depolarisation or repolarisation diagnostic criteria were independently associated with syncope, VT or ACA as an initial manifestation of the disease. A family history of sudden death or ARVC, however, was not associated with a severe disease debut.
- © 2012 by American Heart Association, Inc.