Abstract 20749: Prognostic Implications of Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Long Term Follow up at a Tertiary Hospital in Japan
Introduction: Clinical manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) vary with age and stage of disease. Recently published the modified Task Force Criteria provides guidance on the role of emerging diagnostic modalities and advances in the ARVC/D. However, clinical significance of the diagnostic criteria is unknown.
Hypothesis: Since the diagnostic criteria reflect a nature of the pathologic condition of ARVC/D, its severity is associated with an increased risks of adverse outcome.
Methods: From a tertiary center in Japan between 1990 and 2007, we enrolled patients with ARVC/D classified based on the major and minor in the modified Task Force Criteria 2010. We defined the severity of diagnostic criteria as “ARVC/D score” for two points in major and one point in minor. Patients were followed up to determine adverse outcomes (life-threatening ventricular arrhythmia, heart failure hospitalization, and all-cause death).
Results: We studied 98 patients with ARVC/D (75 men; age at first diagnosis, 46.7±14.0 years; ARVC/D score, 4 to 12). During follow up period of 9.2±5.4 years, 34 patients had ventricular arrhythmia, 14 were hospitalized for heart failure and 10 patients died. ARVC/D score was significantly associated with an increased risk of adverse outcomes (per one score; HR 1.51, 95%CI 1.29–1.77, P<0.001), even after adjusting for age and left ventricular ejection fraction (per one score; HR 1.50, 95%CI 1.28–1.77, P<0.001). In addition, we defined 3 groups of patients according to the ARVC/D score: group 1 (ARVC/D score 4 to 6, N=47), group 2 (7 to 9, N=37), group 3 (10 to 12, N=14). There was a tendency towards a higher risk of adverse outcomes in patients with higher score (P<0.001, Figure).
Conclusions: The magnitude of the pathologic condition according to the Task Force Criteria for ARVC/D contributes to improve the diagnostic accuracy and may have prognostic value.
- © 2010 by American Heart Association, Inc.