title: Usefulness of inducible ventricular tachycardia to predict long-term adverse outcomes in arrhythmogenic right ventricular cardiomyopathy.
abstract: The role of the electrophysiologic (EP) study for risk stratification in patients with arrhythmogenic right ventricular cardiomyopathy is controversial. We investigated the role of inducible sustained monomorphic ventricular tachycardia (SMVT) for the prediction of an adverse outcome (AO), defined as the occurrence of cardiac death, heart transplantation, sudden cardiac death, ventricular fibrillation, ventricular tachycardia with hemodynamic compromise or syncope. Of 62 patients who fulfilled the 2010 Arrhythmogenic Right Ventricular Cardiomyopathy Task Force criteria and underwent an EP study, 30 (48%) experienced an adverse outcome during a median follow-up of 9.8 years. SMVT was inducible in 34 patients (55%), 22 (65%) of whom had an adverse outcome. In contrast, in 28 patients without inducible SMVT, 8 (29%) had an adverse outcome. Kaplan-Meier analysis showed an event-free survival benefit for patients without inducible SMVT (log-rank p = 0.008) with a cumulative survival free of an adverse outcome of 72% (95% confidence interval [CI] 56% to 92%) in the group without inducible SMVT compared to 26% (95% CI 14% to 50%) in the other group after 10 years. The inducibility of SMVT during the EP study (hazard ratio [HR] 2.99, 95% CI 1.23 to 7.27), nonadherence (HR 2.74, 95% CI 1.3 to 5.77), and heart failure New York Heart Association functional class II and III (HR 2.25, 95% CI 1.04 to 4.87) were associated with an adverse outcome on univariate Cox regression analysis. The inducibility of SMVT (HR 2.52, 95% CI 1.03 to 6.16, p = 0.043) and nonadherence (HR 2.34, 95% CI 1.1 to 4.99, p = 0.028) remained as significant predictors on multivariate analysis. This long-term observational data suggest that SMVT inducibility during EP study might predict an adverse outcome in patients with arrhythmogenic right ventricular cardiomyopathy, advocating a role for EP study in risk stratification.
authors.affiliation: Clinic for Cardiology, Cardiovascular Center, University Hospital Zurich, Zurich, Switzerland. ardan.saguner@usz.ch
authors.forename: Ardan M
authors.initials: AM
authors.lastname: Saguner
authors.forename: Argelia
authors.initials: A
authors.lastname: Medeiros-Domingo
authors.forename: Moritz A
authors.initials: MA
authors.lastname: Schwyzer
authors.forename: Chol-Jun
authors.initials: CJ
authors.lastname: On
authors.forename: Laurent M
authors.initials: LM
authors.lastname: Haegeli
authors.forename: Thomas
authors.initials: T
authors.lastname: Wolber
authors.forename: David
authors.initials: D
authors.lastname: Hürlimann
authors.forename: Jan
authors.initials: J
authors.lastname: Steffel
authors.forename: Nazmi
authors.initials: N
authors.lastname: Krasniqi
authors.forename: Sina
authors.initials: S
authors.lastname: Rüeger
authors.forename: Leonhard
authors.initials: L
authors.lastname: Held
authors.forename: Thomas F
authors.initials: TF
authors.lastname: Lüscher
authors.forename: Corinna
authors.initials: C
authors.lastname: Brunckhorst
authors.forename: Firat
authors.initials: F
authors.lastname: Duru
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D018572:Disease-Free Survival; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies; D013557:Switzerland / Q000453:epidemiology; D017180:Tachycardia, Ventricular / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D013997:Time Factors