Author + information
- Gabriela Orgeron,
- Cynthia James,
- Crystal Tichnell,
- Brittney Murray,
- Bryana Rivers,
- Harikrishna Tandri and
- Hugh Calkins
Background: The arrhythmic propensity of probands and family members with ARVD/C is not the same. Clinical predictors of sustained ventricular arrhythmias in follow-up may likewise differ.
Methods: The clinical course of 318 patients from the Johns Hopkins ARVD/C Registry with 1) definite ARVD/C by 2010 Task Force Criteria (TFC) and 2) implantable cardioverter defibrillators (ICD) was analyzed. Univariate cox regression analysis identified the variables significantly associated survival from appropriate ICD therapy in follow-up.
Results: Over a median follow-up of 7 years, 68% (175/258) probands and 28% (17/60) relatives had appropriate ICD interventions. Among probands, male sex (HR: 1.56, 95% CI: 1.33-2.14; p=0.006) and inducible ventricular arrhythmias on electrophysiology study (HR: 2.65 CI: 1.54-4.54; p<0.001) were associated with ICD therapy. Symptoms at presentation (HR: 1.08 CI: 0.55-2.09; p=0.817), pathogenic mutations (HR: 1.32 CI:0.97-1.80; p=0.074), ≥1000 premature ventricular contractions (PVC) on 24h holter monitor (HR: 1.51 CI: 0.83-2.74; p=0.170), ≥3 T wave inversions on electrocardiogram (HR: 1.34 CI: 0.86-2.07; p=0.191) and major cardiac magnetic resonance (CMR) TFC (HR: 0.98 CI: 0.62-1.54; p=0.953) were not significant. In contrast, among family members, ≥1000 PVC/24h (HR: 5.35 CI: 1.27-22.48; p=0.022) and major CMR TFC (HR: 5.05 CI: 1.52-16.71; p=0.008) were associated with ICD therapy, while sex (HR: 1.43 CI: 0.55-3.72; p=0.452), pathogenic mutations (HR: 2.54 CI: 0.59-10.89; p=0.210), symptomatic presentation (HR: 1.40 CI: 0.53-3.64; p=0.490), inducibility on electrophysiology study (HR: 2.02 CI: 0.62-6.51; p=0.237), ≥3 T wave inversions on electrocardiogram (HR: 3.82 CI: 0.89-16.28; p=0.069) were not significant.
Conclusions: Probands are more likely to require ICD therapy than their affected ARVD/C relatives (HR: 3.85, 95%CI: 2.39-6.20; p<0.001). While male sex and a positive electrophysiology study predict ventricular arrhythmias among probands, a high PVC burden on holter monitor and evidence of structural and functional right ventricular abnormalities predict appropriate ICD therapy in ARVD/C patients detected through family screening.
Moderated Poster Contributions
Arrhythmias and Clinical EP Moderated Poster Theater, Poster Hall, Hall C
Friday, March 17, 2017, 3:45 p.m.-3:55 p.m.
Session Title: Ventricular Arrhythmias and Sudden Death Prevention
Abstract Category: 5. Arrhythmias and Clinical EP: Devices
Presentation Number: 1174M-03
- 2017 American College of Cardiology Foundation