Author + information
- Received March 30, 2016
- Revision received June 27, 2016
- Accepted June 30, 2016
- Published online May 1, 2017.
- Ida S. Leren, MD, PhDa,b,
- Jørg Saberniak, MDa,b,c,
- Trine F. Haland, MDa,b,c,
- Thor Edvardsen, MD, PhDa,b,c and
- Kristina H. Haugaa, MD, PhDa,b,c,∗ ()
- aDepartment of Cardiology and Center for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway
- bUniversity of Oslo, Oslo, Norway
- cInstitute for Surgical Research, Oslo University Hospital, Rikshospitalet, Oslo, Norway
- ↵∗Address for correspondence:
Dr. Kristina H. Haugaa, Department of Cardiology, Oslo University Hospital, Rikshospitalet, P.O. Box 4950 Nydalen, NO-0424 Oslo, Norway.
Objectives The aim of this study was to investigate early markers of arrhythmic events (AEs) and improve risk stratification in early arrhythmogenic right ventricular cardiomyopathy (ARVC).
Background AEs are frequent in patients with ARVC, but risk stratification in subjects with early ARVC is challenging.
Methods Early ARVC disease was defined as possible or borderline ARVC diagnosis according to the ARVC Task Force Criteria 2010. We performed resting and signal averaged electrocardiogram (ECG). Using echocardiography, we assessed right ventricular (RV) outflow tract diameter and right ventricular basal diameter (RV diameter). Global longitudinal strain and mechanical dispersion (MD) from strain echocardiography were assessed in both the right and left ventricle. AEs were defined as documented ventricular tachycardia, cardiac syncope, or aborted cardiac arrest.
Results Of 162 included subjects with ARVC (41 ± 16 years of age, 47% female), 73 had early ARVC, including mutation positive family members not fulfilling definite ARVC diagnosis. AEs occurred in 15 (21%) subjects with early ARVC. Those with AEs in early disease had larger RV diameter (40 ± 4 mm vs. 37 ± 5 mm), more pronounced RVMD (39 ± 15 ms vs. 26 ± 11 ms), and more pathological signal averaged ECGs compared with those without AEs (all p ≤ 0.05). Adding measurements of RV diameter and RVMD to electrical parameters improved identification of subjects with AEs compared with electrical parameters alone (p = 0.05).
Conclusions ECG parameters, RV diameter, and RVMD were markers of previous arrhythmic events in patients with early ARVC. A combination of electrical and echocardiographic parameters improved identification of subjects with AEs in early ARVC disease.
- arrhythmic risk
- arrhythmogenic right ventricular cardiomyopathy
- signal averaged ECG
- ventricular arrhythmias
This work was supported by the Norwegian Health Association, Norway; the Norwegian Heart and Lung Patient Association, Norway; Simon Fougner Hartmann’s Family Foundation, Norway; and the Center for Cardiological Innovation, funded by the Norwegian Research Council. All authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received March 30, 2016.
- Revision received June 27, 2016.
- Accepted June 30, 2016.
- 2017 American College of Cardiology Foundation