Author + information
- Received October 15, 2019
- Revision received January 13, 2020
- Accepted February 20, 2020
- Published online April 29, 2020.
- Jarieke C. Hoogendoorn, MDa,
- Marek Sramko, MD, PhDa,b,
- Jeroen Venlet, MDa,
- Konstantinos C. Siontis, MDc,
- Saurabh Kumar, BSc (Med)/MBBS, PhDd,
- Robin Singh, MDd,
- Ikutaro Nakajima, MDe,
- Sebastiaan R.D. Piers, MD, PhDa,
- Marta de Riva Silva, MDa,
- Claire A. Glashan, MDa,
- Thomas Crawford, MDc,
- Usha B. Tedrow, MDd,
- William G. Stevenson, MDe,
- Frank Bogun, MD, PhDc and
- Katja Zeppenfeld, MD, PhDa,∗ ()
- aDepartment of Cardiology, Willem Einthoven Center for Cardiac Arrhythmia Research and Management, Leiden University Medical Center, Leiden, the Netherlands
- bDepartment of Cardiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
- cDepartment of Cardiology, University of Michigan, Ann Arbor, Michigan
- dDepartment of Cardiology, Brigham and Women’s Hospital, Boston, Massachusetts
- eDepartment of Cardiology, Vanderbilt Medical University Center, Nashville, Tennessee
- ↵∗Address for correspondence:
Dr. Katja Zeppenfeld, Department of Cardiology (B4-P),Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, the Netherlands.
Objectives This study sought to investigate the value of electroanatomical voltage mapping (EAVM) to distinguish cardiac sarcoidosis (CS) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in patients with ventricular tachycardia from the right ventricle (RV).
Background CS can mimic ARVC. Because scar in ARVC is predominantly subepicardial, this study hypothesized that the relative sizes of endocardial low bipolar voltage (BV) to low unipolar voltage (UV) areas may distinguish CS from ARVC.
Methods Patients with CS affecting the RV (n = 14), patients with gene-positive ARVC (n = 13), and a reference group of patients without structural heart disease (n = 9) who underwent RV endocardial EAVM were included. RV region-specific BV and UV cutoffs were derived from control subjects. In CS and ARVC, segmental involvement was determined and low-voltage areas were measured, using <1.5 mV for BV and <3.9 mV, <4.4 mV, and <5.5 mV for UV. The ratio between low BV and low UV area was calculated generating 3 parameters: Ratio3.9, Ratio4.4 and Ratio5.5, respectively.
Results In control subjects, BV and UV varied significantly among RV regions. The basal septum was involved in 71% of CS patients and in none of ARVC patients. Ratio5.5 discriminated CS from ARVC the best. An algorithm including Ratio5.5 ≥0.45 and basal septal involvement identified CS with 93% sensitivity and 85% specificity. This was validated in a separate population (CS [n = 6], ARVC [n = 10]) with 100% sensitivity and 100% specificity.
Conclusions EAVM provides detailed information about scar characteristics and scar distribution in the RV. An algorithm combining Ratio5.5 (area BV <1.5 mV/area UV <5.5 mV) and bipolar basal septal involvement allows accurate diagnosis of (isolated) CS in patients presenting with monomorphic ventricular tachycardia from the RV.
- arrhythmogenic right ventricular cardiomyopathy
- cardiac sarcoidosis
- electroanatomical voltage mapping
- right ventricle
- ventricular tachycardia
The Department of Cardiology from Leiden University Medical Center receives unrestricted grants from Edwards Lifesciences, Biotronik, Medtronik, Boston Scientific, and Biosense Webster. Dr. Sramko was supported by the Research Fellowship of the European Society of Cardiology (2017/2018). Dr. Tedrow has received honoraria from Abbott Medical and Biosense Webster; and has served as a consultant for Thermedical. Dr. Stevenson has received speaking honoria from Boston Scientific, Abbott, Biotronik, Johnson and Johnson,; and is a coholder of a patent for irrigated needle ablation that is consigned to Brigham Hospital. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Francis Marchlinski, MD, served as Guest Editor for this paper.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received October 15, 2019.
- Revision received January 13, 2020.
- Accepted February 20, 2020.
- 2020 American College of Cardiology Foundation
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