Different underlying aetiologies in patients presenting with ventricular tachycardia fulfilling task force criteria for arrhythmogenic right ventricular cardiomyopathy: initial suspicion based on the 12-lead electrocardiogram
EP Europace, vol. 27
Abstract
AIMS: The task force criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) are highly sensitive but lack specificity. Atypical RV involvement (aRVi) may indicate different underlying aetiologies and prognosis, requiring specific therapeutic interventions. We aimed to evaluate the role of the baseline 12-lead ECG for initial suspicion of aRVi. METHODS: From the Netherlands Heart Institute Arrhythmogenic Cardiomyopathy (NHI-ACM) registry, patients were selected who (i) fulfilled TFC for definite ARVC, (ii) presented with sustained ventricular tachycardia (VT), and (iii) underwent genetic testing. The first available ECG after VT was evaluated. PR prolongation ≥220 ms and/or a surface area of the maximum R'-wave in V1-V3 of ≥1.65 mm2 was defined as an aRVi-ECG. Patients with an ARVC-related pathogenic/likely pathogenic variant (P/LP+) were classified as 'ARVC'. Data of P/LP- were reviewed by an expert panel and classified as either 'ARVC' or 'different aetiology' based on consensus. RESULTS: A total of 159 patients were included (122 P/LP+ and 37 P/LP- patients). Nineteen patients had an aRVi-ECG [11 (9%) P/LP+ vs. 8 (22%) P/LP-, P = 0.038]. Of the P/LP- patients, 17 (46%) were classified as 'different aetiology' (e.g. myocarditis, ischaemia, sarcoidosis), including all 8 patients with an aRVi-ECG. Among the P/LP+ patients with an aRVi-ECG, 46% carried the p.Arg14del phospholamban pathogenic variant, and 64% died compared to 15 and 19% of P/LP+ patients without an aRVi-ECG, respectively (both P < 0.01). CONCLUSION: In P/LP- patients presenting with VT and fulfilling TFC, an aRVi-ECG should raise suspicion for a different underlying aetiology. In P/LP+ patients, an aRVi-ECG may identify those with poor outcome.
Authors 10
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Jarieke C. Hoogendoorn Aachen Department of Cardiology Willem Einthoven Center for Cardiac Arrhythmia Research and Management
Leiden University Medical Center
Affiliation as printed
Department of Cardiology, Willem Einthoven Center for Cardiac Arrhythmia Research and Management (WECAM), Leiden University Medical Center , Albinusdreef 2, 2333 ZA Leiden ,
Department of Cardiology, Willem Einthoven Center for Cardiac Arrhythmia Research and Management (WECAM), Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands
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Utrecht University · University Medical Center Utrecht
Affiliation as printed
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht , Utrecht ,
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands
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Utrecht University · University Medical Center Utrecht · Haga Hospital
Affiliation as printed
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht , Utrecht ,
Department of Cardiology, Haga Ziekenhuis , Den Haag ,
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands
Department of Cardiology, Haga Ziekenhuis, Den Haag, The Netherlands
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Amsterdam UMC Location University of Amsterdam · ERN GUARD-Heart · Amsterdam Cardiovascular Sciences
Affiliation as printed
Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias , Amsterdam ,
Department of Cardiology, Heart Centre, Amsterdam UMC Location University of Amsterdam , Meibergdreef 9, Amsterdam ,
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart )
Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, The Netherlands
Department of Cardiology, Heart Centre, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, Amsterdam, The Netherlands
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University Medical Center Groningen · University of Groningen
Affiliation as printed
Department of Cardiology, University Medical Center Groningen, University of Groningen , Groningen ,
Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
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Affiliation as printed
Department of Cardiology, Thorax Center, Cardiovascular Institute, Erasmus Medical Center , Rotterdam ,
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart )
Department of Cardiology, Thorax Center, Cardiovascular Institute, Erasmus Medical Center, Rotterdam, The Netherlands
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Utrecht University · University Medical Center Utrecht
Affiliation as printed
Department of Genetics, University Medical Center Utrecht, University of Utrecht , Utrecht ,
Department of Genetics, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands
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Utrecht University · University Medical Center Utrecht
Affiliation as printed
Department of Genetics, University Medical Center Utrecht, University of Utrecht , Utrecht ,
Department of Genetics, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands
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Utrecht University · University Medical Center Utrecht · ERN GUARD-Heart
Affiliation as printed
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht , Utrecht ,
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart )
Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands
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Katja Zeppenfeld corresponding Aachen Department of Cardiology Willem Einthoven Center for Cardiac Arrhythmia Research and Management
Leiden University Medical Center
Affiliation as printed
Department of Cardiology, Willem Einthoven Center for Cardiac Arrhythmia Research and Management (WECAM), Leiden University Medical Center , Albinusdreef 2, 2333 ZA Leiden ,
Department of Cardiology, Willem Einthoven Center for Cardiac Arrhythmia Research and Management (WECAM), Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands
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