ArticleEuropean heart journal2022
Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator.
Article in European heart journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 50 papers, 1 of them a synthesis that pooled it.
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Who cites it
50 citing papers in PubMed, 1 synthesis or guideline pooled it, 99 citations in OpenAlex.
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- Arrhythmogenic right ventricular cardiomyopathy.European heart journal · 2026Review
- Periostin-CCL3 Feedforward Signaling Loop Promotes Cardiac Fibrosis and Cardiomyocyte Necroptosis in Arrhythmogenic Cardiomyopathy.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Yield of Family Screening in Arrhythmogenic Right Ventricular Cardiomyopathy Without a Validated Genetic Cause.Circulation. Arrhythmia and electrophysiology · 2026Article
- Genotype-specific digital twins for arrhythmia ablation targeting in arrhythmogenic right ventricular cardiomyopathy.Communications medicine · 2026Article
- Body surface potential mapping of ventricular depolarization and repolarization in phospholamban and plakophilin-2 cardiomyopathy.Heart rhythm O2 · 2026Article
- Pediatric dilated cardiomyopathy and the progression to heart failure: mechanisms and interventions focusing on the "gene-inflammation-metabolism" axis.Frontiers in cardiovascular medicine · 2026Review
- Genetics of Sudden Cardiac Death.Diseases (Basel, Switzerland) · 2025Review
- Pericardial Fat Radiomics to Predict Left Ventricular Involvement and Provide Incremental Prognostic Value in ARVC.Diagnostics (Basel, Switzerland) · 2025Article
- What Should a Clinical Cardiologist Know About Cardiogenetics?Journal of the American Heart Association · 2025Review
- Genotype-specific Digital Twins for Arrhythmia Ablation Targeting in Arrhythmogenic Right Ventricular Cardiomyopathy.Research square · 2025Article
- Review
- Arrhythmogenic Cardiomyopathy and Biomarkers: A Promising Perspective?Journal of clinical medicine · 2025Review
- Arrhythmogenic Cardiomyopathy: Towards Genotype Based Diagnoses and Management.Journal of cardiovascular electrophysiology · 2025Review
- Leveraging artificial intelligence for risk stratification of inherited cardiomyopathies in under-resourced settings.Heart rhythm O2 · 2025Article
- Cardiology's best friend: Using naturally occurring disease in dogs to understand heart disease in humans.Journal of molecular and cellular cardiology plus · 2025Review
- Family Screening in Relatives at Risk for Plakophilin-2-Associated Arrhythmogenic Right Ventricular Cardiomyopathy.Circulation · 2025Article
- China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy: The ChinaCORE ACM Registry.JACC. Asia · 2025Article
- Arrhythmic Risk Stratification in Patients with Arrhythmogenic Cardiomyopathy.Diagnostics (Basel, Switzerland) · 2025Review
- Predicted Risk of Ventricular Arrhythmias in a Genome-First Population With Genetic Risk for Arrhythmogenic Right Ventricular Cardiomyopathy.Circulation. Arrhythmia and electrophysiology · 2025Article
Corrections and comments
- Commented on by
- Erratum issued
Authors and funding
41 authors at 16 institutions in 10 countries.
Funding
Abstract
aimsTo study the impact of genotype on the performance of the 2019 risk model for arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS AND
resultsThe study cohort comprised 554 patients with a definite diagnosis of ARVC and no history of sustained ventricular arrhythmia (VA). During a median follow-up of 6.0 (3.1,12.5) years, 100 patients (18%) experienced the primary VA outcome (sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator intervention, aborted sudden cardiac arrest, or sudden cardiac death) corresponding to an annual event rate of 2.6% [95% confidence interval (CI) 1.9-3.3]. Risk estimates for VA using the 2019 ARVC risk model showed reasonable discriminative ability but with overestimation of risk. The ARVC risk model was compared in four gene groups: PKP2 (n = 118, 21%); desmoplakin (DSP) (n = 79, 14%); other desmosomal (n = 59, 11%); and gene elusive (n = 160, 29%). Discrimination and calibration were highest for PKP2 and lowest for the gene-elusive group. Univariable analyses revealed the variable performance of individual clinical risk markers in the different gene groups, e.g. right ventricular dimensions and systolic function are significant risk markers in PKP2 but not in DSP patients and the opposite is true for left ventricular systolic function.
conclusionThe 2019 ARVC risk model performs reasonably well in gene-positive ARVC (particularly for PKP2) but is more limited in gene-elusive patients. Genotype should be included in future risk models for ARVC.
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