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Arrhythmic risk stratification in arrhythmogenic cardiomyopathy: new predictors for left-sided variants?
- Source :
- European Heart Journal
- Publication Year :
- 2021
-
Abstract
- Aims This study aims to improve risk stratification for primary prevention implantable cardioverter defibrillator (ICD) implantation by developing a new mutation-specific prediction model for malignant ventricular arrhythmia (VA) in phospholamban (PLN) p.Arg14del mutation carriers. The proposed model is compared to an existing PLN risk model. Methods and results Data were collected from PLN p.Arg14del mutation carriers with no history of malignant VA at baseline, identified between 2009 and 2020. Malignant VA was defined as sustained VA, appropriate ICD intervention, or (aborted) sudden cardiac death. A prediction model was developed using Cox regression. The study cohort consisted of 679 PLN p.Arg14del mutation carriers, with a minority of index patients (17%) and male sex (43%), and a median age of 42 years [interquartile range (IQR) 27–55]. During a median follow-up of 4.3 years (IQR 1.7–7.4), 72 (10.6%) carriers experienced malignant VA. Significant predictors were left ventricular ejection fraction, premature ventricular contraction count/24 h, amount of negative T waves, and presence of low-voltage electrocardiogram. The multivariable model had an excellent discriminative ability {C-statistic 0.83 [95% confidence interval (CI) 0.78–0.88]}. Applying the existing PLN risk model to the complete cohort yielded a C-statistic of 0.68 (95% CI 0.61–0.75). Conclusion This new mutation-specific prediction model for individual VA risk in PLN p.Arg14del mutation carriers is superior to the existing PLN risk model, suggesting that risk prediction using mutation-specific phenotypic features can improve accuracy compared to a more generic approach.<br />Graphical Abstract Malignant VA risk prediction in PLN p.Arg14del carriers. With the mutation specific risk factors low and high risk groups can be identified. PLN, Phospholamban; VA, Ventricular arrhythmia.
- Subjects :
- medicine.medical_specialty
Arrhythmic risk
business.industry
Cardiomyopathy
MEDLINE
Arrhythmias
medicine.disease
Left sided
Risk Assessment
Stratification (mathematics)
Electrocardiography
Sudden cardiac death
Clinical Research
Internal medicine
Implantable cardioverter defibrillator
medicine
Cardiology
Humans
AcademicSubjects/MED00200
Cardiology and Cardiovascular Medicine
business
Arrhythmogenic Right Ventricular Dysplasia
Risk stratification
Phospholamban
Subjects
Details
- ISSN :
- 15229645
- Volume :
- 42
- Issue :
- 29
- Database :
- OpenAIRE
- Journal :
- European heart journal
- Accession number :
- edsair.doi.dedup.....b3e10acd53a451a911968d321fd83f03