The phospholamban (PLN) p.Arg14del risk model; Longitudinal validation and reevaluation of predictors regarding sudden cardiac death (SCD)
| العنوان: | The phospholamban (PLN) p.Arg14del risk model; Longitudinal validation and reevaluation of predictors regarding sudden cardiac death (SCD) |
|---|---|
| المؤلفون: | M Y C Van Der Heide, T E Verstraelen, F H M Van Lint, L P Bosman, R De Brouwer, V M Proost, T Germans, C Dickhoff, B A Schoonderwoerd, A C Houweling, J R Gimeno-Blanes, R A De Boer, M G P J Cox, P Van Tintelen, A A M Wilde |
| المصدر: | Europace. 25 |
| بيانات النشر: | Oxford University Press (OUP), 2023. |
| سنة النشر: | 2023 |
| مصطلحات موضوعية: | Physiology (medical), Cardiology and Cardiovascular Medicine |
| الوصف: | Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): PSIDER (ZOn-MW) PREDICT2 (Hartstichting) Background: The PLN p.Arg14del risk model is a mutation-specific risk model developed to predict individual malignant ventricular arrhythmia (VA) risk to inform decision-making for primary prevention implantable cardioverter defibrillator (ICD) implantation. While the risk model has proven to be valid, two main points of criticisms remain to be discussed: first, the risk model is only validated at baseline and second, appropriate ICD therapy and sustained ventricular tachycardia (VT) Purpose: To evaluate the diagnostic performance of the PLN p.Arg14del risk model longitudinal at 3 year follow-up and to reevaluate the predictors regarding SCD. Methods: Clinical data of 278 PLN p.Arg14del mutation carriers with no history of malignant VA at both baseline and 3 year follow-up were collected. During a median follow-up of 4 years (Interquartile range (IQR) 1.8-6.5), 31 (11%) carriers experienced malignant VA, defined as sustained VA, appropriate ICD intervention, or (aborted) SCD. The reevaluation of the predictors with the 3 year follow up data revealed hazard ratio’s (HR) that were similar to the baseline PLN p.Arg14del risk model for left ventricular ejection fraction (HR 1.10 [95% CI, 1.06-1.12], p To reevaluate the predictive performance of the PLN p.Arg14del risk model for a more strict surrogate of SCD we redefined the outcome as VT >250 beats per minute sustained (lasting ≥30 seconds) or terminated by ICD, ventricular fibrillation, aborted SCD and SCD. We included 669 PLN p.Arg14del carriers with no presentation or history of sustained VA or (aborted) SCD. During a median follow-up of 4.75 years (IQR 1.9-7.9), 33 (5%) carriers experienced the outcome. Results of the univariable analysis are shown in table 1. The PLN p.Arg14del risk model yielded a C-statistic of 0.75 (95% CI 0.70-0.80). Adding risk factors sex, age and sustained VT Conclusion: The PLN p.Arg14del risk model was validated longitudinal at 3 year follow-up and it held its performance with a more strict definition of SCD. |
| تدمد: | 1532-2092 1099-5129 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::54e0bf92a954dbec5bfdba3d9f1637a4 https://doi.org/10.1093/europace/euad122.344 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi...........54e0bf92a954dbec5bfdba3d9f1637a4 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15322092 10995129 |
|---|