FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4306319421> ?p ?o ?g. }

• W4306319421 abstract "Abstract Introduction Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is associated with sudden cardiac death (SCD). The 2019 ARVC risk model has been proposed as a method to quantify arrhythmic risk, but the impact of genotype its performance has not been addressed. Purpose To study arrhythmic outcomes in patients with ARVC and the performance of the 2019 ARVC risk model in predefined genetic subgroups. Methods This is an international, retrospective observational cohort study on consecutively evaluated patients with ARVC recruited from 17 centres in 7 countries. Inclusion criteria were: (i) a definite diagnosis of ARVC according to the 2010 Task Force Criteria; (ii) no history of sustained ventricular arrhythmia (VA) prior to first assessment at the participating centre; (iii) a follow up period of ≥1 month; (iv) age of diagnosis ≥14 years. Sustained ventricular arrhythmia (sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator intervention, aborted SCD, or SCD) comprised the primary outcome (VA). Discriminative ability was assessed by Uno's concordance index (c-statistic) and calibration with the calibration plot slope. Fine-Gray regression was used to model the impact of clinical predictors on the arrhythmic outcome, in the context of competing risks (heart transplantation and non-arrhythmic death). The cumulative probability and 95% confidence intervals (95% CI) for the occurrence of an outcome were estimated using the Aalen-Johansen estimate in order to take into account competing risks. Results The study cohort comprised 554 ARVC patients. During a median follow-up of 6.0 [3.1,12.5] years, 100 patients (18%) experienced VA (Figure). Risk estimates for VA using the 2019 ARVC risk model showed good discriminative ability (c-statistic 0.75 (95% CI 0.70–0.81)) but with overestimation of risk (slope 0.46 (95% CI 0.33–0.63)). The ARVC risk model was compared in 4 gene groups: PKP2 (n=118, 21%); DSP (n=79, 14%); other desmosomal (n=59, 11%); and gene elusive (n=160, 29%). Discrimination and calibration were highest for PKP2 [c-statistic 0.83 (95% CI 0.75–0.91); calibration slope 0.67 (95% CI 0.40–1.04)] and lowest for the gene elusive group [c-statistic 0.65 (95% CI 0.57–0.74); calibration slope 0.26 (95% CI 0.06–0.49)]. Univariable analyses revealed variable performance of individual clinical risk markers in the different gene groups (see heatmap of hazard ratios and statistical significance in Figure). For example, RV dimensions and systolic function are significant risk markers in PKP2 but not in DSP patients and the opposite is true of LV systolic function (Figure). Conclusion The 2019 ARVC risk model performs reasonably well in gene positive ARVC, (particularly for PKP2) but is more limited in gene elusive patients. Genotype specific risk factors should be considered in ARVC patients. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): British Heart Foundation" @default.
• W4306319421 created "2022-10-16" @default.
• W4306319421 creator A5005775971 @default.
• W4306319421 creator A5006283314 @default.
• W4306319421 creator A5009913317 @default.
• W4306319421 creator A5012026969 @default.
• W4306319421 creator A5017716587 @default.
• W4306319421 creator A5022524949 @default.
• W4306319421 creator A5026271676 @default.
• W4306319421 creator A5031854646 @default.
• W4306319421 creator A5038518412 @default.
• W4306319421 creator A5057381638 @default.
• W4306319421 creator A5062816598 @default.
• W4306319421 creator A5076684995 @default.
• W4306319421 creator A5078231013 @default.
• W4306319421 creator A5085386690 @default.
• W4306319421 creator A5090422368 @default.
• W4306319421 date "2022-10-01" @default.
• W4306319421 modified "2023-09-27" @default.
• W4306319421 title "Risk stratification in Arrhythmogenic Right Ventricular Cardiomyopathy: the impact of genotype on the 2019 ARVC risk calculator" @default.
• W4306319421 doi "https://doi.org/10.1093/eurheartj/ehac544.1752" @default.
• W4306319421 hasPublicationYear "2022" @default.
• W4306319421 type Work @default.
• W4306319421 citedByCount "0" @default.
• W4306319421 crossrefType "journal-article" @default.
• W4306319421 hasAuthorship W4306319421A5005775971 @default.
• W4306319421 hasAuthorship W4306319421A5006283314 @default.
• W4306319421 hasAuthorship W4306319421A5009913317 @default.
• W4306319421 hasAuthorship W4306319421A5012026969 @default.
• W4306319421 hasAuthorship W4306319421A5017716587 @default.
• W4306319421 hasAuthorship W4306319421A5022524949 @default.
• W4306319421 hasAuthorship W4306319421A5026271676 @default.
• W4306319421 hasAuthorship W4306319421A5031854646 @default.
• W4306319421 hasAuthorship W4306319421A5038518412 @default.
• W4306319421 hasAuthorship W4306319421A5057381638 @default.
• W4306319421 hasAuthorship W4306319421A5062816598 @default.
• W4306319421 hasAuthorship W4306319421A5076684995 @default.
• W4306319421 hasAuthorship W4306319421A5078231013 @default.
• W4306319421 hasAuthorship W4306319421A5085386690 @default.
• W4306319421 hasAuthorship W4306319421A5090422368 @default.
• W4306319421 hasBestOaLocation W43063194211 @default.
• W4306319421 hasConcept C126322002 @default.
• W4306319421 hasConcept C164705383 @default.
• W4306319421 hasConcept C2775935837 @default.
• W4306319421 hasConcept C2776331378 @default.
• W4306319421 hasConcept C2777093960 @default.
• W4306319421 hasConcept C2777892804 @default.
• W4306319421 hasConcept C2778198053 @default.
• W4306319421 hasConcept C2778797674 @default.
• W4306319421 hasConcept C44249647 @default.
• W4306319421 hasConcept C71924100 @default.
• W4306319421 hasConcept C72563966 @default.
• W4306319421 hasConceptScore W4306319421C126322002 @default.
• W4306319421 hasConceptScore W4306319421C164705383 @default.
• W4306319421 hasConceptScore W4306319421C2775935837 @default.
• W4306319421 hasConceptScore W4306319421C2776331378 @default.
• W4306319421 hasConceptScore W4306319421C2777093960 @default.
• W4306319421 hasConceptScore W4306319421C2777892804 @default.
• W4306319421 hasConceptScore W4306319421C2778198053 @default.
• W4306319421 hasConceptScore W4306319421C2778797674 @default.
• W4306319421 hasConceptScore W4306319421C44249647 @default.
• W4306319421 hasConceptScore W4306319421C71924100 @default.
• W4306319421 hasConceptScore W4306319421C72563966 @default.
• W4306319421 hasIssue "Supplement_2" @default.
• W4306319421 hasLocation W43063194211 @default.
• W4306319421 hasOpenAccess W4306319421 @default.
• W4306319421 hasPrimaryLocation W43063194211 @default.
• W4306319421 hasRelatedWork W1489717041 @default.
• W4306319421 hasRelatedWork W1987431072 @default.
• W4306319421 hasRelatedWork W2000547619 @default.
• W4306319421 hasRelatedWork W2085745880 @default.
• W4306319421 hasRelatedWork W2264583845 @default.
• W4306319421 hasRelatedWork W236717508 @default.
• W4306319421 hasRelatedWork W2510429599 @default.
• W4306319421 hasRelatedWork W2808492546 @default.
• W4306319421 hasRelatedWork W2919365189 @default.
• W4306319421 hasRelatedWork W2978408006 @default.
• W4306319421 hasVolume "43" @default.
• W4306319421 isParatext "false" @default.
• W4306319421 isRetracted "false" @default.
• W4306319421 workType "article" @default.