Dataset Information

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator.

ABSTRACT:

Aims

Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus.

Methods and results

In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%.

Conclusion

Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC.

SUBMITTER: Jorda P

PROVIDER: S-EPMC9392650 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator.

Jordà Paloma P Bosman Laurens P LP Gasperetti Alessio A Mazzanti Andrea A Gourraud Jean Baptiste JB Davies Brianna B Frederiksen Tanja Charlotte TC Weidmann Zoraida Moreno ZM Di Marco Andrea A Roberts Jason D JD MacIntyre Ciorsti C Seifer Colette C Delinière Antoine A Alqarawi Wael W Kukavica Deni D Minois Damien D Trancuccio Alessandro A Arnaud Marine M Targetti Mattia M Martino Annamaria A Oliviero Giada G Pipilas Daniel C DC Carbucicchio Corrado C Compagnucci Paolo P Dello Russo Antonio A Olivotto Iacopo I Calò Leonardo L Lubitz Steven A SA Cutler Michael J MJ Chevalier Philippe P Arbelo Elena E Priori Silvia Giuliana SG Healey Jeffrey S JS Calkins Hugh H Casella Michela M Jensen Henrik Kjærulf HK Tondo Claudio C Tadros Rafik R James Cynthia A CA Krahn Andrew D AD Cadrin-Tourigny Julia J

European heart journal 20220801 32

<h4>Aims</h4>Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published ...[more]

PMID: 35766180

Similar Datasets

Project description:The novel arrhythmogenic right ventricular cardiomyopathy (ARVC)-associated ventricular arrhythmias (VAs) risk-prediction model endorsed by Cadrin-Tourigny et al. was recently developed to estimate visual VA risk and was identified to be more effective for predicting ventricular events than the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. Data regarding its application in Asians are lacking. We aimed to perform an external validation of this algorithm in the Chinese ARVC population. The study enrolled 88 ARVC patients who received implantable cardioverter-defibrillator (ICD) from January 2005 to January 2020. The primary endpoint was appropriate ICD therapies. The novel prediction model was used to calculate a priori predicted VA risk that was compared with the observed rates. During a median follow-up of 3.9 years, 57 (64.8%) patients received the ICD therapy. Patients with implanted ICDs for primary prevention had non-significantly lower rates of ICD therapy than secondary prevention (5-year event rate: 0.46 (0.13-0.66) and 0.80 (0.64-0.89); log-rank p = 0.098). The validation study revealed the C-statistic of 0.833 (95% confidence interval (CI) 0.615-1.000), and the predicted and the observed patterns were similar in primary prevention patients (mean predicted-observed risk: -0.07 (95% CI -0.21, 0.09)). However, in secondary prevention patients, the C-statistic was 0.640 (95% CI 0.510-0.770) and the predicted risk was significantly underestimated (mean predicted-observed risk: -0.32 (95% CI -0.39, -0.24)). The recalibration analysis showed that the performance of the prediction model in secondary prevention patients was improved, with the mean predicted-observed risk of -0.04 (95% CI -0.10, 0.03). The novel risk-prediction model had a good fitness to predict arrhythmic risk in Asian ARVC patients for primary prevention, and for secondary prevention patients after recalibration of the baseline risk.

Project description:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient's sudden cardiac death risk is sufficient to justify placement of an implantable cardioverter-defibrillator. The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people and that sudden death may be the first manifestation of the disease. This decision is particularly important because these are often young patients who are expected to live for many years. Although an implantable cardioverter-defibrillator can save lives in individuals with this disease, it is also well recognized that implantable cardioverter-defibrillator therapy is associated with both short- and long-term complications. Decisions about the placement of an implantable cardioverter-defibrillator are based on an estimate of a patient's risk of sudden cardiac death, as well as their preferences and values. The primary purpose of this article is to provide a review of the literature that concerns risk stratification in patients with ARVC and to place this literature in the framework of the 3 authors' considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include electric instability, including the frequency of premature ventricular contractions and sustained ventricular arrhythmia; proband status; extent of structural disease; cardiac syncope; male sex; the presence of multiple mutations or a mutation in TMEM43; and the patient's willingness to restrict exercise and to eliminate participation in competitive or endurance exercise.

Project description:BackgroundArrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD.MethodsWe assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (?30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping.ResultsA total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77-10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69-0.80) and calibration slope of 0.95 (95% CI, 0.94-0.98) indicating minimal over-optimism.ConclusionsLTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

Dataset Information

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator.

Aims

Methods and results

Conclusion

Publications

Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets