This website hosted by the Netherlands Heart Institute is for information and training purpose only, The Netherlands Heart Institute nor the individual researchers involved in this project carry responsibility for medical decisions made with the information provided by this website.

The ARVC risk calculator is based on clinical data of patients fulfilling ARVC diagnosis as per modified Task Force Criteria (TFC) [1] from 14 academic centers worldwide. The calculator utilizes three prediction algorithms. One algorithm predicts the risk of fast VT (>250bpm), VF, or SCA/SCD [2]. The second predicts the risk of any type of sustained VA (including VT <250bpm) as first event in patients without prior events (primary prevention only). The third algorithm is an adjustment of the second, adding PVS results to increase accuracy [4]. It should be stressed that non of the predicted outcomes estimates the risk of SCD in specific. As the main purpose of ICD implantation is SCD prevention (not VT termination), this is an important limitation when using this calculator for ICD consideration.

The predictions this calculator provides are based on clinical characteristics of patients at time of their diagnosis (within a time frame of ca. 1 year), therefore its application is meant for newly diagnosed patients (according to 2010 TFC). Pending further research, interpretation of predictions by entering characteristics during follow-up is discouraged.

Caution should be exercised when interpreting the prediction for patients <14 years of age, as the cohort this prediction model was based on only contained 2% pediatric patients <14 years.

Our prediction model is currently not part of any clinical guideline and should not be used to replace the standard of care. Our calculator uses the values of the clinical characteristics entered only to estimate the risk observed in patients with similar characteristics in our cohort. Accuracy of the predictions was only internally validated in our cohort. Therefore, we cannot provide any guarantee regarding the validity of predictions for patients outside our own cohort. We do not provide any recommendations for ICD implantation, and the use and interpretation of results from our calculator are at the responsibility of the user.

Before this calculator can be safely recommended for clinical use, future external validation studies should confirm the predictions to be accurate in patients outside of our cohort. We aim to keep track and list all validation studies here for a complete and up-to-date overview of the evidence. If you notice a study is missing, we would highly appreciate if you contact us.

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[1] Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Protonotarios N, Saffitz JE, Sanborn DM, Steinberg JS, Tandri H, Thiene G, Towbin JA, Tsatsopoulou A, Wichter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J 2010;31: .806–814. DOI: 10.1093/eurheartj/ehq025

[2] Cadrin-Tourigny J, Bosman LP, Wang W, Tadros R, Bhonsale A, Bourfiss M, et al. Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): A Multinational Collaboration. Circ. AE 2020. DOI: 10.1161/CIRCEP.120.008509

[3] Cadrin-Tourigny J, Bosman LP, Nozza A, Wang W, Tadros R, Bhonsale A, et al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. European Heart J 2019. DOI: 10.1093/eurheartj/ehz103

[4] Gasperetti A, Carrick RT, Costa S, Compagnucci P, Bosman LP, Chivulescu M, et al. Programmed Ventricular Stimulation as an Additional Primary Prevention Risk Stratification Tool in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Study. Circulation 2022. DOI: 10.1161/CIRCULATIONAHA.122.060866