Longitudinal prediction of ventricular arrhythmic risk in ARVC

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease with a high risk of life-threatening ventricular arrhythmias (i.e. arrhythmias originating in the chambers of the heart) and sudden cardiac death. The disease is typically progressive, meaning that symptoms and signs may develop and possibly worsen over time. ARVC patients are often young, so they need to live with the prospect of a disease that may or may not cause health problems in the future. Accurate risk assessment to quantify the risk of life-threatening arrhythmias is therefore important.

Two years ago, researchers from around the world including members of the ERN GUARD-Heart network published a risk model to predict the risk of sudden cardiac death in patients with ARVC. This model was developed using 7 variables that can be easily obtained using routine cardiologic testing. The model is available at www.arvcrisk.com, and allows for a calculation of an absolute risk of arrhythmias (e.g. 5% per year or 10% in 5 years). This model, a “risk calculator”, facilitates the discussion between a patient and his/her physician regarding treatment options (e.g. medication), lifestyle interventions (e.g. limitation of sports activity), and preventive measures (e.g. implantation of an implantable cardioverter-defibrillator, a device that can stop an arrhythmia, e.g. by delivering a life-saving shock).

Up to the present time, it was however unclear how arrhythmia risk changes over time, and how the risk calculator should be used in patients who come to the cardiologist for repeated evaluations. For example, medications and lifestyle interventions may change a patient’s risk of arrhythmias, and this information was not accounted for in the risk calculator.

In the present manuscript, the same group of researchers showed that risk factors for life-threatening arrhythmias change over time, and that overall risk of arrhythmia decreases after an ARVC diagnosis. This was investigated using a cohort of 408 ARVC patients from the Netherlands and Johns Hopkins University (US). All patients underwent repeated evaluations, and changes in risk factors were analyzed over time. Specifically, the number of extra heartbeats (premature ventricular contractions) decreased by 1200/day on average over 5 years of follow-up, while pump function of the heart and electrical changes on an ECG remained unchanged. The observed risk of life-threatening arrhythmias decreased by 13% at 5 years follow-up. Moreover, the authors showed that updating the risk calculator with new results from cardiologic tests better predicts arrhythmias than the old test results in the risk calculator.

The information that was obtained in this manuscript will help the cardiologist to appropriately use the risk calculator during long-term follow-up. This is important, because ARVC patients will have to come for frequent cardiac evaluations. Therefore, the present manuscript will help physicians to better inform patients and their families regarding risk assessment and prevention of sudden death.


Link to original article

Translated by Anneline te Riele, UMC Utrecht, Utrecht, The Netherlands

Carrick RT, Te Riele ASJM, Gasperetti A, Bosman L, Muller SA, Pendleton C, Tichnell C, Murray B, Yap SC, van den Berg MP, Wilde A, Zeppenfeld K, Hays A, Zimmerman SL, Tandri H, Cadrin-Tourigny J, van Tintelen P, Calkins H, James CA, Wu KC. Longitudinal Prediction of Ventricular Arrhythmic Risk in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy. Circ Arrhythm Electrophysiol. 2022 Nov;15(11):e011207. doi: 10.1161/CIRCEP.122.011207. Epub 2022 Oct 31. PMID: 36315818; PMCID: PMC9669260.