Programmed Ventricular Stimulation as an Additional Primary Prevention Risk Stratification Tool in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Study

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited heart disease that may lead to sudden cardiac death. The disease is usually inherited, and so-called mutations (changes in the DNA) can be found in around 60% of affected patients. However, even when the DNA change is found in a patient or a family member, it is difficult to predict if he or she will develop life-threatening arrhythmias. A better understanding of this risk is important: to protect patients at high risk, and allow low-risk patients to be released from intensive cardiac screening.

One of the most feared complications of ARVC is sudden cardiac death, which may occur due to an arrhythmia. The risk of these life-threatening arrhythmias may be tested using an electrophysiology study (EPS), which is a procedure during which the heart’s electrical system is tested with catheters and wire electrodes in the heart. During the EPS, with the help of programmed ventricular stimulation or PVS (a controlled faster heart rate), it is possible to detect the cause of arrhythmia. This is a very valuable test, but it is invasive (which means it is a small surgical procedure in which wires are advanced towards the heart through the blood vessels in the leg) and also carries a risk of complications. It should therefore be used cautiously and mainly in patients in whom it is likely to change clinical treatment.

Several years ago, researchers from around the world including members of the ERN GUARD-Heart network published a “risk calculator” to predict the risk of sudden cardiac death in patients with ARVC. This calculator was based on 7 easily obtained markers during routine cardiac testing, without the use of an electrophysiology study. The model is available at, and allows for a calculation of an absolute risk of arrhythmias (e.g. 5% per year or 10% in 5 years).

In the present study, researchers from ERN GUARD-Heart tested the value of electrophysiology study on top of the ARVC risk calculator. The authors included 288 patients and followed them over an average of 5.3 years. During electrophysiology study, 61% of the patients showed a tendency to develop arrhythmias. This tendency was associated with an increased risk of sudden cardiac death during follow-up. Subsequently, the authors incorporated the electrophysiology study in the risk calculator. They showed that in the “low-to-intermediate risk” patients (i.e. those with an estimated 5-year risk of <25%), the electrophysiology study had a negative predictive value of 93% for life-threatening arrhythmias: this means that in 93% of patients with a normal electrophysiology study in which no arrhythmias could be provoked, no life-threatening ventricular arrhythmias occurred over time. As such, the cardiologist may reassure these patients that they are at a relatively low risk of arrhythmias.

Results from this study provide important insights to cardiologists who care for ARVC patients, and help them to appropriately use electrophysiology studies in these patients. The results of this study are incorporated on the website this website now provides updated risk percentages based on presence or absence of an abnormal electrophysiology study.


Link to original article

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

Gasperetti A, Carrick RT, Costa S, Compagnucci P, Bosman LP, Chivulescu M, Tichnell C, Murray B, Tandri H, Tadros R, Rivard L, van den Berg MP, Zeppenfeld K, Wilde AAM, Pompilio G, Carbucicchio C, Dello Russo A, Casella M, Svensson A, Brunckhorst CB, van Tintelen JP, Platonov PG, Haugaa KH, Duru F, Te Riele ASJM, Khairy P, Tondo C, Calkins H, James CA, Saguner AM, Cadrin-Tourigny J. Programmed Ventricular Stimulation as an Additional Primary Prevention Risk Stratification Tool in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Study. Circulation. 2022 Nov 8;146(19):1434-1443. doi: 10.1161/CIRCULATIONAHA.122.060866. Epub 2022 Oct 7. PMID: 36205131; PMCID: PMC9640278.