Arrhythmogenic Activity During Exercise in ARVC Patients

In this study, ARVC patients with different genotypes will perform a series of different physical activities, including treadmill walking, cycling at different intensities, biceps curls and loaded squats while undergoing extensive cardiovascular monitoring.

Inclusion criteria

• Definitive ARVC diagnosis according to the modified 2010 ARVC Task Force Criteria
• Pathogenic / likely pathogenic variant in one of the following desmosomal genes: plakophilin-2 (PKP-2), desmoglein-2 (DSG-2) or desmoplakin (DSP) or gene-elusive definite ARVC (i.e., genetic test performed but no pathogenic / likely pathogenic variant found)
• Control group: diagnosed ischemic heart disease with ejection fraction above 35%
• Agreement with responsible primary cardiologist that study participation is not considered beyond acceptable levels of physical activity for this particular patient
• No history of exercise-induced syncope in the 6 months prior to study participation
• No history of sudden cardiac arrest in the 6 months prior to study participation
• In patients who suffered from sudden cardiac arrest/arrhythmic syncope, only those having an implantable cardioverter-defibrillator (ICD) and being under beta-blockers/antiarrhythmic drugs are eligible for study inclusion
• Informed Consent as documented by signature (Appendix Informed Consent Form)
• Age: > 14 years
• Body-Mass-Index (BMI): 18.5 - 39.9 kg･m-2
• Willing to adhere to the following study rules:

• No intense exercise 48h prior to testing
• No exercise 24h prior to testing
• Sleep at least 7h the two nights before testing On test day
• No caffeinated food or drink before testing on test day
• No alcohol before testing on test day
• Fully compliant with normal medication regimen including beta-blockers and antiarrhythmic medication

Exclusion criteria

• No genetic test history for ARVC variants
• Heart failure with severely reduced left ventricular ejection fraction (LVEF <35%)
• For women: Pregnancy, breastfeeding, or intention to become pregnant during the study.
• Known or suspected non-compliance, drug, or alcohol abuse
• Inability to follow the procedures of the study, e.g., due to language problems, psychological disorders, dementia, etc. of the participant
• Previous enrolment into the current study