Exercise testing for long-term follow-up in arrhythmogenic right ventricular cardiomyopathy

• Published in: Journal of electrocardiology 2017-03, Vol.50 (2), p.176-183
• Main Authors: Karlsson, Daniel, Engvall, Jan, Ando, Agota Alfoldine, Aneq, Meriam Åström
• Format: Article
• Language: English
• Subjects: Adult; Arrhythmia; Arrhythmogenic Right Ventricular Dysplasia - diagnosis; Arrhythmogenic Right Ventricular Dysplasia - physiopathology; ARVC; Cardiac arrhythmia; Cardiomyopathy; Cardiovascular; Electrocardiography; Electrocardiography - methods; Exercise Test - methods; Exercise testing; Exercise Tolerance; Female; Follow up; Follow-Up Studies; Humans; Longitudinal Studies; Male; Mutation; Prognosis; Reproducibility of Results; Sensitivity and Specificity
• ISSN: 0022-0736; 1532-8430; 1532-8430
• DOI: 10.1016/j.jelectrocard.2016.10.011

Abstract Objectives We investigated arrhythmia, electrocardiography and physical work capacity (PWC) in the follow-up of ARVC. Design Twenty-three patients (13 men; age 41 ± 12 yrs) fulfilling diagnostic criteria were re-investigated after at least five years. Results Ventricular arrhythmia during exercise testing (ET) was present in 14 patients (61%) and showed variation between examinations. In eleven (48%), complex ventricular ectopic activity was observed at peak exercise or immediately thereafter. Mutations known to be pathogenic in ARVC were present in 13 patients (57%) of which 11 developed complex ventricular arrhythmia at ET. PWC at baseline was 190 ± 66 W (104 ± 26%) decreasing to 151 ± 61 W (91 ± 23%, p = 0.008) after 10.7 yrs. Conclusion The appearance of ventricular arrhythmia during exercise testing showed temporal variation but was frequent in patients with relevant genetic mutation. Physical exercise capacity decreased over time in patients with ARVC in excess to the age-related deterioration and regardless of medication.