Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children

Heart Vessels. 2021 Jun;36(6):863-873. doi: 10.1007/s00380-020-01754-2. Epub 2021 Jan 29.


It can be difficult to distinguish children with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) from those with benign premature ventricular contraction (PVC). We retrospectively evaluated six school-aged children with ARVC and compared with those of 20 with benign PVC. The median age at initial presentation was 11.4 and 10.2 years in ARVC and benign PVC, respectively. None of the ARVC patients fulfilled the diagnostic criteria of ARVC at initial presentation. At ARVC diagnosis, the treadmill exercise test and Holter monitoring showed provoked PVC during exercise and pleomorphic PVC in all ARVC cases, respectively. During the observation period, terminal activation duration (TAD) was prolonged in all ARVC patients. In addition, ΔTAD (5.5 [3-10] ms) were significantly longer than those with benign PVC (p < 0.001). A new notched S-wave in V1 appeared in four (67%) ARVC patients, who had myocardial abnormalities in the right ventricle, and in zero benign PVC. Our electrocardiographic findings, such as provoked PVC during exercise, pleomorphic PVC, prolonged TAD, and a new notched S-wave in V1 could contribute to the early detection of ARVC in school-aged children.

Keywords: Arrthythmogenic right ventricular cardiomyopathy; Notched S-wave; Premature ventricular contraction; School-aged children.

MeSH terms

  • Adolescent
  • Arrhythmogenic Right Ventricular Dysplasia / diagnosis*
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology
  • Child
  • Child, Preschool
  • Electrocardiography / methods*
  • Female
  • Follow-Up Studies
  • Heart Conduction System / physiopathology*
  • Heart Ventricles / physiopathology*
  • Humans
  • Male
  • Retrospective Studies
  • Schools