Immunosuppressive therapy in childhood-onset arrhythmogenic inflammatory cardiomyopathy

Pacing Clin Electrophysiol. 2021 Mar;44(3):552-556. doi: 10.1111/pace.14153. Epub 2021 Jan 18.

Abstract

We present, to our knowledge, the first case of immunosuppressive therapy (IST) application in a 12-year-old child with arrhythmogenic inflammatory cardiomyopathy resulting from the overlap between autoimmune myocarditis and primary arrhythmogenic cardiomyopathy. Indication to off-lable IST was compelling, because of recurrent drug-refractory ventricular arrhythmias (VAs). We show that IST was feasible, safe, and effective on multiple clinical endpoints, including symptoms, VA recurrences, and T-troponin release. Remarkably, all diagnostic and therapeutic strategies were worked out by a dedicated multidisciplinary team, including specialized pediatric immunologists.

Keywords: ARVC; childhood; immunosuppressive therapy; multidisciplinary; myocarditis; pediatric; ventricular arrhythmias.

Publication types

  • Case Reports

MeSH terms

  • Arrhythmogenic Right Ventricular Dysplasia / drug therapy*
  • Arrhythmogenic Right Ventricular Dysplasia / immunology*
  • Azathioprine / therapeutic use
  • Biomarkers / blood
  • Child
  • Echocardiography
  • Electrocardiography
  • Humans
  • Immunosuppression Therapy*
  • Magnetic Resonance Imaging
  • Male
  • Myocarditis / drug therapy
  • Myocarditis / immunology
  • Prednisone / therapeutic use
  • Recurrence
  • Risk Factors

Substances

  • Biomarkers
  • Azathioprine
  • Prednisone