A case of pioneering subcutaneous implantable cardioverter defibrillator intervention in Timothy syndrome

BMC Pediatr. 2024 Nov 13;24(1):729. doi: 10.1186/s12887-024-05216-w.

Abstract

This case report presents a notable instance of subcutaneous implantable cardioverter defibrillator (S-ICD) implantation in a 9-year-old patient diagnosed with Timothy syndrome (TS), which is a rare condition characterized by mutations in the CACNA1c gene. Conventional therapies often have limited efficacy in managing TS. This case is significant, as it represents the youngest age for S-ICD implantation recorded in mainland China. While the absence of ventricular arrhythmias during hospitalization and follow-up is encouraging, it is not sufficient to conclusively establish the safety and feasibility of this intervention in young TS patients. Further research is needed to evaluate the long-term outcomes and to consider S-ICD as a potential standard treatment option for TS. Additionally, there is a need for a more detailed exploration of the molecular mechanisms underlying gene therapy and personalized interventions.

Keywords: Case report; Long QT; Subcutaneous implantable cardioverter defibrillator (S-ICD); Timothy syndrome; Ventricular arrhythmias.

Publication types

  • Case Reports

MeSH terms

  • Autistic Disorder* / therapy
  • Child
  • Defibrillators, Implantable*
  • Female
  • Humans
  • Long QT Syndrome* / genetics
  • Long QT Syndrome* / therapy
  • Male
  • Syndactyly* / therapy

Supplementary concepts

  • Timothy syndrome