A rare indication of permanent pacemaker implantation in children: congenital long QT syndrome

Tasar M., Yaman N., Dursin H., Simsek M., Ozgur S.

CARDIOLOGY IN THE YOUNG, vol.30, no.12, pp.1880-1881, 2020 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 30 Issue: 12
  • Publication Date: 2020
  • Doi Number: 10.1017/s1047951120003108
  • Journal Name: CARDIOLOGY IN THE YOUNG
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, Agricultural & Environmental Science Database, CINAHL, EMBASE, MEDLINE
  • Page Numbers: pp.1880-1881
  • Keywords: Long QT syndrome, sudden death, permanent pacemaker, SINUS BRADYCARDIA, INFANT, DEATH
  • Ankara University Affiliated: Yes

Abstract

Congenital Long QT Syndrome (LQTS) is a dangerous arrhythmic disorder that can be diagnosed in children with bradycardia. It is characterised by a prolonged QT interval and torsades de pointes that may cause sudden death. Long QT syndrome is an ion channelopathy with complex molecular and physiological infrastructure. Unlike the acquired type, congenital LQTS has a genetic inheritance and it may be diagnosed by syncope, stress in activity, cardiac dysfunction, sudden death or sometimes incidentally. Permanent pacemaker implantation is required for LQTS with resistant bradycardia even in children to resolve symptoms and avoid sudden death.