A Review of Long QT Syndrome: Everything a Hospitalist Should Know

Hosp Pediatr. 2020 Apr;10(4):369-375. doi: 10.1542/hpeds.2019-0139. Epub 2020 Mar 6.


In this article, we will review various aspects of long QT syndrome (LQTS) necessary for hospitalists who care for children, adolescents, and young adults who have known LQTS and also review presenting features that should make one consider LQTS as a cause of hospitalization. Pediatric hospitalists care for patients who have suffered near-drowning, unexplained motor vehicular accidents, brief resolved unexpected events, sudden infant death syndrome, recurrent miscarriages, syncope, or seizures. These common conditions can be clinical clues in patients harboring 1 of 16 LQTS genetic mutations. LQTS is commonly caused by a channelopathy that can cause sudden cardiac death. Over the years, guidelines on management and recommendations for sports participation have evolved with our understanding of the disease and the burden of arrhythmias manifested in the pediatric age group. This review will include the genetic causes of LQTS, clinical features, and important historical information to obtain when these presentations are encountered. We will review medical and surgical treatments available to patients with LQTS and long-term care recommendations and prognosis for those diagnosed with LQTS.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Child
  • Death, Sudden, Cardiac
  • Electrocardiography
  • Hospitalists*
  • Humans
  • Infant
  • Long QT Syndrome* / etiology
  • Long QT Syndrome* / genetics
  • Prognosis
  • Young Adult