title: The congenital long QT syndrome.
abstract: OBJECTIVE
  The long QT syndrome (LQTS) is a disorder of the electrical system of the heart, due to dysfunction of the ion channels and involving the repolarisation process. The inherited form occurs when there is a mutation in one of the genes which encode the making of a channel. Prolongation of the QT interval renders the patient vulnerable to an arrythmia called torsade de pointes, resulting in syncope and sudden death.
  
  
  METHODS
  Three children with the congenital long QT syndrome presented to the pediatric department, one of them also having a 2:1 atrio-ventricular block. The parents and siblings of these children were screened for the long QT syndrome with an electrocardiogram. 2D echocardiography was done to rule out structural abnormalities and audiometry for deafness.
  
  
  RESULTS
  Four family members were identified on screening to have LQTS. Propranolol was started on all children with LQTS. The child with heart block also received a pacemaker. LQTS must be considered in all patients presenting with syncope especially if associated with deafness and/or a family history of sudden deaths in infancy or childhood.
  
  
  CONCLUSIONS
  The corrected QT interval must be determined in all children with heart block since the two conditions are often associated.
authors.affiliation: Pediatric Intensive Care Unit (PICU), Department of Pediatrics, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai, India. pshanbag@yahoo.com
authors.forename: Preeti
authors.initials: P
authors.lastname: Shanbag
authors.forename: Preetha T
authors.initials: PT
authors.lastname: Govindakumar
authors.forename: Mamta
authors.initials: M
authors.lastname: Vaidya
authors.forename: Varsha
authors.initials: V
authors.lastname: Joshi
authors.forename: Sukhbir Kaur
authors.initials: SK
authors.lastname: Shahid
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000151:congenital* / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D010138:Pacemaker, Artificial; D010375:Pedigree; D011379:Prognosis; D011433:Propranolol / Q000008:administration & dosage; D018570:Risk Assessment; D016896:Treatment Outcome
chemical_list: D011433:Propranolol