The congenital long QT syndrome

Indian Journal of Pediatrics
Preeti ShanbagSukhbir Kaur Shahid

Abstract

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. 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. 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. The corrected QT interval must be determined in all children with heart block since the two conditions are often associated.

References

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Citations

Jan 11, 2014·Indian Journal of Pediatrics·Bhavesh ThakkarTarun Madan
Aug 10, 2011·Paediatric Anaesthesia·Banashree MandalSachin Mahajan
Oct 18, 2005·Journal of Electrocardiology·Ramarao S LankipalliGan-Xin Yan
Feb 6, 2010·The American Journal on Addictions·Edward W BoyerSteven Marcus
Jul 4, 2006·Indian Journal of Pediatrics·M P Jayakrishnan, P Krishnakumar
May 26, 2005·The Journal of Experimental Biology·Renate KoppBernd Pelster

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