Comorbid Epilepsy and Developmental Disorders in Congenital Long QT Syndrome With Life-Threatening Perinatal Arrhythmias

JACC. Clinical Electrophysiology
Aya MiyazakiHideo Ohuchi

Abstract

Given the association of long QT syndrome (LQTS) and neurological disorders, we speculated that the more severe LQTS phenotype, perinatal LQTS, would exhibit more frequent comorbid neurodevelopmental anomalies than LQTS without perinatal arrhythmias (nonperinatal LQTS). Congenital LQTS with life-threatening perinatal arrhythmias (perinatal LQTS) has a poor life prognosis. Twenty-one consecutive LQTS patients diagnosed before 1 year of age at our institution and 3 previously reported perinatal LQTS patients with neurological seizures were enrolled. In total, the clinical course was evaluated in 24 patients. Among 21 infantile LQTS patients, 5 of 6 with perinatal LQTS (83%) were diagnosed with epilepsy and 4 (67%) with developmental disorders, but none with nonperinatal LQTS were. The total development quotient by Kinder Infant Development Scale scores was 17 to 72 (median 67) in 5 epileptic perinatal LQTS. In the 8 perinatal LQTS patients with neurological disorders, including 3 previously reported cases, epileptic seizures occurred at 2 days to 2.5 years of age and 5 had developmental disorders. Mutations in these 8 patients were located in the transmembrane loop of KCNH2, and D3/S4-S5 linker, D4/S4, or the D4/S6 segment of SCN...Continue Reading

References

Nov 1, 1995·American Heart Journal·D L TrippelP C Gillette
Dec 3, 1998·Journal of Cardiovascular Electrophysiology·A P GorgelsZ Al Halees
Jul 19, 2002·Cardiovascular Research·Carmen R ValdiviaJonathan C Makielski
Mar 31, 2007·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Sarah E HeronJohn C Mulley
Sep 11, 2007·Seminars in Fetal & Neonatal Medicine·Janet M RennieNicola J Robertson
Mar 20, 2009·Lancet Neurology·Douglas E Crompton, Samuel F Berkovic
Aug 22, 2009·Journal of the American College of Cardiology·Carla SpazzoliniG Michael Vincent
Sep 5, 2009·Ultrasound in Obstetrics & Gynecology : the Official Journal of the International Society of Ultrasound in Obstetrics and Gynecology·J M SimpsonH Gill
Oct 8, 2009·Circulation. Arrhythmia and Electrophysiology·Dao W WangAlfred L George
Oct 30, 2009·Epilepsia·Sarah E HeronJohn C Mulley
Nov 21, 2009·Journal of the American College of Cardiology·Wataru ShimizuScott McNitt
Mar 4, 2010·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Peter F AzizMaully J Shah
Jun 30, 2010·Neuroradiology·Leonardo Guilhermino GutierrezLeandro Tavares Lucato
May 17, 2012·Circulation. Cardiovascular Genetics·Hiromi KimuraMinoru Horie
Sep 20, 2012·Physiological Reviews·Jamie I VandenbergAdam P Hill
May 21, 2013·Journal of the American College of Cardiology·Peter J SchwartzArthur A M Wilde
Sep 3, 2013·Circulation. Arrhythmia and Electrophysiology·Bettina F CuneoD Woodrow Benson
Sep 17, 2013·Circulation. Arrhythmia and Electrophysiology·Masao YoshinagaMasami Nagashima
Oct 2, 2013·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Kristina H HaugaaErik Taubøll
Oct 10, 2013·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Jason H AndersonMichael J Ackerman
Jun 29, 2014·Heart Rhythm : the Official Journal of the Heart Rhythm Society·James R PriestEuan A Ashley

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Citations

Feb 1, 2017·Circulation·James A Reiffel

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