Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-24T07:39:46.271Z Has data issue: false hasContentIssue false

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

Published online by Cambridge University Press:  30 September 2020

Mehmet Taşar*
Affiliation:
Pediatric Cardiovascular Surgery Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey
Nur Dikmen Yaman
Affiliation:
Pediatric Cardiovascular Surgery Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey
Huseyin Dursin
Affiliation:
Pediatric Cardiovascular Surgery Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey
Murat Şimşek
Affiliation:
Pediatric Cardiovascular Surgery Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey
Senem Özgür
Affiliation:
Pediatric Cardiology Department, Dr. Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital, Ankara, Turkey
*
Author for correspondence: Mehmet Taşar, Ankara Dr Sami Ulus Obstetrics and Gynaecology, Children’s Health and Diseases Training and Research Hospital, Cardiovascular Surgery, Cardiovascular Surgery, Ankara, Turkey. Tel: +90 505 442 47 80; Fax: +90 312 317 03 53. E-mail: [email protected]

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.

Type
Original Article
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

El-Sherif, N, Turitto, G, Boutjdir, M. Congenital Long QT syndrome and torsade de pointes. Ann Noninvasive Electrocardiol 2017; 22: e12481. CrossRefGoogle ScholarPubMed
Van Niekerk, C, Van Deventer, BS, du Toit-Prinsloo, L. Long QT syndrome and sudden unexpected infant death. J Clin Pathol 2017; 70: 808813. CrossRefGoogle ScholarPubMed
Waddell-Smith, KE, Earle, N, Skinner, JR. Must every child with long QT syndrome take a beta blocker? Arch Dis Child 2015; 100: 279282. CrossRefGoogle Scholar
Ildarova, RA, Shkolnikova, MA, Termosesov, SA. Implantation of cardioverter-defibrillator in children with Long-QT syndrome: assessment of indications, efficacy, and safety based on 10-year experience. Kardiologiia 2018; 58: 5258. CrossRefGoogle Scholar
Wilders, R, Verkerk, AO. Long QT syndrome and Sinus Bradycardia-A mini review. Front Cardiovasc Med 2018; 5: 106. CrossRefGoogle ScholarPubMed
Lupoglazoff, JM, Denjoy, I, Villain, E, et al. Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations. J Am Coll Cardiol 2004; 43: 826830. CrossRefGoogle ScholarPubMed
McCrossan, B, d’Udekem, Y, Davis, AM, et al. Successful implantation of a dual-chamber pacemaker in an ELBW infant for long QT syndrome. Cardiol Young 2015; 25: 600602. CrossRefGoogle Scholar
Früh, A, Siem, G, Holmström, H, et al. The jervell and Lange-Nielsen syndrome; atrial pacing combined with ß-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome? Heart Rhythm 2016; 13: 21862192. CrossRefGoogle ScholarPubMed
Marzuillo, P, Benettoni, A, Germani, C, et al. Acquired long QT syndrome: a focus for the general pediatrician. Pediatr Emerg Care 2014; 30: 257261. CrossRefGoogle ScholarPubMed
Baruteau, AE, Baruteau, J, Joomye, R, et al. Role of congenital long-QT syndrome in unexplained sudden infant death: proposal for an electrocardiographic screening in relatives. Eur J Pediatr 2009; 168: 771777. CrossRefGoogle ScholarPubMed
van den Berg, MP, Wilde, AA, Viersma, TJW, et al. Possible bradycardic mode of death and successful pacemaker treatment in a large family with features of long QT syndrome type 3 and Brugada syndrome. J Cardiovasc Electrophysiol 2001; 12: 630636. CrossRefGoogle Scholar