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

Published online by Cambridge University Press:  30 September 2020

Mehmet Taşar Open the ORCID record for Mehmet Taşar [Opens in a new window] ,
Nur Dikmen Yaman ,
Huseyin Dursin ,
Murat Şimşek  and
Senem Özgür
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]
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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.

Keywords

Long QT syndromesudden deathpermanent pacemaker
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 12 , December 2020 , pp. 1880 - 1881
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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