Diagnostic lacunae and implications of an automated implantable cardioverter defibrillator implantation in a child with type 3 Long QT (LQT3) syndrome

A diagnosis of congenital long QT interval syndrome based on history and electrocardiogram was made in a child in the absence of readily available genetic testing. A genotype 3 (LQT3) was suspected after exclusion of other variants as the child was non-responsive to beta-blocker and sodium channel b...

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Bibliographic Details
Main Authors: Madan M Maddali, Pranav S Kandachar, Ismail A Al-Abri, Mohammed I Al-Yamani
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2022-01-01
Series:Annals of Cardiac Anaesthesia
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Online Access:http://www.annals.in/article.asp?issn=0971-9784;year=2022;volume=25;issue=2;spage=210;epage=213;aulast=Maddali
Description
Summary:A diagnosis of congenital long QT interval syndrome based on history and electrocardiogram was made in a child in the absence of readily available genetic testing. A genotype 3 (LQT3) was suspected after exclusion of other variants as the child was non-responsive to beta-blocker and sodium channel blocker medication. As the child continues to show episodic bradycardia, polymorphic ventricular ectopy, and T-wave alternans, a single-chamber automated implantable cardioverter-defibrillator implantation was done successfully. This report highlights how the diagnosis of LQT3 was arrived at as well as the anesthetic challenges in the management of patients with LQTS.
ISSN:0971-9784