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


1 Department of Cardiac Anesthesia, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman
2 Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman
3 Department of Pediatric Cardiology, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman

Correspondence Address:
Madan M Maddali
National Heart Center, Royal Hospital, P.B. No: 1331, P.C: 111, Seeb, Muscat
Sultanate of Oman
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.aca_13_21

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Year : 2022  |  Volume : 25  |  Issue : 2  |  Page : 210-213

 

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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.






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1 Department of Cardiac Anesthesia, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman
2 Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman
3 Department of Pediatric Cardiology, National Heart Center, Royal Hospital, Muscat, Sultanate of Oman

Correspondence Address:
Madan M Maddali
National Heart Center, Royal Hospital, P.B. No: 1331, P.C: 111, Seeb, Muscat
Sultanate of Oman
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.aca_13_21

Rights and Permissions

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.






[FULL TEXT] [PDF]*


        
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