Archives of the Turkish Society of Cardiology
The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2020; 48(4): 434-438 | DOI: 10.5543/tkda.2020.40480

The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome

Adem Atici1, Ramazan Asoğlu2, Hasan Ali Barman3, Irfan Sahin4
1Department of Cardiology, İstanbul Medeniyet University Faculty of Medicine, İstanbul, Turkey
2Department of Cardiology, Adıyaman University Training and Research Hospital, Adıyaman, Turkey
3Department of Cardiology, Okmeydanı Training ünd Research Hospital, İstanbul, Turkey
4Department of Cardiology, Bağcılar Training and Research Hospital, İstanbul, Turkey

Congenital long QT syndrome (LQTS) is a genetic channelopathy associated with a high incidence of sudden cardiac death in children and young adults. QT interval prolongation is typically the primary finding on the electrocardiography (ECG) recordings, but a normal QT interval may be seen in as many as 40% of patients with LQTS due to incomplete penetrance. A normal QT interval on ECG in patients with LQTS is known as hidden LQTS. An epinephrine provocation test can help in the diagnosis of hidden LQTS. This case report describes the use of an epinephrine provocation test to diagnose hidden LQTS in 3 patients who had normal QT interval and corrected QT interval on ECG and a family history of sudden cardiac death.

Keywords: Hidden long QT syndrome, epinephrine provocation test; sudden death

How to cite this article
Adem Atici, Ramazan Asoğlu, Hasan Ali Barman, Irfan Sahin. The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome. Turk Kardiyol Dern Ars. 2020; 48(4): 434-438

Corresponding Author: Ramazan Asoğlu, Türkiye
© Copyright 2020 Archives of the Turkish Society of Cardiology
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