The Importance Of The Epinephrine Provocation Test In Hidden Type 1 Congenital Long Qt Syndrome. [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. Ahead of Print: TKDA-40480 | DOI: 10.5543/tkda.2020.40480

The Importance Of The Epinephrine Provocation Test In Hidden Type 1 Congenital Long Qt Syndrome.

Adem Atici1, Ramazan Asoğlu2, hasan ali barman3, Irfan sahin4
1Istanbul Medeniyet University, Department of Cardiology, Istanbul, Turkey.
2Adıyaman University Training And Research Hospital, Cardiology Department, Adiyaman, Turkey.
3Okmeydani Training And Research Hospital, Department Of Cardiology, Istanbul, Turkey
4Bağcılar Training And Research Hospital, Cardiology Department, Istanbul, Turkey.

Congenital long QT syndrome (LQTS) is a genetic channelopathy associated with a high incidence of sudden cardiac death in children and young adults. The QT interval prolongation is the main finding on electrocardiography (ECG) but normal QT interval is seen in approximately 40% of patients with LQTS due to incomplete penetration. Hidden LQTS has been known as a normal QT interval on ECG in patients with LQTS. Epinephrine provocation test may help in the diagnosis of hidden LQTS. In this case series report, epinephrine provocation test was used to diagnose the hidden LQTS in three subjects who had a normal QT and corrected QT intervals on ECG and had a family history of sudden cardiac death.

Keywords: Hidden long QT syndrome, Epinephrine provocation test, Sudden death,

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Corresponding Author: Ramazan Asoğlu, Türkiye
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