The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
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| Enthalten in: |
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir - 48(2020), 4 vom: 29. Juni, Seite 434-438 |
| Sprache: |
Englisch |
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| Weiterer Titel: |
Gizli tip 1 konjenital uzun QT sendromunda epinefrin provokasyon testinin önemi |
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| Beteiligte Personen: |
Atici, Adem [VerfasserIn] |
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| Links: |
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| Themen: |
9007-49-2 |
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| Anmerkungen: |
Date Completed 07.12.2021 Date Revised 14.12.2021 published: Print Citation Status MEDLINE |
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| doi: |
10.5543/tkda.2020.40480 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM310993202 |
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| 245 | 1 | 4 | |a The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome |
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| 520 | |a 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 | ||
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