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Journals / Türk Kardiyoloji Derneği Arşivi / 2020 / Cilt: 48 - Sayı: 4

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

Gizli tip 1 konjenital uzun QT sendromunda epinefrin provokasyon testinin önemi

Adem ATICI
Hasan Ali BARMAN
Ramazan ASOĞLU
İrfan ŞAHİN
Pages
434–438
Publication Date
2022-11-06
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

This case report describes the use of anepinephrine provocation test to diagnose hidden LQTS in 3patients who had normal QT interval and corrected QT interval on ECG and a family history of sudden cardiac death.

📌 3 Key Takeaways

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Objective & Hypothesis:Congenital long QT syndrome (LQTS) is agenetic channelopathy associated with a high incidenceof sudden cardiac death in children and young adults.
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Key Findings & Data:QTinterval prolongation is typically the primary finding on theelectrocardiography (ECG) recordings, but a normal QTinterval may be seen in as many as 40% of patients withLQTS due to incomplete penetrance.
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Conclusion & Impact:This case report describes the use of anepinephrine provocation test to diagnose hidden LQTS in 3patients who had normal QT interval and corrected QT interval on ECG and a family history of sudden cardiac death.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

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

Özet

Konjenital uzun QT sendromu (UQTS) çocuklarda ve genç erişkinlerde ani kardiyak ölüm insidansı ile ilişkili genetik bir kanalopatidir. QT aralığı uzaması elektrokardiyografide (EKG) ana bulgudur, ancak eksik penetrasyon nedeniyle UQTS hastaların yaklaşık %40’ında normal QT aralığı görülür. UQTS’li hastalarda EKG’de normal QT aralığı gizli UQTS olarak bilinmektedir. Epinefrin provokasyon testi gizli UQTS tanısında yardımcı olabilir. Bu olgu serisinde EKG’de normal QT ve düzeltilmiş QT aralıkları olan ve ailesinde ani kalp ölümü öyküsü olan üç bireyde gizli UQTS tanısı için epinefrin provokasyon testi kullanıldı.

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Cite This Article

ATICI, A., BARMAN, H. A., ASOĞLU, R., ŞAHİN, İ. (2022). The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome. Türk Kardiyoloji Derneği Arşivi, Cilt: 48 - Sayı: 4, 434–438. https://ajindex.com/articles/631942

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