A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome

Journal of the American College of Cardiology
Ofer Havakuk, Sami Viskin

Abstract

The Brugada syndrome (BrS) and long-QT syndrome (LQTS) present as congenital or acquired disorders with diagnostic electrocardiograms (ST-segment elevation and prolonged QT interval, respectively) and increased risk for malignant arrhythmias. Our understanding of the 2 disease forms (congenital vs. acquired) differs. A female patient on quinidine for atrial fibrillation who develops ventricular fibrillation is diagnosed with "acquired LQTS" and is discharged with no therapy other than instructions to avoid QT-prolonging medications. In contrast, an asymptomatic male patient who develops a Brugada electrocardiogram on flecainide is diagnosed with "asymptomatic BrS" and could be referred for an electrophysiological evaluation that could result in defibrillator implantation. The typical patient undergoing defibrillator implantation for BrS is asymptomatic but has a Brugada electrocardiogram provoked by a drug. The authors describe how the histories of LQTS and BrS went through the same stages, but in different sequences, leading to different conclusions.

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Citations

Jun 1, 2016·Frontiers in Pharmacology·Paola ImbriciDiana Conte
Nov 5, 2016·Nature Reviews. Cardiology·Juan SieiraPedro Brugada
Feb 25, 2017·Journal of the American College of Cardiology·Valentin Fuster
Dec 16, 2016·Physiological Reviews·Christopher L-H Huang
May 12, 2018·Journal of the American Heart Association·Sami ViskinRaphael Rosso
Oct 10, 2019·Journal of Cardiovascular Electrophysiology·Víctor NeiraAdrian Baranchuk
Aug 11, 2018·BMJ Case Reports·Russell StanleyAnjana Nair
Feb 25, 2017·The Journal of General Physiology·Eric J HsuJonathan R Silva
Jan 1, 2017·Archivos de cardiología de México·Bortolo MartiniAndrea Nava
Aug 28, 2018·Frontiers in Physiology·Michelle M MonaskyLuigi Anastasia
Jan 5, 2017·Proceedings of the National Academy of Sciences of the United States of America·Ricardo CaballeroJuan Tamargo
Feb 20, 2021·JACC. Clinical Electrophysiology·Sami ViskinRaphael Rosso
Apr 4, 2021·Journal of Clinical Medicine·Nicolò MartiniBortolo Martini
May 16, 2018·JACC. Clinical Electrophysiology·Sami Viskin, Raphael Rosso
Oct 16, 2021·Heart·E Madelief J MarsmanCarol Ann Remme

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