A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome
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.
References
Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome
Citations
Electrophysiological Mechanisms of Brugada Syndrome: Insights from Pre-clinical and Clinical Studies
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