Never Out of the Woods: Onset of Events in Long QT Syndrome Late in Life Provoked by Atrial Arrhythmias

• Main Authors: Justin Ng, MD, Chirag Barbhaiya, Tobias Reichlin, Koichi Nagashima, Roy John
• Format: Article
• Language: English
• Published: Elsevier 2014-09-01
• Series: Indian Pacing and Electrophysiology Journal
• Subjects: Long QT Syndrome; Atrial Arrhythmias
• Online Access: http://www.sciencedirect.com/science/article/pii/S0972629216307987
• ISSN: 0972-6292

The assessment of risk in the asymptomatic patient with long QT syndrome can often be a challenging task, particularly when the available evidence is limited to relatively small retrospective registries, not to mention the need to consider the effect of individual patient factors which are often difficult to quantitate. We describe the relatively uncommon case of a man with a long-standing diagnosis of Long QT 2 syndrome who suffered his first cardiac event in his late 60's, likely precipitated by the development of paroxysmal atrial tachycardia. A brief review of the available literature on risk assessment in adults with genetically confirmed long QT syndrome who have remained asymptomatic late into adulthood will follow the case.