The Role of the Sympathetic Nervous System in the Long QT Syndrome: The Long Road from Pathophysiology to Therapy

This article reviews the role of the sympathetic nervous system in long QT syndrome (LQTS), in light of clinical and personal experience. The evolution of concepts related to LQTS is examined after 40 years of research and treatment. Studies stimulated by the sympathetic imbalance hypothesis have generated many unsuspected novel data and have provided an extremely strong rationale for the therapeutic use of left cardiac sympathetic denervation to prevent ventricular fibrillation, not only in LQTS but also in other life-threatening diseases associated with high risk for arrhythmic sudden death.