Left cardiac sympathetic denervation as the first-line therapy for congenital long QT syndrome

Abstract

Congenital long QT syndrome (LQTS) is a cardiac electrophysiological disorder due to genetic mutations. Patients with LQTS, if untreated, have a high incidence of ventricular tachycardia and cardiac arrest. Adrenergic activities are believed to play a major role in triggering the onset of cardiac events. The current mainstay of therapy for LQTS is oral β-blockers, which improves clinical symptoms and reduces the incidence of sudden cardiac death in approximately 70% of the patients. Left cardiac sympathetic denervation (LCSD) is an alternative therapy for patients who are resistant to β-blockers. Its clinical use, however, has been hindered by the complexity of the procedure and complications after the surgery. Video-assisted thoracoscopic sympathectomy has been used to treat patients with palmar and axillary hyperhidrosis. We suggest that the use of the microinvasice thoracoscopic technique may greatly simplify the LSCD procedure, making it the first-line therapy for LQTS.