Premium
Clinical Management of Patients with the Long QT Syndrome: Drugs, Devices, and Gene‐Specific Therapy
Author(s) -
MOSS ARTHUR J.
Publication year - 1997
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.1997.tb03627.x
Subject(s) - medicine , long qt syndrome , cardiology , qt interval , sudden cardiac death , sudden death
The familial long QT syndrome (LQTS) is now recognized as a genetic cbannelopatby with a propensity to arrhythmogenic syncope and sudden death. Three genetic mutations have been identified that involve the slow and fast delayed potassium rectifier currents and the sodium current. Distinctive ECG T wave phenotypes are associated with each of the tbree genotypes. Current day therapy includes: β‐adrenergic blocking drugs; pacemakers; left cervicothoracic sympathetic ganglionectomy; implanted cardioverter defibrillators; and possibly, drugs that improve mutant ionic cbannel dysfunction. LQTS bas provided unique insight into the complex relationsbip between ionic cbannel dysfunction and ventricular tacbyarrbytbmias.