Short QT syndrome: pharmacological treatment

作者: Fiorenzo Gaita , Carla Giustetto , Francesca Bianchi , Rainer Schimpf , Michel Haissaguerre

DOI: 10.1016/J.JACC.2004.02.034

关键词: Ventricular fibrillationShort QT syndromeSudden deathQuinidineSotalolAnesthesiaFlecainideQT intervalInternal medicineIbutilideMedicineCardiology

摘要: Abstract Objectives The purpose of this study was to evaluate the efficacy various antiarrhythmic drugs at prolonging QT interval into normal range and preventing ventricular arrhythmias in patients with short syndrome. Background Short syndrome is a recently described genetic disease characterized by interval, high risk sudden death, atrial fibrillation, refractory periods. Methods Six syndrome, five whom had received an implantable cardioverter-defibrillator (ICD) one child, were tested different drugs, including flecainide, sotalol, ibutilide, hydroquinidine, determine whether they could prolong thus prevent symptoms arrhythmia recurrences. Results Class IC III did not produce significant prolongation. Only hydroquinidine administration caused prolongation, which increased from 263 ± 12 ms 362 25 (calculated 290 13 405 26 ms). Ventricular programmed stimulation showed prolongation effective period ≥200 ms, fibrillation no longer induced. Conclusions ability quinidine has potential be therapy for patients. This particularly important because these are death birth, ICD implant feasible very young children.

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