Çongenital long-QT syndrome (LQTS) is an inherited disorder that presents with syncope, polymorphic ventricular tachycardia, torsade de pointes and sudden death. The incidence rate of LQTS is 1 to 2 per 100000 and mainly involves children and young individuals. Because of familial and genetic underling and predisposing factors for life threatening arrhythmias in patients, diagnosis and treatment are an important aspects in the management of this syndrome. Ïn this study LQTS is described in a 49 years old woman with syncope, ventricular fibrillation and torsade de pointes who was referred to the Fatemeh Zahra (S) teaching hospital and treated successfully.

Keywords: Long-QT syndrome, sudden cardiac death, polymorphic ventricular tachycardia, syncope

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