Clinical Advances in Congenital Long QT Syndrome

Abstract

Abstract. Long QT syndrome is an inherited arrhythmia characterized by a prolonged QT interval and increased risk of life-threatening cardiac events, including arrhythmogenic syncope, seizures, and sudden cardiac death with a structurally normal heart. Since its first description in the 1950s, extensive researches allowed a better understanding of the cause and mechanisms of this disease, which improved our ability of early diagnosis, risk stratification, and precise therapy of these patients. This article provides an updated review of the clinical and molecular profiles of this potentially lethal inherited disorder and summarizes current knowledge regarding diagnosis, risk stratification, and therapy.