Heart, Lung and Circulation

Guidelines for the Diagnosis and Management of Familial Long QT Syndrome

      Long QT syndrome (LQTS) is a familial condition causing syncope and sudden death through rapid ventricular tachycardia (torsade de pointes), which can deteriorate to ventricular fibrillation, in otherwise fit and healthy young people. Prevalence is at least 1 in 5000 and may be up to 1 in 1000. Clinical diagnosis is made from a combination of suspicious history, family history and the 12 lead ECG, which typically reveals a heart-rate corrected QT interval (QT//R–R interval = QTc) of greater than 0.46 s in women and 0.45 s in men. Exercise testing is often helpful in making the diagnosis.
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      Further reading

        • Khan I.A.
        Clinical and therapeutic aspects of congenital and acquired long QT syndrome.
        Am J Med. 2002; 112 ([review article]): 58-66
        • Schwartz P.J.
        • Priori S.G.
        • Spazzolini C.
        • Moss A.J.
        • Vincent G.M.
        • Napolitano C.
        • et al.
        Genotype-phenotype correlation in the long QT syndrome: gene-specific triggers for life-threatening arrhythmias.
        Circulation. 2001; 103: 89-95
        • Moss A.J.
        • Zareba W.
        • Hall W.J.
        • Schwartz P.J.
        • Crampton R.S.
        • Benhorin J.
        • et al.
        Effectiveness and limitations of beta-blocker therapy in congenital long QT syndrome.
        Circulation. 2000; 101: 616-623
        • Priori S.G.
        • Schwartz P.J.
        • Napolitano C.
        • Bloise R.
        • Ronchetti E.
        • Grillo M.
        • et al.
        Risk stratification in the long-QT syndrome.
        N Engl J Med. 2003; 348: 1866-1874
        • Roden D.M.
        Drug-induced prolongation of the QT interval.
        N Engl J Med. 2004; 350: 1013-1022