Diagnosis and clinical management of long-QT syndrome

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Abstract

Purpose of review

To give an overview over the substantial advances in the diagnosis and management of Long-QT syndrome since its first description 60 years ago.

Recent findings

LQT syndrome remains the most common inherited arrhythmia and is a leading cause for sudden unexplained death accounting for up to 20–25% of cases. Rapid progress of genetic technology over the past 2 decades has significantly improved our understanding of molecular and genetic mechanisms of LQT. Despite all those novel insights, phenotype assessment and appropriate risk stratification in LQT remains challenging – even for the expert.

Summary

This review outlines our current understanding and approach to the clinical diagnosis and management of LQT as well as recent insights into genotype–phenotype correlations. Genetic testing has evolved beyond a pure diagnostic tool and is in addition increasingly integrated as complementary prognostic marker. With regard to the management of LQT, there is now evidence that the protective effect of beta-blockers is rather substance-specific than a class effect. Novel approaches – in conjunction with standard beta-blockers – are emerging including gene-specific treatment for certain subtypes of LQT. A specialized inherited arrhythmia clinic is the preferred resource for the complex risk stratification and individualized management of individuals with LQT.

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