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Diagnosis and clinical management of long-QT syndrome

Steinberg, Christian

Current Opinion in Cardiology: January 2018 - Volume 33 - Issue 1 - p 31–41
doi: 10.1097/HCO.0000000000000465
ARRHYTHMIAS: Edited by David Birnie

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.

Quebec Heart and Lung Center, Laval University, 2725, chemin Sainte-Foy, Quebec, QC G1V 4G5, Canada

Correspondence to Dr Christian Steinberg, MD, FRCPC, Quebec Heart and Lung Center, Laval University, 2725, chemin Sainte-Foy, Quebec, QC G1V 4G5, Canada. Tel: +418 656 8711 #4598; fax: +418 656 4581; e-mail:

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