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Arrhythm Electrophysiol Rev. 2019 Mar;8(2):105-110. doi: 10.15420/aer.2019.1.1.

Long QT Syndrome Modelling with Cardiomyocytes Derived from Human-induced Pluripotent Stem Cells.

Author information

1
Istituto Auxologico Italiano, IRCCS, Laboratory of Cardiovascular Genetics Milan, Italy.
2
Coronary Care Unit and Laboratory of Experimental Cardiology for Cell and Molecular Therapy, IRCCS Policlinico San Matteo Foundation Pavia, Italy.
3
Department of Medicine, University of Cape Town Cape Town, South Africa.
4
Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin Milan, Italy.
5
Cardiovascular Genetics Laboratory, Hatter Institute for Cardiovascular Research in Africa, Department of Medicine, University of Cape Town Cape Town, South Africa.

Abstract

Long QT syndrome (LQTS) is a potentially severe arrhythmogenic disorder, associated with a prolonged QT interval and sudden death, caused by mutations in key genes regulating cardiac electrophysiology. Current strategies to study LQTS in vitro include heterologous systems or animal models. Despite their value, the overwhelming power of genetic tools has exposed the many limitations of these technologies. In 2010, human-induced pluripotent stem cells (hiPSCs) revolutionised the field and allowed scientists to study in vitro some of the disease traits of LQTS on hiPSC-derived cardiomyocytes (hiPSC-CMs) from LQTS patients. In this concise review we present how the hiPSC technology has been used to model three main forms of LQTS and the severe form of LQTS associated with mutations in calmodulin. We also introduce some of the most recent challenges that must be tackled in the upcoming years to successfully shift hiPSC-CMs from powerful in vitro disease modelling tools into assets to improve risk stratification and clinical decision-making.

KEYWORDS:

Long QT syndrome; cardiac arrhythmias; cardiomyocytes; human-induced pluripotent stem cells; life-threatening arrhythmias; precision medicine; stem cells; sudden cardiac death

Conflict of interest statement

Disclosure: The authors have no conflicts of interest to declare.

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