Distinct electrophysiological and mechanical beating phenotypes of long QT syndrome type 1-specific cardiomyocytes carrying different mutations


Both LQT1-CM-types showed prolonged repolarization, but only those with G589D presented early after-depolarizations at baseline. Increased amounts of abnormal Ca2 + transients were detected in both types of LQT1-CMs. Surprisingly, also the mechanical beating behavior demonstrated clear abnormalities and additionally the abnormalities were different with the two mutations: prolonged contraction was seen in G589D-CMs while impaired relaxation was observed in ivs7-2A>G-CMs. The CMs carrying two different LQT1 specific mutations (G589D or ivs7-2A>G) presented clear differences in their electrical properties as well as in their mechanical beating behavior. Results from different methods correlated well with each other suggesting that simply mechanical beating behavior of CMs could be used for screening of diseased CMs and possibly for diagnostic purposes in the future.

Authors Kiviaho AL, Ahola A, Larsson K, Penttinen K, Swan H, Pekkanen-Mattila M, Venäläinen H, Paavola K, Hyttinen J, Aalto-Setälä K
Journal International journal of cardiology. Heart & vasculature
Publication Date 2015 Sep 1;8:19-31
PubMed 28785673
PubMed Central PMC5497295
DOI 10.1016/j.ijcha.2015.04.008

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