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- W2332935631 abstract "Long QT syndrome (LQTS) is inherited disorder with high risk of sudden death (SD). It is characterized by increase in duration of action potential and increase of transmural heterogeneity in myocardium. Study aims at investigating relationship between contraction and relaxation of the myocardium and ECG characteristics of systole in young LQTS pts. Methods: Simultaneously recorded ECG and echocardiography were analyzed in 93 LQTS pts aged 2 to 20 years and in 40 controls matched by age and heart rate (HR). Electromechanical dissociation (EMD) was measured as interval from the aortic valve (AOv) closure (or the end of systolic wall motion) to the end of QT interval. Isovolumic relaxation time (IVRT) and IVRT dispersion were assessed by spectral (SpD) and tissue Doppler (TD) in 12 segments; longitudinal strain was assessed oin 16 segments by the speckle tracking. SD risk factors, and ECG characteristics such as HR, QT, QTc and QT dispersion (QTd) were analyzed. Results: In comparison with the control subjects LQTS pts characterized by increased global strain (p=0.01). In 52 LQTS pts (56%) abnormal longitudinal LV strain (picture) as a plateau or a double-peaked motion after maximum myocardial shortening in ≥ 5 segments was registered. This pts have more severe cource of LQTS (syncope or QTc>500ms), significantly lower HR, prolonged QT and QTc (p=0.05), and by higher QTd (p=0.05). They have higher EMD by SpD (p=0.002) and by TD (p=0.001), prolonged IVRT SpD and IVRT TD (p=0.000), higher IVRT dispersion (p= 0.011). Abnormal strain was registered after closure of the AOv and coincided with IVRT. Abnormal longitudinal strain LV pattern Conclusion: Young pts with more severe manifestations of the LQTS syndrome demonstrate abnormal contraction during prolonged IVRT, due to significant disparity between electrical and mechanical systole." @default.
- W2332935631 created "2016-06-24" @default.
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- W2332935631 date "2013-08-02" @default.
- W2332935631 modified "2023-09-27" @default.
- W2332935631 title "Abnormal mechanical contraction and relaxation in young patients with congenital Long QT syndrome" @default.
- W2332935631 doi "https://doi.org/10.1093/eurheartj/eht310.p5587" @default.
- W2332935631 hasPublicationYear "2013" @default.
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