ArticleJACC. Cardiovascular imaging2015
Cardiac Mechanical Alterations and Genotype Specific Differences in Subjects With Long QT Syndrome.
Article in JACC. Cardiovascular imaging, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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Who cites it
34 citing papers in PubMed.
- Structural and Echocardiographic Abnormalities in Congenital Long QT Syndrome: A Review of the Literature.Medicina (Kaunas, Lithuania) · 2026Review
- Electromechanical and structural phenotypes from cardiac imaging and epicardial mapping in inherited arrhythmia syndromes.Frontiers in cardiovascular medicine · 2026Review
- Clinical, Electrical, and Mechanical Parameters in Potassium Channel-Mediated Congenital Long QT Syndrome.Journal of clinical medicine · 2025Article
- Left ventricular mechanical dispersion as a predictor of the need for pacemaker implantation after transcatheter aortic valve implantation: MeDiPace TAVI study.European heart journal. Cardiovascular Imaging · 2024Article
- Predictive Value of Electromechanical Window for Risk of Fatal Ventricular Arrhythmia.Journal of Korean medical science · 2023Article
- Article
- Subtle QRS changes are associated with reduced ejection fraction, diastolic dysfunction, and heart failure development and therapy responsiveness: Applications for artificial intelligence to ECG.Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc · 2022Review
- Eosinophilic Infiltration of the Sino-Atrial Node in Sudden Cardiac Death Caused by Long QT Syndrome.International journal of molecular sciences · 2022Article
- Electromechanical reciprocity and arrhythmogenesis in long-QT syndrome and beyond.European heart journal · 2022Review
- Myocardial electrophysiological and mechanical changes caused by moderate hypothermia-A clinical study.Physiological reports · 2022Article
- Long QT syndrome and left ventricular non-compaction in a family with KCNH2 mutation: A case report.Frontiers in pediatrics · 2022Article
- Reversed Apico-Basal Myocardial Relaxation Sequence During Exercise in Long QT Syndrome Mutations Carriers With History of Previous Cardiac Events.Frontiers in physiology · 2021Article
- Exercise Induced Worsening of Mechanical Heterogeneity and Diastolic Impairment in Long QT Syndrome.Journal of clinical medicine · 2020Article
- Changes in left ventricular electromechanical relations during targeted hypothermia.Intensive care medicine experimental · 2020Article
- Isogenic Sets of hiPSC-CMs Harboring Distinct KCNH2 Mutations Differ Functionally and in Susceptibility to Drug-Induced Arrhythmias.Stem cell reports · 2020Article
- Electro-mechanics or mechano-electrics, an intricate interplay.European heart journal. Cardiovascular Imaging · 2020Article
- Article
- Left Ventricular Contraction Duration Is the Most Powerful Predictor of Cardiac Events in LQTS: A Systematic Review and Meta-Analysis.Journal of clinical medicine · 2020Article
- Murine model of left ventricular diastolic dysfunction and electro-mechanical uncoupling following high-fat diet.International journal of obesity (2005) · 2020Article
- Letter by Garcia A, et al. regarding article: Coronary artery disease risk reclassification by a new acoustic-based score.The international journal of cardiovascular imaging · 2020Article
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8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesThis study aimed to explore systolic and diastolic function and to investigate genotype-specific differences in subjects with long QT syndrome (LQTS).
backgroundLQTS is an arrhythmogenic cardiac ion channelopathy that traditionally has been considered a purely electrical disease. The most commonly affected ion channels are the slow potassium channel, IKs (KCNQ1 gene/LQT1), and the rapid potassium channel, IKr (KCNH2 gene/LQT2). Recent reports have indicated mechanical abnormalities in patients with LQTS.
methodsWe included 192 subjects with genotyped LQTS (139 LQT1, 53 LQT2). Healthy persons of similar age and sex as patients served as controls (n = 60). Using echocardiography, we assessed systolic function by left ventricular (LV) ejection fraction (EF), global longitudinal strain (GLS), and contraction duration (16 LV segments). Mechanical dispersion was calculated as standard deviation of contraction duration. Time difference between contraction duration and QT interval from electrocardiography (ECG) was defined as electromechanical time difference. We assessed diastolic function by transmitral filling velocities, early diastolic myocardial velocity (e'), and left atrial volume index (LAVI). Heart rate corrected QT interval (QTc) was assessed from 12-lead ECG.
resultsSystolic function by GLS was reduced in subjects with LQTS compared with healthy controls (-22.1 ± 2.1% vs. -23.0 ± 2.0%, p = 0.01), and GLS was worse in subjects with LQT2 compared with subjects with LQT1 (p = 0.01). Subjects with LQTS had longer contraction duration (426 ± 41 ms vs. 391 ± 36 ms, p < 0.001) and more dispersed contractions (33 ± 14 ms vs. 21 ± 7 ms, p < 0.001) compared with healthy controls. Diastolic function was also reduced in subjects with LQTS compared with healthy controls; e' was lower (10.7 ± 2.7 cm/s vs. 12.5 ± 2.0 cm/s, p < 0.001), and LAVI was increased (30 ± 8 ml/m(2) vs. 26 ± 5 ml/m(2), p = 0.01), also when adjusted for age and other possible confounders.
conclusionsSubjects with LQTS had a consistent reduction in both systolic and diastolic function compared with healthy controls. Differences in myocardial function between subjects with LQT1 and subjects with LQT2 may indicate that mechanical alterations in LQTS are genotype specific.
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