Phase-contrast magnet resonance imaging reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in patients with long QT syndrome

Brado, Johannes; Dechant, Markus Johann; Menza, Marius; Komancsek, Adriana; Lang, Corinna N.; Bugger, Heiko; Foell, Daniela; Jung, Bernd; Stiller, Brigitte; Bode, Christoph; Odening, Katja E.

doi:10.1016/j.hrthm.2017.04.045

Cited by 25 publications

(26 citation statements)

References 23 publications

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“…High spatiotemporal resolution techniques are needed to detect such distinct alterations. With this study we showed a prolonged myocardial contraction duration in paediatric LQTS patients compared to age- and gender-matched controls using cTDI, which confirms the results of a recent study in paediatric patients by Brado et al [ 12 ] where a prolonged myocardial contraction duration was seen in paediatric LQTS patients using MRI. The prolongation of the myocardial contraction in our study is in approximately the same order of magnitude as described by Haugaa et al [ 8 ] in adult LQTS patients using cTDI (about 40 ms) and had a concordant high inter- and intra-observer validity.…”

Section: Discussionsupporting

confidence: 91%

“…It is postulated that a deranged calcium homeostasis may play a role in the underlying mechanism of this mechanical dysfunction in LQTS [ 3 , 4 , 6 – 8 , 12 , 41 ]. A prolonged QT-interval in LQTS implies a prolonged action potential duration (APD), and this may increase the calcium influx via L-type calcium channel and a concomitant increase in the calcium-induced calcium release from the sarcoplasmic reticulum.…”

Section: Discussionmentioning

confidence: 99%

“…Although, patients with LQTS have a normal left ventricular function, several studies have shown mechanical abnormalities in the contraction and relaxation pattern in adult LQTS patients using M-Mode echocardiography,[ 3 – 5 ] continuous-wave Doppler images,[ 6 ] colour Tissue Doppler Imaging (cTDI) [ 7 , 8 ] and Speckle Tracking [ 9 – 11 ]. One study has found mechanical dysfunction in children with the use of MRI [ 12 ]. These abnormalities were almost absent in healthy individuals, but prevalent among LQTS patients whereas in symptomatic patients this was even more frequent, suggesting their potential value in the diagnosis and risk stratification in LQTS.…”

Section: Introductionmentioning

confidence: 99%

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A Potential Diagnostic Approach for Foetal Long-QT Syndrome, Developed and Validated in Children

et al. 2018

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In patients with Long-QT Syndrome (LQTS), mechanical abnormalities have been described. Recognition of these abnormalities could potentially be used in the diagnosis of LQTS, especially in the foetus where an ECG is not available and DNA-analysis is invasive. We aimed to develop and validate a marker for these mechanical abnormalities in children and to test its feasibility in foetuses as a proof of principle. We measured the myocardial contraction duration using colour Tissue Doppler Imaging (cTDI) in 41 LQTS children and age- and gender-matched controls. Children were chosen to develop and validate the measurement of the myocardial contraction duration, due to the availability of a simultaneously recorded ECG. Feasibility of this measurement in foetuses was tested in an additional pilot study among seven LQTS foetuses and eight controls. LQTS children had a longer myocardial contraction duration compared to controls, while there was no statistical difference in heart rate. Measuring the myocardial contraction duration in children had a high inter- and intra-observer validity and reliably correlated with the QT-interval. There was an area under the curve (AUC) of 0.71, and the optimal cut-off value showed an especially high specificity in diagnosing LQTS. Measuring the myocardial contraction duration was possible in all foetuses and had a high inter- and intra-observer validity (ICC = 0.71 and ICC = 0.88, respectively). LQTS foetuses seemed to have a longer myocardial contraction duration compared to controls. Therefore, a prolonged contraction duration may be a potential marker for the prenatal diagnosis of LQTS in the future. Further studies are required to support the measurement of the myocardial contraction duration as a diagnostic approach for foetal LQTS.Electronic supplementary materialThe online version of this article (10.1007/s00246-018-1911-y) contains supplementary material, which is available to authorized users.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A Potential Diagnostic Approach for Foetal Long-QT Syndrome, Developed and Validated in Children

et al. 2018

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“…Data interpretation : Spatiotemporal EM heterogeneity is exaggerated in inherited LQTS with increased dispersion of myocardial repolarization during exercise and recovery preceding arrhythmias . Mechanical heterogeneity, reflected by prolonged myocardial contraction and increased regional and transmural mechanical dispersion, is known to be more pronounced in symptomatic LQTS .…”

Section: Discussionmentioning

confidence: 99%

Exercise worsening of electromechanical disturbances: A predictor of arrhythmia in long QT syndrome

Charisopoulou

Koulaouzidis

Rydberg

et al. 2018

Clinical Cardiology

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Background Electromechanical (EM) coupling heterogeneity is significant in long QT syndrome (LQTS), particularly in symptomatic patients; EM window (EMW) has been proposed as an indicator of interaction and a better predictor of arrhythmia than QTc. Hypothesis To investigate the dynamic response of EMW to exercise in LQTS and its predictive value of arrhythmia. Methods Forty‐seven LQTS carriers (45 ± 15 years, 20 with arrhythmic events), and 35 controls underwent exercise echocardiogram. EMW was measured as the time difference between aortic valve closure on Doppler and the end of QT interval on the superimposed electrocardiogram (ECG). Measurements were obtained at rest, peak exercise (PE) and 4 minutes into recovery. Results Patients did not differ in age, gender, heart rate, or left ventricular ejection fraction but had a negative resting EMW compared with controls (−42 ± 22 vs 17 ± 5 ms, P < 0.0001). EMW became more negative at PE (−89 ± 43 vs 16 ± 7 ms, P = 0.0001) and recovery (−65 ± 39 vs 16 ± 6 ms, P = 0.001) in patients, particularly the symptomatic, but remained unchanged in controls. PE EMW was a stronger predictor of arrhythmic events than QTc (AUC:0.765 vs 0.569, P < 0.001). B‐blockers did not affect EMW at rest but was less negative at PE (BB: −66 ± 21 vs no‐BB: −113 ± 25 ms, P < 0.001). LQT1 patients had worse PE EMW negativity than LQT2. Conclusion LQTS patients have significantly negative EMW, which worsens with exercise. These changes are more pronounced in patients with documented arrhythmic events and decrease with B‐blocker therapy. Thus, EMW assessment during exercise may help improve risk stratification and management of LQTS patients.

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“…Aufgrund der elektromechanischen Kopplung bedingt eine verlängerte Repolarisation eine verlängerte Kontraktionsdauer, welche durch neuere Methoden der kardialen Bildgebung (Speckle-Tracking-Echokardiografie, Phasen-Kontrast-Kardio-MRT) nachgewiesen werden können. So konnte gezeigt werden, dass LQTS-Patienten nicht nur eine längere Kontraktionsdauer, sondern auch eine erhöhte räumliche Dispersion der Kontraktionsdauer aufweisen [4] und dass das Ausmaß der mechanischen Dispersion (im Gegensatz zu den oben erwähnten EKG-Parametern) mit dem Auftreten von Arrhythmien korreliert [5]. Um verlässliche und im klinischen Alltag verwendbare "Cut-off"-Werte zu erhalten, werden die Verfahren aktuell in größeren klinischen Studien getestet.…”

Section: Long-qt-syndromunclassified

Familiäre Arrhythmieerkrankungen mit strukturell normalen Herzen

Brado

Odening

2018

Aktuel Kardiol

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ZusammenfassungAngeborene kardiale Ionenkanalerkrankungen/familiäre Arrhythmieerkrankungen sind häufige Ursachen für arrhythmogene Synkopen und den plötzlichen Herztod bei jungen Erwachsenen. Sie sind mittlerweile bei rechtzeitiger Diagnose und adäquater antiarrhythmischer Therapie gut beherrschbar. Der (verbesserten) Risikostratifizierung kommt in der Behandlung eine entscheidende Rolle zu. Da sich das arrhythmogene Risiko mit der Zeit ändern kann, sind regelmäßige Verlaufskontrollen indiziert. Die genetischen und pathophysiologischen Grundlagen werden zunehmend besser verstanden und so gezieltere (mechanismusbasierte) Therapien möglich.

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Phase-contrast magnet resonance imaging reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in patients with long QT syndrome

Cited by 25 publications

References 23 publications

A Potential Diagnostic Approach for Foetal Long-QT Syndrome, Developed and Validated in Children

A Potential Diagnostic Approach for Foetal Long-QT Syndrome, Developed and Validated in Children

Exercise worsening of electromechanical disturbances: A predictor of arrhythmia in long QT syndrome

Familiäre Arrhythmieerkrankungen mit strukturell normalen Herzen

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