Increased temporal dispersion of myocardial repolarization in myotonic dystrophy type 1: beyond the cardiac conduction system |
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Authors: | Magrì Damiano Piccirillo Gianfranco Bucci Elisabetta Pignatelli Giulia Cauti Filippo Maria Morino Stefania Latino Pamela Santini Daria Marrara Federica Volpe Massimo Antonini Giovanni Testa Marco |
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Affiliation: | a Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital of Muenster, Muenster, Germanyb Department of Clinical Radiology, University Hospital of Muenster, Muenster, Germany |
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Abstract: | ObjectivesTo assess ventricular dysfunction and ventricular interaction after repair of Tetralogy of Fallot (ToF) employing echocardiography speckle-tracking and cardiac magnetic resonance imaging (CMR).BackgroundSevere pulmonary regurgitation and right ventricular (RV) dysfunction are common after repair of ToF and may also affect the shape and function of the left ventricle (LV). Recent studies suggest that LV dysfunction may be of particular prognostic value.Methods and resultsTwenty-one consecutive adults with repaired ToF (15 male, mean age 38 ± 11 years, 7 with severe PR) underwent a comprehensive echocardiographic exam including speckle-tracking analysis, CMR and cardiopulmonary exercise testing. Twenty-one subjects without relevant heart disease served as controls. Echocardiographically measured RV diameters correlated with RV volumes obtained from CMR (r = 0.63; p = 0.006). In addition, a close correlation was found between RV and LV function on CMR (r = 0.74, p = 0.002), speckle-tracking LV and RV peak longitudinal 2D strain (r = 0.66, p = 0.003) and mitral and tricuspid annular plain systolic excursion (r = 0.71, p = 0.0003). While LV ejection fraction was normal in the majority of patients and not different from controls, LV longitudinal strain was significantly reduced in ToF patients (− 16.5 ± 3.3 vs. -20.5 ± 2.7%, p = 0.0001).ConclusionLeft and right ventricular function both by CMR and speckle-tracking is interrelated in adults with repaired ToF. Despite normal LV ejection fraction, 2D longitudinal strain is significantly reduced in ToF patients, suggesting subclinical LV myocardial damage. Considering the potential prognostic value of LV dysfunction in ToF, this measurement may gain importance and should be included in future outcome studies. |
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Keywords: | BSA, body surface area CMR, cardiac magnetic resonance CPET, cardiopulmonary testing LV, left ventricle LVEDD, left ventricular end-diastolic diameter LVEDV, left ventricular end-diastolic volume LVEDVi, left ventricular end-systolic diameter index LVEF, left ventricular ejection fraction LVESD, left ventricular end-systolic diameter LVESV, left ventricular end-systolic volume MAPSE, mitral annulus plane systolic excursion NYHA, New York Heart Association PV, pulmonary valve RV, right ventricle RVEDVi, right ventricular end-diastolic volume index RVEF, right ventricular ejection fraction RVESVi, right ventricular end-systolic volume index TAPSE, tricuspid annulus plane systolic excursion ToF, Tetralogy of Fallot VO2, peak oxygen consumption |
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