Yolanda Vives-Gilabert1, Jorge Sanz-Sánchez2, Pilar Molina3, Antonio Cebrián1, Begoña Igual4, Pilar Calvillo-Batllés5, Diana Domingo2, José Millet1, Luis Martínez-Dolz6, Francisco Castells7, Esther Zorio8. 1. Instituto ITACA, Universitat Politècnica de València, Valencia, Spain. 2. Unidad de Cardiopatías Familiares, Muerte Súbita y Mecanismos de Enfermedad (CaFaMuSMe), Instituto de Investigación Sanitaria La Fe, Valencia, Spain; Servicio de Cardiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 3. Unidad de Cardiopatías Familiares, Muerte Súbita y Mecanismos de Enfermedad (CaFaMuSMe), Instituto de Investigación Sanitaria La Fe, Valencia, Spain; Servicio de Patología, Instituto de Medicina Legal y Ciencias Forenses, Valencia, Spain. 4. Servicio de Cardiología, Hospital General Universitario, Valencia, Spain; Unidad de Imagen Cardiaca, ERESA, Valencia, Spain. 5. Servicio de Radiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 6. Servicio de Cardiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 7. Instituto ITACA, Universitat Politècnica de València, Valencia, Spain. Electronic address: fcastells@eln.upv.es. 8. Unidad de Cardiopatías Familiares, Muerte Súbita y Mecanismos de Enfermedad (CaFaMuSMe), Instituto de Investigación Sanitaria La Fe, Valencia, Spain; Servicio de Cardiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. Electronic address: zorio_est@gva.es.
Abstract
BACKGROUND: Diagnostic Task Force Criteria (TFC) for arrhythmogenic cardiomyopathy (AC) exhibit poor performance for left dominant forms. TFC only include right ventricular (RV) dysfunction (akinesia, dyssynchrony, volumes and ejection fraction). Moreover, cardiac magnetic resonance imaging (CMRI) assessment of left ventricular (LV) dyssynchrony has hitherto not been described. Thus, we aimed to comprehensively characterize LV CMRI behavior in AC patients. METHODS: Thirty-five AC patients with LV involvement and twenty-three non-affected family members (controls) were enrolled. Feature-tracking analysis was applied to cine CMRI to assess LV ejection fraction (LVEF), LV end-systolic and end-diastolic volume indexes, strain values and dyssynchrony. Regions with more frequent strain and dyssynchrony impairment were also studied. RESULTS: Radial dyssynchrony and LVEF were selected (sensitivities 54.3% and 48.6%, respectively at 100% specificity), with a threshold of 70 ms for radial dyssynchrony and 48.5% for LVEF. 71.4% of patients exceeded these thresholds (31.4% both, 22.9% only dyssynchrony and 17.1% only LVEF). Considering these cut-off values as a novel combined criterion, 30% of patients with 'borderline' or 'possible' AC following 2010 TFC would move to a 'definite' AC diagnosis. Strain was globally impaired whereas dyssynchronous regions were more often apical and located at the inferolateral wall. CONCLUSIONS: Mirroring the RV evaluation, we suggest including LVEF and LV dyssynchrony to improve the diagnosis of AC. Two independent mechanisms can be claimed in AC patients with LV involvement: 1) decreased myocardial deformation with global LV affectation and 2) delayed myocardial contraction at localized regions.
BACKGROUND: Diagnostic Task Force Criteria (TFC) for arrhythmogenic cardiomyopathy (AC) exhibit poor performance for left dominant forms. TFC only include right ventricular (RV) dysfunction (akinesia, dyssynchrony, volumes and ejection fraction). Moreover, cardiac magnetic resonance imaging (CMRI) assessment of left ventricular (LV) dyssynchrony has hitherto not been described. Thus, we aimed to comprehensively characterize LV CMRI behavior in AC patients. METHODS: Thirty-five AC patients with LV involvement and twenty-three non-affected family members (controls) were enrolled. Feature-tracking analysis was applied to cine CMRI to assess LV ejection fraction (LVEF), LV end-systolic and end-diastolic volume indexes, strain values and dyssynchrony. Regions with more frequent strain and dyssynchrony impairment were also studied. RESULTS: Radial dyssynchrony and LVEF were selected (sensitivities 54.3% and 48.6%, respectively at 100% specificity), with a threshold of 70 ms for radial dyssynchrony and 48.5% for LVEF. 71.4% of patients exceeded these thresholds (31.4% both, 22.9% only dyssynchrony and 17.1% only LVEF). Considering these cut-off values as a novel combined criterion, 30% of patients with 'borderline' or 'possible' AC following 2010 TFC would move to a 'definite' AC diagnosis. Strain was globally impaired whereas dyssynchronous regions were more often apical and located at the inferolateral wall. CONCLUSIONS: Mirroring the RV evaluation, we suggest including LVEF and LV dyssynchrony to improve the diagnosis of AC. Two independent mechanisms can be claimed in AC patients with LV involvement: 1) decreased myocardial deformation with global LV affectation and 2) delayed myocardial contraction at localized regions.
Authors: Nick van Osta; Aurore Lyon; Feddo Kirkels; Tijmen Koopsen; Tim van Loon; Maarten J Cramer; Arco J Teske; Tammo Delhaas; Wouter Huberts; Joost Lumens Journal: Philos Trans A Math Phys Eng Sci Date: 2020-05-25 Impact factor: 4.226