1. Value of Global Longitudinal Strain for Identification and Monitoring of Left Ventricular Dysfunction in Becker Muscular Dystrophy
- Author
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Erik H. Niks, Nina Ajmone Marsan, Jeroen J. Bax, Tea Gegenava, Nienke M van de Velde, A. A. W. Roest, Douwe E. Atsma, Steele C Butcher, Zaïda Koeks, and Pietro Spitali
- Subjects
Adult ,Male ,medicine.medical_specialty ,Longitudinal strain ,medicine.drug_class ,Ventricular Function, Left ,Pulmonary function testing ,Ventricular Dysfunction, Left ,Predictive Value of Tests ,Serum biomarkers ,Internal medicine ,Outcome Assessment, Health Care ,Troponin I ,medicine ,Natriuretic peptide ,Humans ,Muscular dystrophy ,Cause of death ,Ejection fraction ,business.industry ,Stroke Volume ,Middle Aged ,medicine.disease ,Muscular Dystrophy, Duchenne ,Echocardiography ,Case-Control Studies ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Cardiac involvement is the main cause of death in Becker muscular dystrophy (BMD). Identification of left ventricular (LV) function is crucial, but standard echocardiographic measurements such as LV ejection fraction (LVEF) might not be sensitive enough to detect early myocardial dysfunction. We explored the value of LV global longitudinal strain (GLS) as a more accurate echocardiographic parameter to detect and monitor LV dysfunction in BMD. Furthermore, we studied possible factors associated with LV dysfunction and progression. A total of 40 patients with BMD (age 39.0 +/- 13.2 years) and 21 matched controls were included. Clinical variables, pulmonary tests, serum biomarkers, and echocardiograms were collected at baseline and after 2 years. LV systolic function was assessed by LVEF and LV GLS; a significant progression in LV dysfunction was defined as an absolute LV GLS deterioration >= 15%. Responsiveness to cardiac disease progression was determined using standardized response means. Patients showed impaired LVEF and LV GLS compared with controls (p
- Published
- 2022
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