Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients
Corresponding Author
Raphael Henrique Déa Cirino MD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Correspondence to: R.H. Déa Cirino; e-mail: [email protected]Search for more papers by this authorRosana Herminia Scola MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorRenata Dal-Prá Ducci MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorAna Cristina Camarozano Wermelinger MD, PhD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Search for more papers by this authorClaudia Suemi Kamoi Kay MD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorPaulo José Lorenzoni MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorLineu Cesar Werneck MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorEliane Ribeiro Carmes MD, MSc
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorClaudio Leinig Pereira Da Cunha MD, PhD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorCorresponding Author
Raphael Henrique Déa Cirino MD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Correspondence to: R.H. Déa Cirino; e-mail: [email protected]Search for more papers by this authorRosana Herminia Scola MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorRenata Dal-Prá Ducci MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorAna Cristina Camarozano Wermelinger MD, PhD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Search for more papers by this authorClaudia Suemi Kamoi Kay MD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorPaulo José Lorenzoni MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorLineu Cesar Werneck MD, PhD
Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorEliane Ribeiro Carmes MD, MSc
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorClaudio Leinig Pereira Da Cunha MD, PhD
Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12 º andar Curitiba, 80060-900 Paraná, Brazil
Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil
Search for more papers by this authorConflicts of Interest: None of the authors have any conflict of interest to disclose.
ABSTRACT
Introduction: Early detection of left ventricular systolic dysfunction (LVSD) is important for therapeutic strategies for Duchenne muscular dystrophy (DMD) patients. We analyzed myocardial strain using echocardiography for early detection of LVSD and determined the predictors of early LVSD. Methods: This investigation was a cross-sectional study of 40 DMD patients with normal left ventricular ejection fraction. Global longitudinal strain (GLS) was used to analyze subtle disturbances in longitudinal contraction of the myocardium. Patients were determined to have early LVSD (GLS > –18) or normal left ventricular systolic function (GLS ≤ –18). Results: Patients who had early LVSD were older and had a higher frequency of corticosteroid therapy and of mutations in exons 45, 46, 47, 48, 49, 50, and 52. Discussion: Myocardial strain measurements are useful for the early diagnosis of LVSD in DMD patients. Older age, use of corticosteroids, and mutations within the “hot-spot” region of the DMD gene are associated with early LVSD. Muscle Nerve 58: 84–89, 2018
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