Integrated Backscatter in Becker Muscular Dystrophy Patients With Functionally Normal Heart: Myocardial Ultrasound Tissue Characterization Study

Cardiac involvement (leading to cardiomyopathy and heart failure) is frequent, age-dependent, and unpredictable (1). Since there is no direct relationship between severity of skeletal and cardiac involvement, cardiomyopathy frequently develops in patients with normal skeletal muscle function (2). In...

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Veröffentlicht in:Journal of the American College of Cardiology 2006-02, Vol.47 (3), p.686-688
Hauptverfasser: Giglio, Vincenzo, Pasceri, Vincenzo, Messano, Loredana, Parisi, Quintino, Rinelli, Gabriele, Pasquini, Luciano, Dello Russo, Antonio, Broccolini, Aldo, Galluzzi, Giuliana, Pescatori, Mario, Mirabella, Massimiliano, Gidaro, Teresa, Mangiola, Fortunato, Ricci, Enzo
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container_end_page 688
container_issue 3
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container_title Journal of the American College of Cardiology
container_volume 47
creator Giglio, Vincenzo
Pasceri, Vincenzo
Messano, Loredana
Parisi, Quintino
Rinelli, Gabriele
Pasquini, Luciano
Dello Russo, Antonio
Broccolini, Aldo
Galluzzi, Giuliana
Pescatori, Mario
Mirabella, Massimiliano
Gidaro, Teresa
Mangiola, Fortunato
Ricci, Enzo
description Cardiac involvement (leading to cardiomyopathy and heart failure) is frequent, age-dependent, and unpredictable (1). Since there is no direct relationship between severity of skeletal and cardiac involvement, cardiomyopathy frequently develops in patients with normal skeletal muscle function (2). In BMD patients, differences in the amount of myocardial dystrophin and in the site of the deletion, as well as other still-unidentified factors, are probably the molecular basis of the cardiac involvement that is common in BMD patients, with ECG changes in about 70% of cases (2) and reduced systolic function in up to 60% (1). Since the muscle involvement is often mild and heart disease is a frequent cause of death in BMD (in up to 50% of patients) (5), cardiac transplantation has been successfully performed in these patients.
doi_str_mv 10.1016/j.jacc.2005.11.001
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Since there is no direct relationship between severity of skeletal and cardiac involvement, cardiomyopathy frequently develops in patients with normal skeletal muscle function (2). In BMD patients, differences in the amount of myocardial dystrophin and in the site of the deletion, as well as other still-unidentified factors, are probably the molecular basis of the cardiac involvement that is common in BMD patients, with ECG changes in about 70% of cases (2) and reduced systolic function in up to 60% (1). 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source MEDLINE; Elsevier ScienceDirect Journals Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection
subjects Adolescent
Adult
Age
Cardiology
Cardiomyopathy
Child
Child, Preschool
Echocardiography
Heart
Humans
Hypotheses
Muscular dystrophy
Muscular Dystrophy, Duchenne - diagnostic imaging
Muscular Dystrophy, Duchenne - physiopathology
Musculoskeletal system
Myocardial Contraction
Physical properties
Rodents
Ultrasonic imaging
title Integrated Backscatter in Becker Muscular Dystrophy Patients With Functionally Normal Heart: Myocardial Ultrasound Tissue Characterization Study
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