Prevalence of left ventricular systolic dysfunction in Duchenne muscular dystrophy: An echocardiographic study

Kermadec, Jean-Michel de; Becane, H.M.; Chenard, Alain; Tertrain, F.; Weiss, Y

doi:10.1016/0002-8703(94)90672-6

Cited by 88 publications

(52 citation statements)

References 24 publications

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“…Three of 4 regions manifested significantly decreased circumferential strain, as did the basal and midventricular segments by repeated-measures ANOVA. In contrast to the present findings, previous investigations with standard echocardiography and radionuclide imaging have reported the primary defects of cardiac involvement in DMD to reside within the inferior 20,21 or posterolateral walls. 22 The reason for this difference most probably reflects the enhanced sensitivity of CMR for detecting widespread but subtle changes of intramural contractile function, which are interpretable only at regional or global levels with the use of alternative technologies.…”

Section: Discussioncontrasting

confidence: 99%

Occult Cardiac Contractile Dysfunction in Dystrophin-Deficient Children Revealed by Cardiac Magnetic Resonance Strain Imaging

et al. 2005

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Background-Duchenne muscular dystrophy (DMD) is an inherited disease characterized by early onset of skeletal muscle degeneration and progressive weakness. Although dilated cardiomyopathy may occur during adolescence, it is often undetected early in its course because of physical inactivity and generalized debilitation. The purpose of this study was to apply the technique of cardiac magnetic resonance (CMR) tagging to detect occult cardiac dysfunction in young subjects with DMD by measuring myocardial strain and torsion. Methods and Results-Thirteen DMD pediatric subjects without clinically apparent heart disease and 9 age-matched healthy males were recruited. Each was scanned on a 1.5-T clinical scanner to acquire contiguous short-axis planes from the apex to the mitral valve plane and then 3 tagged images at base, midventricle, and apex. Global and segmental myocardial net twist and circumferential strain were computed with the use of 2D homogeneous strain analysis. Ventricular torsion was computed by normalizing net twist by the distance from apex to mitral valve plane. DMD patients exhibited normal left ventricular volumes and ejection fractions but manifested reduced midventricular and basal cross-sectional global circumferential strain compared with the reference group (PϽ0.005). These alterations also appeared in segmental analyses in the septal, anterior, lateral, and inferior walls (PϽ0.05). Conclusions-In patients predisposed to cardiomyopathies because of dystrophinopathy, occult regional cardiac dysfunction can be diagnosed with CMR tagging. This method of strain imaging analysis may offer a sensitive approach for delineating the presence and progression of cardiovascular disease and for assessing therapies designed to modulate the onset and course of heart failure.

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Section: Discussioncontrasting

confidence: 99%

Occult Cardiac Contractile Dysfunction in Dystrophin-Deficient Children Revealed by Cardiac Magnetic Resonance Strain Imaging

et al. 2005

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“…[104][105][106] The myocardium at autopsy displays areas of myocyte hypertrophy, atrophy, and fibrosis. 107 Progressive cardiomyopathy is currently a major source of morbidity and mortality in DMD and Becker muscular dystrophy, particularly since advances have been made in the treatment of the muscle disease and pulmonary function.…”

Section: Cardiac Managementmentioning

confidence: 99%

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Bushby

Finkel

Birnkrant

et al. 2010

The Lancet Neurology

1,719

1,596

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“…Cardiomyopathy is a common feature developing in the second decade. [2], [3], [4] Death usually occurs in the second to third decades of life from respiratory or cardiac failure. As the treatment of skeletal and respiratory systems improves and patients live longer, an estimated 10-20% of DMD patients are dying of complications from cardiomyopathy.…”

Section: Introductionmentioning

confidence: 99%

Dystrophin-deficient cardiomyopathy in mouse: Expression of Nox4 and Lox are associated with fibrosis and altered functional parameters in the heart

Spurney

Knoblach

Pistilli

et al. 2008

Neuromuscular Disorders

136

133

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Duchenne muscular dystrophy (DMD; dystrophin-deficiency) causes dilated cardiomyopathy in the second decade of life in affected males. We studied the dystrophin-deficient mouse heart (mdx) using high frequency echocardiography, histomorphometry, and gene expression profiling. Heart dysfunction was prominent at 9-10 months of age and showed significantly increased LV internal diameter (end systole) and decreased posterior wall thickness. This cardiomyopathy was associated with a 30% decrease in shortening fraction. Histologically, there was a 10-fold increase in connective tissue volume (fibrosis). mRNA profiling with RT-PCR validation showed activation of key profibrotic genes, including Nox4 and Lox. The Nox gene family expression differed in mdx heart and skeletal muscle, where Nox2 was specifically induced in skeletal muscle while Nox4 was specifically induced in heart. This is the first report of an altered profibrotic gene expression profile in cardiac tissue of dystrophic mice showing echocardiographic evidence of cardiomyopathy.

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Prevalence of left ventricular systolic dysfunction in Duchenne muscular dystrophy: An echocardiographic study

Cited by 88 publications

References 24 publications

Occult Cardiac Contractile Dysfunction in Dystrophin-Deficient Children Revealed by Cardiac Magnetic Resonance Strain Imaging

Occult Cardiac Contractile Dysfunction in Dystrophin-Deficient Children Revealed by Cardiac Magnetic Resonance Strain Imaging

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

Dystrophin-deficient cardiomyopathy in mouse: Expression of Nox4 and Lox are associated with fibrosis and altered functional parameters in the heart

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