Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy

Takenaka, Akira; Yokota, Mitsuhiro; Iwase, Mitsunori; Miyaguchi, Kazuhiko; Hayashi, Hiroshi; Saito, Hironori

doi:10.1093/eurheartj/14.5.669

Cited by 32 publications

(19 citation statements)

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“…There were insignificant A wave changes of diastolic mitral valve flow, expressed in A-vmax and A-tvi, respectively. Similar findings were reported by Takenaka et al [34]. Thus far there is no explanation for this behavior.…”

Section: Discussionsupporting

confidence: 90%

X-Chromosomal (p21) Muscular Dystrophy and Left Ventricular Diastolic and Systolic Function

et al. 1998

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In order to screen for cardiac abnormalities, we prospectively studied 15 patients (age 8-25 years, mean 15.5 years) with Duchenne's (DMD) (n = 9) and Becker's (BMD) (n = 6) muscular dystrophy using the echocardiogram. Data were compared to a control group of 92 healthy individuals (age 7.9-25 years, mean 14.3 years). Left ventricular filling in diastole showed a different pattern when comparing echocardiographic Doppler results in patients and controls: Patients had lower peak velocity of early left ventricular diastolic filling (E-vmax)(P < 0.0001) and smaller time velocity integral of the E-wave (E-tvi)(P < 0.0001). In contrast, the atrial component (A-vmax, A-tvi) of diastolic filling in DMD/ BMD showed no significant difference to controls. The mean area of the mitral valve orifice was significantly larger in patients (P < 0.0001) without presence of mitral regurgitation. Systolic left ventricular function was significantly impaired in the DMD/BMD group; we found lower heart rate corrected fiber shortening velocity VCFc (P < 0.001) and higher peak systolic wall stress (P < 0.001) in DMD/BMD. In 8 of 15 patients, peak systolic wall stress was above 95th percentile of controls. In 6 of 15 patients, VCFc was lower than the 5th percentile of controls. Systolic and diastolic myocardial impairment was found even in young patients and at low stages of disability--equally among patients with DMD or BMD. Diastolic left ventricular impairment predominantly affected the early diastolic filling, but atrial compensation was poor. Peak systolic wall stress measurements were particularly useful in patients with CMP, reflecting the left ventricular afterload.

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Section: Discussionsupporting

confidence: 90%

X-Chromosomal (p21) Muscular Dystrophy and Left Ventricular Diastolic and Systolic Function

et al. 1998

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“…Severe systolic dysfunction was found in single patients already at their early teens. Diastolic dysfunction was not observed, resulting in the conclusion that DMD patients predominantly develop progressive left ventricular systolic dysfunction [72]. In a study on 23 DMD patients, 5 patients demonstrated atrophic heart, defined as decreasing LVEDD over years [73].…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

“…Classical ECG abnormalities at the subclinical stage are sinus tachycardia, tall R waves in V1, increased R/S ratio in V1, deep Q waves in I, aVL, V5–V6, II, III and aVF and increased QT dispersion. ECG abnormalities predict the cardiac pathology [72]. Echocardiographically, there may be regional wall motion abnormalities and systolic dysfunction, starting in the 1st decade of life.…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

The Heart in Human Dystrophinopathies

2003

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Dystrophinopathies are due to mutations in the dystrophin gene on chromosome Xp21.1 and comprise the allelic entities Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) and X-linked dilative cardiomyopathy (XLDCM). In all three entities, the heart is affected to various degrees, depending on the stage of the disease and the type of the mutation (cardiac involvement, CI). The pathoanatomic evidence of CI in dystrophinopathies is the replacement of myocardium by connective tissue or fat. In DMD/BMD, the left ventricular posterobasal and lateral walls are most extensively affected, sparing the right ventricle and the atrium. Degree and dynamics of CI vary among the three entities. In DMD/BMD, CI usually remains subclinical in the early stages of the disease. Typical initial manifestations of CI in DMD/BMD are sinus tachycardia, tall R1 in V1, prominent Q in I, aVL, V6 or in II, III, and aVF, increased QT dispersion and possibly autonomic dysfunction. Initially, echocardiography is normal or shows regional wall motion abnormalities in areas of fibrosis. With spreading of fibrosis, left ventricular dysfunction and ventricular arrhythmias additionally occur. In the final stages of the disease, systolic function may lead to heart failure and sudden death. Subclinical or clinical CI is present in about 90% of the DMD/BMD patients but is the cause of death in only 20% of the DMD and 50% of the BMD patients. XLDCM is a rapidly progressive, almost exclusively myocardial disorder, starting in teenage males as heart failure due to dilative cardiomyopathy (CMP), leading to death from intractable heart failure within 1–2 years after diagnosis. Therapy of arrhythmias and CMP in all three disorders follows the established cardiological recommendations. Due to its protective effect, ACE inhibitors are recommended already at the early stages of the disease. β-Blockers may be an additional option if indicated.

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“…Takenaka et al [20] found significant decrease in EF values in MD patients. Also Farah et al [3] showed significant decrease in both EF and FS values.…”

Section: Discussionmentioning

confidence: 93%

A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

et al. 1998

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Muscular dystrophies are a group of sex-linked diseases with frequent myocardial involvement. In this study 14 patients with Duchenne Muscular Dystrophy (DMD), 7 with Becker Muscular Dystrophy (BMD), and 8 female carriers who were asymptomatic were evaluated with echocardiography and multigated radionuclide ventriculography (MUGA). All showed predominant systolic and minor diastolic left ventricular dysfunction determined both by echocardiography and MUGA when compared with healthy controls. In conclusion, it is recommended that DMD, BMD, and female carriers be evaluated and closely monitored for cardiac functions. Though MUGA and echocardiography are both effective and sensitive techniques, echocardiography is more convenient for availability and serial evaluation.

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Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy

Cited by 32 publications

References 0 publications

X-Chromosomal (p21) Muscular Dystrophy and Left Ventricular Diastolic and Systolic Function

X-Chromosomal (p21) Muscular Dystrophy and Left Ventricular Diastolic and Systolic Function

The Heart in Human Dystrophinopathies

A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

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