The Heart Rate in Progressive Muscular Dystrophy

Boas, Ernst P.; Lowenburg, Henrietta

doi:10.1001/archinte.1931.00140210039003

Cited by 41 publications

(6 citation statements)

References 5 publications

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“…The most frequent electrocardiographic abnormalities reported havebeentachycardia (Boas and Lowenberg, 1931;Manning and Cropp, 1958;Levin et al, 1959), tall R waves in right praecordial leads, with deep Q waves in limb leads and! Mean ±SD 8-9…”

Section: Discussionmentioning

confidence: 99%

Echocardiographic assessment of left ventricular function in Duchenne's muscular dystrophy.

Ahmad¹,

Sanderson²,

Dubowitz³

et al. 1978

Heart

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suMMARY Left ventricular function was assessed echocardiographically in 13 children with Duchenne's muscular dystrophy. Ejection fraction, mean velocity of circumferential fibre shortening, normalised left ventricular posterior wall velocity, maximal systolic endocardial velocity, and maximal diastolic endocardial velocity were determined manually by measuring.directly from the echocardiographic tracings. The echocardiograms were then digitised and results computed in order to study the peak rates of change of dimension, the peak lengthening rates, the peak shortening rates, and the time relation between the mitral valve and left ventricular posterior wall movement in early diastole.All the systolic phase indices were within normal limits. Though maximal diastolic endocardial velocity (11.1 ± 3 2 cm/s = mean ± SD) was reduced in patients with Duchenne's muscular dystrophy compared with normal controls (15.3 3-2 cm/s), the peak rates of change of dimension and the peak lengthening rates were identical with normal subjects. The time relation between the mitral valve and left ventricular posterior wall movement was not significantly different from normal. The maintenance of this close time relation and the fact that the ventricle is filling normally, as evidenced by normal peak filling rates, suggest that the myocardial function is well preserved in children with Duchenne's muscular dystrophy. The reduced maximal diastolic endocardial velocity may be the result of reduced overall movement of the heart within the thorax.Cardiac involvement is now a well-recognised feature of Duchenne's muscular dystrophy though heart failure is not a common cause of death. Electrocardiographic abnormalities are frequent and until recently have given the earliest indication of myocardial involvement. Since the changes in the electrocardiogram commonly suggest abnormality of the posterior left ventricular wall, a part of the heart which is well suited to study by echocardiography, the use of this non-invasive technique is particularly attractive for the study of children. Kovick et al. (1975)

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Section: Discussionmentioning

confidence: 99%

Echocardiographic assessment of left ventricular function in Duchenne's muscular dystrophy.

Ahmad¹,

Sanderson²,

Dubowitz³

et al. 1978

Heart

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“…However, although most patients die from respiratory failure, a significant number succumb to cardiac causes (Gilroy et al, 1936;Perloff et al, 1966). Cardiac involvement is observed in almost all cases of DMD (Ahmed et al, 1978;Boas, 1931). It is characterized by sinus tachycardia (Gilroy et al, 1936, Heymsfield et al, 19781, arrhythmias, non-specific murmurs, and a distinctive electrocardiographic (ECG) pattern (Fitch and Ainger, 1976).…”

Section: Introductionmentioning

confidence: 99%

Dystrophin expression in the developing conduction system of the human heart

Ginjaar

Virágh²,

Markman

et al. 1995

Microscopy Res & Technique

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Duchenne muscular dystrophy (DMD) is frequently associated with myocardial involvement. Dystrophin, the DMD protein, is found at the plasmamembrane of striated muscle fibers. Although dystrophin is missing in most or all muscle fibers of DMD patients, cardiac muscle is not as severely affected as skeletal muscle. Therefore it is of great importance to study the expression of dystrophin in normal cardiac muscle. We performed immunohistochemical studies and examined cardiac muscle of fetuses of 8 to 13 weeks of development on dystrophin expression. At these stages dystrophin is observed in the myocytes of the developing ventricular conduction system and in the atrial cardiomyocytes. Dystrophin was absent from the heart of a 12-week-old DMD fetus.

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“…Patients with Duchenne muscular dystrophy exhibit normal MAP and increased HR (Boas & Lowenburg, 1931; Sander et al 2000). Our results are consistent with this pattern in that MAP did not differ and HR was higher in Sgcd−/− vs. control mice when measured during the day when activity levels were low in both groups of mice (Fig 1, Table 1).…”

Section: Discussionmentioning

confidence: 99%

Angiotensin‐dependent autonomic dysregulation precedes dilated cardiomyopathy in a mouse model of muscular dystrophy

Sabharwal

Weiß

Zimmerman

et al. 2015

Experimental Physiology

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Sarcoglycan mutations cause muscular dystrophy. Patients with muscular dystrophy develop autonomic dysregulation and dilated cardiomyopathy (DCM), but the temporal relationship and mechanism of autonomic dysregulation are not well understood. We hypothesized that activation of the renin-angiotensin system (RAS) causes autonomic dysregulation prior to development of DCM in sarcoglycan-delta (Sgcd) deficient mice, and that the severity of autonomic dysfunction at a young age predicts the severity of DCM at older ages. At 10-12 weeks of age, when left ventricular function assessed by echocardiography remained normal, Sgcd−/− mice exhibited decreases in arterial pressure, locomotor activity, baroreflex sensitivity (BRS) and cardiovagal tone, and increased sympathetic tone compared with age-matched C57BL/6 control mice (P<0.05). Systemic and skeletal muscle RAS were activated, and angiotensin II type 1 receptor (AT1R) expression, superoxide and fibrosis were increased in dystrophic skeletal muscle (P<0.05). Treatment with the AT1R blocker losartan for 7-9 weeks beginning at 3 weeks of age prevented or strongly attenuated the abnormalities in Sgcd−/− mice (P<0.05). Repeated assessment of phenotypes between 10 and 75 weeks of age demonstrated worsening of autonomic function, progressive cardiac dysfunction and DCM, and increased mortality in Sgcd−/− mice. High sympathetic tone predicted subsequent left ventricular dysfunction. We conclude that RAS activation causes severe autonomic dysregulation in young Sgcd−/− mice, which portends a worse long-term prognosis. Therapeutic targeting of RAS at a young age may improve autonomic function and slow disease progression in muscular dystrophy.

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The Heart Rate in Progressive Muscular Dystrophy

Cited by 41 publications

References 5 publications

Echocardiographic assessment of left ventricular function in Duchenne's muscular dystrophy.

Echocardiographic assessment of left ventricular function in Duchenne's muscular dystrophy.

Dystrophin expression in the developing conduction system of the human heart

Angiotensin‐dependent autonomic dysregulation precedes dilated cardiomyopathy in a mouse model of muscular dystrophy

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