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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cardiac involvement is common in patients with Duchenne type muscular dystrophy (DMD). However, published reports of long-term follow-up studies in the same patients are very rare. The purpose of this study was to clarify the natural course of cardiac dysfunction in DMD, and to analyze the relationship between the deterioration of cardiac function and skeletal muscular function. The cardiac function was serially evaluated for 2-12 years in 34 patients with DMD. The systolic time intervals and echocardiography were used for evaluating the cardiac function. The PEP/ET (pre-ejection period/ejection time) of systolic time intervals significantly deteriorated in 9-12 years (p less than 0.001). The maximal systolic and diastolic endocardial velocity (MSEV and MDEV) and the D/S ratio of echocardiography significantly deteriorated in 5 years (p less than 0.001). The D/S ratio is the ratio of the mean systolic velocity and the mean diastolic velocity of the left ventricular posterior wall. In almost half the patients, the deterioration of these parameters was correlated with that of Swinyard's stage. The Swinyard's functional ability stage demonstrates the grade of the skeletal muscular dysfunction. However, there was no correlation between the deterioration of cardiac function and Swinyard's stage in the other half of the patients. Correlation coefficient in the whole group was 0.32 (p = n.s.) between the PEP/ET and Swinyard's stage, and was -0.15 (p = n.s.) between the D/S ratio and Swinyard's stage. Although the mean left ventricular ejection fraction calculated using echocardiography was slightly decreased for 5 years, no significant change was detected in the whole group. One patient had progressive deterioration of left ventricular function despite mild skeletal muscular dysfunction, and died from congestive heart failure. The cardiac dysfunction was predominant. We named it 'cardiac type' DMD. Since deterioration of cardiac function was not always correlated with deterioration of skeletal muscular function (Swinyard's stage), cardiac function must be carefully monitored in patients with DMD.
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PMID:Long-term observation of cardiac function in Duchenne's muscular dystrophy. Evaluation using systolic time intervals and echocardiography. 227 53

In patients with Duchenne muscular dystrophy (DMD), heart failure appears in later stage of the disease due to myocardial degeneration and respiratory insufficiency, and sometimes causes death. However, there have been no adequate parameters which can be used easily to evaluate the grade of heart failure in DMD, except cardiac enlargement and pulmonary congestion observed by chest X-ray picture. Thus, we measured the plasma concentrations of atrial natriuretic peptide (ANP) in the patients with muscular dystrophy of various types, and studied a relationship between plasma ANP concentration and heart failure, expecting that it could be an index of heart failure in DMD patients. The plasma ANP concentrations in patients with DMD were 35.5 +/- 3.3pg/ml (mean +/- SE) and higher than in normal subjects (19.3 +/- 1.0pg/ml). In the patients with limb-girdle muscular dystrophy, facioscapulohumeral muscular dystrophy and neurogenic muscular atrophy, the plasma ANP concentration showed a tendency to elevate. However, no elevation of plasma ANP levels was observed in the patients with other types of muscular dystrophy. In DMD, number of the patients having a high plasma ANP concentration was increased with progress of disability grade, and decrease in serum creatine kinase activity and serum myoglobin concentration. There was a significant correlation (p less than 0.01) between plasma ANP concentration and cardiothoracic ratio or PEP/LVET, but no correlation between the concentration and respiratory failure. Immunohistochemistry of the atrial cardiac muscle of an autopsied DMD case revealed many ANP-positive atrial muscle cells, indicating the preservation of ANP-secreting function.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Secretion and clinical significance of atrial natriuretic peptide in patients with muscular dystrophy]. 252 1

Nine patients, 7 males, 2 females, mean age 36 years, with myotonic muscular dystrophy who had no cardiac symptoms underwent M-mode echocardiography (e.), systolic time intervals (STI) measurement by simultaneous recordings of the electrocardiogram, phonocardiogram and carotid arterial pulse, and single-pass radionuclide angiocardiography (RNA) in order to assess the left ventricular function. The ejecting phase indexes measured by echocardiography (fractional shortening, mean velocity of circumferential fiber shortening) were slightly depressed in 1 case and an abnormal PEP/LVET ratio was found in 3 cases. The ejection fraction measured by radionuclide angiocardiography was abnormal in 1 case who showed a diffuse hypokinesia. The IVI%, a new isovolumic phase index obtained by echocardiography, was abnormal in all patients. It is concluded that the IVI% seems more sensitive than the ejecting phase indexes calculated by echocardiography or radionuclide angiocardiography and the PEP/LVET ratio in detecting abnormalities of left ventricular function in patients with myotonic muscular dystrophy and no clinical signs of heart disease.
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PMID:[Preclinical changes in left ventricular function in myotonic muscular dystrophy. Value of the isovolumetric index]. 652

Patients with progressive muscular dystrophy rarely survive beyond the second or third decades: the commonest causes of death are respiratory failure and cardiac failure. An easily repeatable method for early recognition of cardiac dysfunction and its follow-up would therefore be valuable. We performed a polycardiographic study of systolic time intervals in 11 patients with dystrophy (each subject representing an age from 7 to 17 years) in a group comprising 10 of their mothers and in control groups matched for age and sex. The mean values of QS2, PEP and QS1 were significantly higher in patients compared to controls (QS2 : 543 +/- 26 msec compared to 519 +/- 19 msec; PEP : 142 +/- 21 msec compared to 123 +/- 18 msec, and QS1 : 105 +/- 22 msec compared to 75 +/- 12 msec) whilst mean ICT was shorter (36 +/- 11 msec, compared to 49 +/- 5 msec). Mean LVET was comparable in patients and control. These results indicate a reduction in compliance and an increase in end diastolic left ventricular tension. When the systolic time intervals were analysed in each child, an increase in PEP and reduction in LVET were characteristic of the more serious phases of the disease with cardiac failure. It is emphasized that early stages of the disease are characterised by restrictive changes resulting in a reduction in ICT whilst terminal stages with reduced cardiac output cause a reduction in LVET and an increase in PEP. No significant changes were found in the mothers of patients compared to the control group. These results indicate that systolic time intervals may be useful in following up patients with Duchenne's cardiomyopathy.
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PMID:[Evaluation of systolic intervals in Duchenne cardiomyopathy]. 681 20

The purposes of this study were to: (1) evaluate the progression of cardiac involvement in Duchenne's muscular dystrophy using systolic time intervals (PEP/LVET); (2) determine if the degree of cardiac involvement bears a relation to the severity of skeletal muscle disease; and (3) describe the M-mode and two-dimensional echocardiographic findings. In 1970, systolic time intervals were studied in 16 patients. During the 10-year interim, two patients were lost to follow-up study, and five patients died. Nine remaining patients were re-studied in 1980 with M-mode and two-dimensional echocardiography as well as systolic time intervals. The PEP/LVET value of these nine patients increased from 0.37 +/- 0.05 (mean +/- SD) in 1970 to 0.47 +/- 0.07 (p less than 0.005) in 1980. Three patients remained ambulatory, and their PEP/LVET value (0.41 +/- 0.04) was significantly better than that of the nonambulatory patients (0.50 +/- 0.07, p less than 0.05). The M-mode echocardiography percentage diameter change was also worse in the nonambulatory group (21 +/- 4 percent versus 34 +/- 7 percent, p less than 0.02). The five patients who were nonambulatory in 1970 died in the intervening 10 years. This study demonstrated that the heart disease of Duchenne's muscular dystrophy is progressive and that the severity of skeletal muscle disease is probably associated with the degree of cardiac dysfunction.
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PMID:Cardiac function in Duchenne's muscular dystrophy. Results of 10-year follow-up study and noninvasive tests. 711 81

Routine echocardiographic examinations were performed on 53 individuals with muscular dystrophy (29 with myotonic muscular dystrophy [MMD] and 24 with Duchenne's dystrophy [DD]). Five of 29 (17%) with MMD and six of 24 (25%) with DD had typical mitral valve prolapse evidenced by echocardiography. All patients with DD and mitral valve prolapse had severe thoracic skeletal deformities, while none with MMD and mitral valve prolapse had such abnormalities. Other echocardiographic parameters analyzed included the fractional shortening of the left ventricle (% delta S), excursions of the left ventricular posterior wall (LVPW) and the ventricular septum, pre-ejection period/left ventricular ejection time ratios (PEP/LVET), and thickness of the LVPW and ventricular septum. Abnormal prolongation of the PEP/LVET ratio and increased thicknesses of the LVPW and ventricular septum were noted in some individuals in the MMD group. Decreased % delta S with diminished excursion and diastolic velocity of the LVPW were noted in some with DD.
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PMID:Echocardiographic evaluation of cardiac abnormalities in Duchenne's dystrophy and myotonic muscular dystrophy. 738 44