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

An attempt was made to investigate the incidence and significance of high frequency notches and slurs on the QRS complex in patients with progressive muscular dystrophy of the Duchenne type (PMD). The patients were classified into eight stages from the most mild, S(1), to the most severe, S(8), according to Swinyard-Deaver's criteria. Cases where the sum of high frequency notches in the combined leads exceeded none were generally limited to those more advanced than stage S(4). Also, the sum of the notch count tended to be higher in S(5) to S(6) than in the remaining groups and to be lower in the milder cases, S(1) to S(4), and most severe cases, S(7) and S(8). It should be emphasized that cases in groups S(7) and S(8) who had a history of congestive heart failure and/or developed congestive heart failure during the observation period, tended to show a smaller number of notches. High frequency slurs showed almost the same tendencies as the high frequency notches. It is thus anticipated that a significant increase or decrease in the number of notches and slurs on the QRS complex may be suggestive of more intense myocardial derangement.
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PMID:Orthogonal electrocardiographic study on progressive muscular dystrophy of the Duchenne type. 45 86

We measured plasma levels of atrial natriuretic peptide (ANP) in 9 patients of Duchenne muscular dystrophy (DMD) and 3 patients of Becker muscular dystrophy with congestive heart failure (CHF). Administration of digitalis, catecholamine and angiotensin converting enzyme inhibitor resulted in decrease of ANP levels as well as improvement of clinical symptoms of CHF and cardiomegaly. Four DMD patients whose ANP levels were more than 200 pg/ml after the treatment of CHF showed poor prognosis. These results suggest that ANP is a useful marker for the treatment of CHF in progressive muscular dystrophy.
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PMID:[Alteration of atrial natriuretic peptide in progressive muscular dystrophy with congestive heart failure]. 142 36

Becker muscular dystrophy is associated with abnormal cardiac features in about 75% of cases; up to one-third will develop ventricular dilatation leading to congestive cardiac failure. As this form of muscular dystrophy is relatively benign, failure to respond to medical treatment warrants assessment for cardiac transplantation.
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PMID:Cardiac transplantation in Becker muscular dystrophy. 148 41

Sleep-disordered breathing may occur in a wide variety of neuromuscular syndromes, and may present with diverse, often isolated, symptoms or findings such as excessive daytime sleepiness, pulmonary hypertension, congestive heart failure, morning headaches, or hypoxia-induced nocturnal seizures. The authors report two sisters with congenital muscular dystrophy in whom central sleep apnoea resulted in the isolated symptom of nocturnal seizures in one, and morning headaches in the other. Review of the literature reveals that sleep-disordered breathing may be common in neuromuscular disorders, and may often be present when clinical weakness is mild, and insufficient to result in diurnal respiratory dysfunction.
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PMID:Central sleep apnoea in congenital muscular dystrophy. 194 Sep 43

This is a clinical report of a rare case of Charcot-Marie-Tooth disease associated with dilated cardiomyopathy. A seventy-seven-year-old Japanese male first visited our outpatient clinic with a ten-year history of muscular weakness in his bilateral lower extremities and gait disturbance characterized by classical features of peroneal muscular atrophy and inverted champagne bottle legs. Biopsy findings of the m. quadriceps femoris and the n. gastrocnemius revealed clustered atrophy of myofibrils and segmental demyelinization mingled with remyelinization. Because of his other problem of dilated cardiomyopathy, he had been treated with salt restriction, digitalis, diuretics and vasodilators, until his third hospitalization, when he developed terminal stage of severe congestive heart failure. Despite our intensive cardiac care, the patient died because of profound pump failure. Autopsy findings disclosed a remarkably dilated left ventricular chamber and an increased total heart weight of 600 grams. Grossly, the cross sectional view of the left ventricle revealed diffuse, but not homogenous fibrosis that was most prominent in the posterior wall. On light microscopic examination, the left ventricular myocardium revealed diffusely scattered muscular degeneration interlaced with fibrosis. Although large epicardial coronary arteries revealed only mild intimal atheromatous thickening, most of the small intramuscular coronary arteries were free from atherosclerosis. Neither diabetic nor amyloid lesions could be detected. It has been well known that cardiomyopathy is often associated with various forms of muscular dystrophy and Friedreich's ataxia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Charcot-Marie-Tooth disease associated with dilated cardiomyopathy: an autopsy case report]. 204 12

The plasma concentration of atrial natriuretic peptide was measured in patients with muscular dystrophies to study its relationship with congestive heart failure. In patients with Duchenne muscular dystrophy, the plasma atrial natriuretic peptide concentration was 35.5 +/- 3.3 pg/mL (mean +/- SE), which was higher than that in age-matched normal subjects (9.8 +/- 0.6 pg/mL). It increased with progression of disability and showed significant correlations with the cardiothoracic ratio and the ratio of the preejection period to the left ventricular ejection time. In patients with other types of muscular dystrophy, the plasma atrial natriuretic peptide concentration showed no significant change. Immunohistochemical examination demonstrated many atrial natriuretic peptide-positive cells in atrial muscle of an autopsied patient, indicating preservation of the peptide until the end stage. These findings suggest that measurement of the plasma atrial natriuretic peptide concentration is useful for evaluating heart failure in patients with Duchenne muscular dystrophy.
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PMID:Secretion and clinical significance of atrial natriuretic peptide in patients with muscular dystrophy. 214 80

To study the relationship between plasma alpha-atrial natriuretic peptide (alpha-ANP) and cardiac function in patients with Duchenne's progressive muscular dystrophy (DMD), we measured plasma alpha-ANP levels in 51 patients with DMD and evaluated them according to the clinical severity of the disease and the echocardiographic left ventricular function. In many of the patients with DMD, plasma alpha-ANP levels were mildly elevated, but no clear correlation was observed between alpha-ANP levels and wall motion abnormalities of the left ventricle. The alpha-ANP level was moderately or markedly elevated (81 to 120 pg/ml) in 3 of the 16 patients with respiratory failure and extremely high (1208 pg/ml) in the one patient with congestive heart failure. Two patients with respiratory failure and one with congestive heart failure, in whom alpha-ANP levels were elevated, died within 2 months. Elevation of left atrial pressure as a result of left ventricular dysfunction caused by impairment of the myocardium and elevation of right atrial pressure as a result of pulmonary hypertension caused by impairment of the respiratory muscles are considered to be involved in the mechanism of increased plasma alpha-ANP levels in patients with DMD. It can be concluded that a moderate or marked elevation in plasma alpha-ANP levels in patients with terminal DMD is a sign of a poor prognosis and may be a useful index for the management of the disease.
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PMID:Plasma levels of atrial natriuretic peptide in patients with Duchenne's progressive muscular dystrophy. 214 65

A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities. Myopathy and heart disease were not clinically evident in her family, although three relatives had unexplained shortened Achilles tendons without weakness. Tendon contractures may be a partial expression of this myopathic disorder, suggesting an autosomal dominant inheritance with variable penetrance. A muscular dystrophy clinically similar to that of the Emery-Dreifuss (EDMD) type can thus occur in women. Rather than the cardiac arrhythmias typical of EDMD, a dilated cardiomyopathy may occur and present with severe congestive heart failure. This is the first report of cardiac transplantation in such a case.
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PMID:Cardiac transplantation in female Emery-Dreifuss muscular dystrophy. 223 Aug 49

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

Emery-Dreifuss muscular dystrophy is sometimes referred to as benign X-linked muscular dystrophy. We present two young patients with this disorder who had severe cardiovascular complications including: conduction disease, major thromboembolic events, valvular dysfunction with congestive cardiac failure, ventricular tachyarrhythmias, and sudden cardiac death. Of these cardiac problems, only conduction disease has been previously emphasized. This form of muscular dystrophy is not benign--it has malignant cardiac manifestations.
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PMID:Benign X-linked (Emery-Dreifuss) muscular dystrophy is not benign. 244 2


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