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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighty patients with various forms of heart disease were studied with the use of a newly developed ultrasonic system having 20 transducers arranged in a linear array. This system allows visualization of the heart in two dimensions in real time. All 15 patients with the mitral valve prolapse syndrome, 13 patients with mitral stenosis, five patients with pericardial effusion, four patients with atrial septal defect, and one patient with left ventricular dyssynergy were properly recognized with this system. One of five patients with hypertrophic myopathy and one of four patients with congestive myopathy were not recognized with this system. Criteria for the recognition of these system. Criteria for the recognition of these conditions are presented as well as the probable cause for false-positive and false-negative diagnoses in this series. Since only qualitative criteria were used, it was not possible to differentiate patients with coronary artery disease or patients with left ventricular volume overload from patients without cardiac pathology. The accuracy of this new system was judged against the clinical examination, conventional echocardiography, cardiac catheterization, and left ventricular angiography. It is assumed that the criteria for diagnosis developed during this study will be supplemented and the equipment improved in the future; however, the ease of operation of this system and the relative accuracy of diagnosis at this stage of its development are extremely interesting. It presents an excellent opportunity to obtain additional information about the cardiac patient without using invasive procedures and without risk.
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PMID:Diagnostic accuracy of an ultrasonic multiple transducer cardiac imaging system. 12 12

A case is presented of a familial form of apparently primary cardio-myopathy with findings on investigation and histology which were in favour of a generalised subclinical muscular disorder: a raised serum creatinine phosphokinase, persistent carnosinuria on a vegetarian diet, and under the light microscope several features indicative of a myogenic dystrophic condition on deltoid biopsy. From their clinical features, these original cases may be classified somewhere between primary familial heart disease and the cardiac complications of myopathies. The value of the creatinine phosphokinase isoenzymes and of muscle biopsy in situations such as these is discussed.
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PMID:[Familial myopathy with exclusively cardiac clinical expression]. 41 19

Outbreaks of Hepatosis Dietetica (HD), Nutritional Myopathy (NM) and Mulberry Heart Disease (MHD) in Western Australia are described. Hepatic selenium concentrations were low in pigs with HD and NM, but not with MHD. The mean hepatic selenium concentration in pigs unaffected with these conditions was 1.07 +/- 0.12 ppm dry wt. Cereal grain in an area of Western Australia where HD and NM frequently occur was found to be low in selenium and the addition of 0.1 ppm selenium to pig rations appeared to be an effective prophylactic measure. The extensive use of lupin seed as a protein source replacing much of the meat meal in pig rations may have contributed to an increase in the incidence of HD and NM.
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PMID:Hepatosis dietetica, nutritional myopathy, mulberry heart disease and associated hepatic selenium level in pigs. 53 87

A syndrome of slowly progressive muscle weakness with scapulo-ilio-peroneal distribution and cardiopathy was identified in 26 members of two families. Inheritance was autosomal dominant. Onset of the disease was between 17 and 42 years. Cardiopathy did not antedate skeletal muscle disease and patients had no symptoms of cardiopathy until a late phase of the disease. Initial ECG changes were non-specific, disturbances of conduction and impulse formation developed subsequently. Skeletal muscle biopsies showed neurogenic and myopathic changes with inflammatory cell reaction and perivascular cuffing. The combination of myopathy with neurogenic-like changes is characteristic of many cases of SPA. The inflammatory cell reaction is considered as part of a secondary polymyositis which is at leart partly responsible for muscle pathology.
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PMID:Inflammatory myopathy in scapulo-ilio-peroneal atrophy with cardiopathy. A study of two families. 121 74

McLeod syndrome was originally described on the basis of a specific blood group phenotype with weak expression of Kell antigens. This erythrocyte abnormality also causes acanthocytosis. The haematological findings are associated with abnormalities in other organ systems, including neuromuscular manifestations. A 51-year-old patient was followed up for 11 years. He presented with persistent muscle creatine kinase elevation and progressive heart disease and later developed a slowly progressive neuropathy and choreic movements. His younger brother presented with grand mal seizures, involuntary movements and high muscle creatine kinase when aged 43 years. Clinical myopathy was absent in both, yet muscle biopsy showed mild myopathic changes. The presence of a motor axonopathy was supported by electrophysiological findings. One brother also showed sensory axonopathy. The movement disorder suggested accompanying basal ganglia dysfunction. Earlier reports of McLeod syndrome are reviewed with respect to neuromuscular involvement. Absence of the Kx membrane protein seems to be the cause of this multi-system disorder.
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PMID:McLeod syndrome: a distinct form of neuroacanthocytosis. Report of two cases and literature review with emphasis on neuromuscular manifestations. 151 5

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

Five generations of an Italian family with an autosomal dominant restrictive cardiomyopathy are described. Members of four generations were examined. Symptoms usually developed in the third or fourth decade but the disease did occur in childhood. Initially the condition was characterised by normal ventricular size and systolic function with increased diastolic filling pressures in both ventricles and consequent bi-atrial enlargement. Cardiac catheterisation showed a left ventricular filling pattern of "dip and plateau". The electrocardiogram typically showed non-specific changes in the ST segment and T wave and changes indicating considerable atrial enlargement, which were confirmed by echocardiography. Light microscopy of two endocardial biopsy specimens showed no specific features but excluded the endomyocardial fibrosis of eosinophilic heart disease, amyloid, and specific heart muscle diseases. At necropsy in one case examined under light microscopy extensive patchy fibrosis was found throughout the endocardium, myocardium, and subepicardium, but there were no features typical of eosinophilic heart disease. Histopathological and biochemical examination of skeletal muscle identified no abnormality. The disease often had an insidious course over five to ten years after presentation. Bundle branch blocks, leading to complete atrioventricular block, however, often occurred and may be the first manifestation. Some individuals who survived into the fifth decade developed a progressive, non-wasting skeletal myopathy.
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PMID:Familial restrictive cardiomyopathy with atrioventricular block and skeletal myopathy. 231 4

Anesthesia of patients suffering from one of the various types of myopathy and with a view to orthopedic operation, cannot be univocal. Operating indications, preparation, results, vary according to the type of suffering. The absence of associated cardiopathy allows surgery even with severe respiratory failures. The experiment of an orthopedic surgery department over a 10 years period shows that these patients may be effectively taken charge of by a multidisciplinary group, thus restricting to a maximum their hospital stay for the benefit of at home hospitalization techniques.
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PMID:[Anesthesia in neuromuscular diseases. Experience in orthopedic and spinal surgery]. 236 82

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Poland's syndrome, 3; Marfan's syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical repair of pectus excavatum. 320 60

Four patients with Multicore Myopathy, a rare morphologically distinct myopathy, are described. Although previously considered to be a non-progressive or only slowly progressive myopathy, progression to significant disability was seen in three of our cases. The association of cardiac disease with Multicore Myopathy has not been previously emphasised. All four patients in this study had a cardiomyopathy, and heart disease was the cause of death in two of the patients. Multicore Myopathy is not always a benign entity. Cardiac involvement, when present, adversely affects prognosis.
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PMID:Multicore myopathy: not always a benign entity. 334 55


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