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 51,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mitral valve prolapse is a condition that is being recognized with increased frequency. It is not known whether its incidence is increasing, or whether we are better able to diagnose it today. In the idiopathic or familial variety, the mitral valve pathology is almost always that of myxomatous degeneration. Some authors have suggested the presence of a cardiomyopathy because of significant left ventricular dysfunction in many cases. Idiopathic prolapse occurs predominantly in females, often at a young age, and may be associated with chest pain, dyspnea, fatigue, presyncope, syncope, and/or sudden death. The clinical findings are variable and typically consist of a nonejection click and/or late systolic murmur, heard best at the cardiac apex. Diagnosis can be confirmed by echocardiography and/or ventricular cineangiography, the latter permitting accurate recognition of the anatomy of the prolapsed leaflets. The complications of infective endocarditis, severe mitral insufficiency, and life-threatening ventricular arrhythmias represent the major problems of management. It is important to distinguish the idiopathic form of mitral valve prolapse from that due to coronary artery disease and to realize that mitral valve prolapse may occur in Marfan's syndrome, Turner's syndrome, or in association with secundum atrial septal defect or ruptured chordae tendineae. Typical clicks and/or murmurs have also been described in patients with a history of rheumatic fever and in hypertrophic cardiomyopathy. Although much descriptive knowledge has accumulated over the past 15 years, many unanswered questions remain regarding the idiopathic type of prolapse. What is the nature and cause(s) of myxomatous degeneration? What is the relation of the valve pathology to the left ventricular dysfunction? What is the relation of both of these factors to disabling chest pain, electrocardiographic changes, and life-threatening arrhythmias? Hopefully, answers to these and other important questions regarding mitral valve prolapse will be forthcoming.
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PMID:Mitral valve prolapse. 77 95

Mitral valve prolapse (MVP) now is a commonly recognized syndrome with an apparent prevalence of approximately 4-6%. It appears to occur more frequently in females and occasionally it is familial. In most instances, the syndrome is idiopathic, although it occurs in association with many other conditions, particularly Marfan's syndrome, rheumatic heart disease, coronary heart disease, congestive cardiomyopathy, ostium secundum atrial septal defect, Ehlers-Danlos syndrome or abnormalities of the thoracic cage. The majority of patients with the syndrome have minimal, if any, symptoms and have a benign course. When symptoms do occur, more frequently they are palpitations, chest pain, dyspnea on exertion or fatigue. Neuropsychiatric symptoms or even transient ischemic episodes may occur rarely. Very rarely, complications such as severe mitral regurgitation, arrhythmias or infective endocarditis may occur. Characteristically, patients have a midsystolic click, occasionally followed by a systolic murmur. The timing of the click and the onset of the murmur usually is variable, depending on the ventricular volume. The electrocardiogram frequently shows ST-T wave changes. The diagnosis usually can be confirmed by echocardiography or left ventricular angiography. Most patients with MVP require no treatment other than reassurance. If a systolic murmur is present, prophylaxis against infective endocarditis during dental work probably is useful. Patients with palpitations or chest pain usually respond well to treatment with propranolol. Patients with progressive severe mitral regurgitation require mitral valve replacement.
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PMID:Mitral valve prolapse. 699 66

Referrals to pulmonary physicians for polysomnography to evaluate snoring or the possibility of sleep apnea syndrome often evolve into a multidisciplinary clinical problem. We present a young woman with two congenital abnormalities (Marfan's syndrome and retrognathism) which both may affect her decreased exercise tolerance, daytime hypersomnolence, and fatigue. Polysomnography and pulmonary exercise testing documented improvement in her upper airway diameter and exercise capacity. Her improved sleep pattern and her understanding of her exercise performance limitations afforded her a new outlook on life.
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PMID:Pulmonary dysfunction secondary to mandibular retrognathia in Marfan's syndrome. 818 76

Muscle may suffer from a number of diseases or disorders, some being fatal to humans and animals. Their management or treatment depends on correct diagnosis. Although no single method may be used to identify all diseases, recognition depends on the following diagnostic procedures: (1) history and clinical examination, (2) blood biochemistry, (3) electromyography, (4) muscle biopsy, (5) nuclear magnetic resonance, (6) measurement of muscle cross-sectional area, (7) tests of muscle function, (8) provocation tests, and (9) studies on protein turnover. One or all of these procedures may prove helpful in diagnosis, but even then identification of the disorder may not be possible. Nevertheless, each of these procedures can provide useful information. Among the most common diseases in muscle are the muscular dystrophies, in which the newly identified muscle protein dystrophin is either absent or present at less than normal amounts in both Duchenne and Becker's muscular dystrophy. Although the identification of dystrophin represents a major breakthrough, treatment has not progressed to the experimental stage. Other major diseases of muscle include the inflammatory myopathies and neuropathies. Atrophy and hypertrophy of muscle and the relationship of aging, exercise, and fatigue all add to our understanding of the behavior of normal and abnormal muscle. Some other interesting related diseases and disorders of muscle include myasthenia gravis, muscular dysgenesis, and myclonus. Disorders of energy metabolism include those caused by abnormal glycolysis (Von Gierke's, Pompe's, Cori-Forbes, Andersen's, McArdle's, Hers', and Tauri's diseases) and by the acquired diseases of glycolysis (disorders of mitochondrial oxidation). Still other diseases associated with abnormal energy metabolism include lipid-related disorders (carnitine and carnitine palmitoyl-transferase deficiencies) and myotonic syndromes (myotonia congenita, paramyotonia congenita, hypokalemic and hyperkalemic periodic paralysis, and malignant hyperexia). Diseases of the connective tissues discussed include those of nutritional origin (scurvy, lathyrism, starvation, and protein deficiency), the genetic diseases (dermatosparaxis, Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, homocystinuria, alcaptonuria, epidermolysis bullosa, rheumatoid arthritis in humans, polyarthritis in swine, Aleutian disease of mink, and the several types of systemic lupus erythematosus) and the acquired diseases of connective tissues (abnormal calcification, systemic sclerosis, interstitial lung disease, hepatic fibrosis, and carcinomas of the connective tissues). Several of the diseases of connective tissues may prove to be useful models for determining the relationship of collagen to meat tenderness and its other physical properties. Several other promising models for studying the nutrition-related disorders and the quality-related characteristics of meat are also reviewed.
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PMID:Diseases and disorders of muscle. 839 47

A redo-operation for active prosthetic valve endocarditis after modified Bentall operation is reported. A 42-year-old man associated with Marfan syndrome was transferred to our hospital with complaints of high fever and general fatigue. A modified Bentall operation for acute aortic dissection was done five years ago. The aortic arch and descending thoracic aorta were replaced with prosthetic graft because of aneurysmal dilatation four years ago. Transesophagial echogram revealed a developing vegetation below the prosthetic valve and at the left ventricular outflow. A redo-operation of translocation method with Pieler method for coronary reconstruction was performed using a prosthetic valve of SJM 21 mm in size. After operation, hemolysis suddenly appeared and hepatic dysfunction gradually progressed. Reoperation was necessary for the redo-operation and and a SJM 21 mm valve was replaced with a new 25 mm SJM valve interposed between the graft. Hemolysis was immediately improved. A redo operation of translocation method with Pieler method for coronary reconstruction showed a good results.
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PMID:[A case of successful redo-operation for active prosthetic valve endocarditis after modified Bentall operation]. 852 75

A patient with Marfan's syndrome and obstructive sleep hypopnea syndrome is reported. She had complained about insomnia, tiredness and hypersomnolence lasting 2 years. A complete nightly polysomnography revealed 94 obstructive hypopneas (the hypopnea index was 12.85) and no obstructive apneas. Presumably an increased laxity of the pharyngeal wall was responsible for the phenomenon. A therapy with CPAP was started, but was not tolerated by the patient. Oxygen therapy on the other hand turned out to be effective and well tolerated.
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PMID:Obstructive sleep hypopnea syndrome in a patient with Marfan syndrome treated with oxygen therapy. 855 69

We present data from an exploratory study of 174 adults with Marfan syndrome regarding their cognitive perceptions of the condition as postulated by the self-regulatory model (Leventhal H, Benyamini Y, Brownlee S et al. In: Petrie KI, Weinman JA, eds. Perceptions of Health and Illness: Current Research and Applications. Amsterdam, The Netherlands: Harwood Academic, 1997: 19-45; Leventhal H, Nerenz DR, Steele DJ. In: Baum A, Taylor SE, Singer JE, eds. Handbook of Psychology and Health. Hillsdale, NJ: Lawrence Erlbaum Associates, 1984: 219-252). The vast majority of the respondents had adequate general knowledge about Marfan syndrome. Eighty-three percent of the respondents perceived Marfan syndrome as having had significant adverse consequences on their lives. Having striae, pain (sore joints), and depression were each independently correlated with this view. Fifty-eight percent of the respondents indicated that they felt they had low to moderate control over their condition, demonstrating variability. History of aortic dissection, pain (sore joints), and depressive symptoms were each negatively correlated with the view that Marfan syndrome is a curable/controllable condition. Moreover, approximately 28% view the condition as a lethal condition, whereas 67% view it as a serious condition. Forty-four percent of the cohort were found to have significant symptomatology of depression independent of beta- and Ca2+-channel blockade use. Respondents cited both advantages and disadvantages of being affected. Genetic counseling that addresses patients' perceptions of Marfan syndrome, and its associated pain, fatigue, and depressive symptoms, may enhance patient adaptation to the condition.
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PMID:Living with Marfan syndrome I. Perceptions of the condition. 1168 73

We investigated the perceptions of and adherence to medication and physical activity guidelines in 174 adults with Marfan syndrome. Over 80% of those prescribed beta- and Ca2+-channel blockade reportedly adhere well to their medication regimen. The presence of cardiovascular symptoms and fatigue were positively correlated with the medication use. Medication-takers reported that they are psychologically receptive to the use of medication for prophylactic treatment of their cardiovascular problems. However, all do not view their medication as essential for their health. Duration of the medication regimen, type of medication, and perception of controllability of the condition were each independently associated with respondents' perceptions of the necessity of taking beta- or Ca2+-channel blockers. Over 80% of the respondents reported that they choose their physical activities with their diagnosis in mind. Modifying exercise activities was significantly correlated with an increased perception of Marfan syndrome as having negative consequences on the respondents' lives. Genetic counseling should address beliefs about medication use and physical activity restrictions, as perceptions of these health behaviors may have significant impact on how adults with Marfan syndrome adhere to these recommendations and cope with their condition.
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PMID:Living with Marfan syndrome II. Medication adherence and physical activity modification. 1168 74

A 10-year-old boy with a medical history of fatigue became nauseous, short of breath and cyanotic within 24 hours after a frightening incident. He was successfully resuscitated after a cardiac arrest. A CT scan revealed a ruptured aneurysm of the ascending aorta. During emergency surgery the ascending aorta and aortic arch were replaced with a 22 mm synthetic graft. No postoperative complications occurred. There was no associated trauma, syphilis, collagen diseases (Marfan's syndrome, Ehlers-Danlos syndrome), congenital heart disease or autoimmune disease. The cause of the aneurysm and rupture remain unclear.
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PMID:[Rupture of thoracic aneurysm in a 10-year old boy]. 1177 Feb 68

The knowledge pertaining to mitral valve prolapse is mainly based on studies in adults. In this study, the clinical profile as described in adults was compared with that found in children up to the age of 13 years. Forty-five children with echocardiographic-proven mitral valve prolapse and who met the inclusion criteria were included in the study. The male:female ratio in this study was 1:1.37 and was not statistically significantly different from reported ratios. Most of the children were asymptomatic. Twenty-one of the 31 patients referred from outside the hospital had an incidentally found murmur. The symptoms found in this study were not similar to those described in adults. The most commonly found symptoms were shortness of breath and fatigue, in contrast to those of chest pain and palpitations described in adults. Comparing males to females in this study, significantly lower weight (p = 0.005) and body mass index (p = 0.003) were found in girls, and a significantly lower pulse rate (p = 0.002) in boys. Left-sided cardiac enlargement was diagnosed in 11 patients on chest X-ray and in six patients on electrocardiogram. One patient had Marfan syndrome and four others had a Marfanoid appearance. In conclusion, most children with mitral valve prolapse are asymptomatic. Mitral valve prolapse is not an uncommon finding in children younger than 13 years of age. Patients with mitral valve regurgitation were advised to take infective endocarditis prophylaxis prior to invasive procedures.
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PMID:Mitral valve prolapse: a study of 45 children. 1453 60


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