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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine patients with the Marfan syndrome and 40 of their first degree relatives were evaluated for the presence of cardiac, skeletal and ophthalmologic abnormalities. Aortic root dilatation and mitral valve prolapse were sought by echocardiography, and the metacarpal index was calculated from hand X-rays. Abnormalities of all the tests performed were present in all nine index cases, except for one normal eye exam. Mitral prolapse was present in thirteen relatives (33%) and aortic root dilatation in seven (18%). At least one cardiac abnormality was present in nineteen (47%) relatives. Aortic root dilatation was more common in male relatives; the incidence of mitral prolapse was approximately equal in the two sexes. Abnormal metacarpal index (greater than 8.0) occurred in fifteen of twenty-six relatives examined (58%). Ophthalmologic abnormalities were found in only four relatives. Two relatives had abnormalities of all three organ systems evaluated, five others had abnormalities of two systems, and fourteen had abnormalities of one system. We conclude that cardiac and skeletal abnormalities are demonstrable in a high percentage of first degree relatives of patients with the Marfan syndrome.
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PMID:Cardiac, skeletal and ophthalmologic abnormalities in relatives of patients with the Marfan syndrome. 13 15

Clinical and echo-phonomechanocardiographic reports. The results concerning 7 subjects with mitral valve prolapse (M.V.P.), all members of the same familiar group, are reported. The anamnestic investigation for cardio-vascular deseases, Marfan's syndrome or other abnormalities referred to mesenchimopathies has been negative. No subject with M.V.P. has referred subjective cardio-vascular symptoms nor radiological, ecgraphic and echo-phonomechanocardiographic signs of reduced cardiac function, have been pointed out. The phonomechanocardiographic tracings have shown a variable and low-amplitude click. The echocardiographic tracings have shown a U-shaped (3 cases) and double U-shaped (1 case) pansystolic, early systolic (2 cases) and of doubtful classification (1 case) prolapse. The hypothesis of an autosomal dominant form of inheritance of the desease with not yet clear mechanism of genetic transmission is confirmed. Furthermore, environmental and genetic factors that interfere with the orderly valvular formation at a certain stage of the fetal development, are referred. The Authors remark the necessity of further investigations, among more familiar groups, for identifying the genetic-environmental factors, that, eventually, could have any implication in the ethiopathogenetic mechanism of the disease. The report of "silent" forms and the not yet completely known evolution of the M.V.P. don't justify its not-recognition.
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PMID:[Mitral valve prolapse in a family group. Clinico-instrumental findings]. 59 36

Echocardiograms from 562 patients were examined for evidence of the pattern of tricuspid valve prolapse. Criteria for the diagnosis can be established similar to those applicable to mitral prolapse. In 500 consecutive patients without mitral valve prolapse, there were no cases of isolated tricuspid valve prolapse. Eleven of 53 (21%) patients with mitral valve prolapse also had tricuspid valve prolapse. Four of six (67%) patients with Marfan's syndrome and mitral valve prolapse also had tricuspid valve prolapse. The occurrence of this echocardiographic pattern as an isolated finding as well as associated with mitral valve prolapse was significantly less than previous angiographic reports. Patients with both these findings tended to be older than those with mitral valve prolapse alone, but clinically differed in no other way. Use of standardized technique can minimize errors in diagnosis.
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PMID:Occurrence and significance of echocardiographically demonstrated tricuspid valve prolapse. 67 79

For half a century the systolic click and late systolic murmur lay dormant as innocent auscultatory curiosities. The thirteen years since Barlow related these phenomena to mitral leaflet prolapse have witnessed an astonishing information explosion. We have sought to bring together the accumulated data in this review. An Historical Perspective traces the evolution from the now abandoned "pericardial" or "extracardiac" phases, through the leafletchordal phase (redundancy), the myocardial phase (segmental left ventricular contraction abnormalities), to the anular phase (dilatation and faulty systolic contraction). Functional Anatomy is dealt with in terms of pathology, pathophysiology, hemodynamics, angiocardiography, echocardiography, and physical and pharmacological interventions. Clinical Manifestations are concerned with prevalence, natural history, symptoms, physical signs, electrocardiographic abnormalities and roentgen fingings. The four Major Complications- sudden death, infective endocarditis, spontaneous rupture of chordae tendineae, and progressive mitral regurgitation- are examined. Associated Cardiac Diseases, i.e., Marfan's syndrome, ostium secundum atrial septal defect and atherosclerotic coronary artery disease, are discussed, and a section on Treatment deals chiefly with prophylaxis for infective endocarditis and the management of arrhythmias and chest pain. A final section on Evolving Information considers etiologic concepts, the nature of left ventricular contration abnormalities, the cause of chest pain, the relationship to Marfan's syndrome and ostium secundum atrial septal defect, and the effect of aging and sex differences on leaflet chordal redundancy.
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PMID:Mitral valve prolapse. 77 40

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

Echocardiographic studies of 11 patients with Marfan's syndrome showed that in all adult patients aortic root diameters were larger than 35 mm (normal range 24 to 35, mean 30+/-2 mm). Prolapse of the mitral valve was present in seven of 11 patients. There was no relationship between the degree of aortic root enlargement and the presence of prolapse. However, in five of seven patients with prolapse there were premature ventricular contractions recorded during the echocardiogram or on a separate electrocardiogram. In four patients without prolapse, no premature atrial or ventricular contractions were seen.
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PMID:Echocardiographic findings in Marfan's syndrome. 84 81

The cross-sectional echocardiographic features of mitral valve prolapse were defined in 26 children (ages 2-18 years) using a real-time, multiple-crystal ultrasound scanner. In each patient the physical findings of the mitral valve click-murmur syndrome were present and mitral valve prolapse had been diagnosed previously by conventional single crystal echocardiography. Mitral prolapse occurred in a familial setting in eight patients and was associated with the Marfan syndrome in five. Real-time two-dimensional echocardiography uniformly disclosed maximum mitral arching and the superior-posterior prolapse. These visual observations were confirmed by M-mode recordings derived from single elements within the array of 20 crystals. The method allowed a complete M-mode description of the phasic motion of the entire mitral apparatus and observations of the spectrum of prolapse from discrete late systolic prolapse to "hammock-like" holosystolic prolapse. Further, the recording of multiple systolic M-mode lines occurred when the ultrasound beam intersected the arched leaflets more than once. Pseudosystolic anterior motion was observed often and resulted clearly from a superimposition of echoes from the mitral annulus and from the posterior-superiorly arched prolapsed leaflets. A major finding in 22 patients was the association with prolapse of biconvex enlargement of the aortic sinuses of Valsalva and a significant increase in the diameter of the aortic root. Aortic root dilatation was most marked in, but not confined to, patients with the Marfan syndrome and was a prominent finding in six patients with minor musculoskeletal abnormalities. The presence of aortic root dilatation in children with normal body habitus raises important questions concerning the generalized nature of an abnormality of cardiac connective tissue in patients with mitral prolapse. The cross-sectional approach significantly enhances the noninvasive evaluation of mitral valve prolapse and provides an explanation for many of the single crystal observations reported previously.
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PMID:Mitral valve prolapse in children: a problem defined by real-time cross-sectional echocardiography. 94 87

Straited membranous structures (SMS), which consisted of sheets or ribbons of 130 to 220 A in thickness, showed variable patterns of periodic substructure, and resembled SMS described in renal and ocular tissues in various diseases, were found in extracellular locations in a) mitral valve (2 patients) and tricuspid valve (1 patient) of 2 patients with mitral valvular prolapse, b) mitral valve and femoral artery of 1 patient with Marfan's syndrome and prolapsed mitral valve, and c) myocardium (2 patients) and thickened endocardium (3 patients) of 3 patients with congenital heart disease associated with muscular obstruction to right ventricular outflow. Striated membranous structures measured up to several microns in diameter, often were highly folded and convoluted, and sometimes appeared circular in outline. Some SMS measured from 130 to 150 A in thickness and had indistinct edges and poorly defined periodicity. The majority of SMS, however, had greater thicknesses, in the range of 200 A, and a periodicity characterized by alternating light and dark bands with a spacing that varied from 100 to 160 A. The structures were associated with thickened basement membranes, elastic fibers, and membrane-bound bodies of the type thought to be involved in elastogenesis. Evidence available suggests that SMS results from an unusual pattern of arrangement of a component, possibly Type IV collagen, of basement membrane material.
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PMID:Striated membranous structures in human hearts. An ultrastructural study. 97 Apr 44

A patient with Marfan's syndrome and subacute bacterial endocarditis is presented. Echocardiographic studies demonstrated dilatation of the aortic root, prolapse of the posterior leaflet of the mitral valve, and the appearance of shaggy echoes on the anterior miltral leaflet, of the kind previously described as representing bacterial vegetations.
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PMID:Bacterial endocarditis in a patient with Marfan's syndrome. 100 Oct 61

Idiopathic prolapse of the mitral valve is a common disorder, but many cases are clinically subtle. Thoracic skeletal abnormalities, reported recently to accompany the syndrome, may serve as an easily identifiable clinical indicator. The prevalence of these abnormalities was defined in 24 patients with proved prolapse of the mitral valve. The valvular syndrome was defined clinically, by echocardiography and, in seven cases, by left ventricular angiography. The skeletal deformities were defined clinically and radiographically. Pectus excavatum was present in 62 percent of the patients, "straight back" in 17 percent and severe scoliosis in 8 percent. Eighteen of the 24 patients (75 percent) had a definite thoracic skeletal deformity. The association of idiopathic prolapse of the mitral valve with these skeletal deformities may represent a forme fruste of Marfan's syndrome. Patients with "straight back" and pectus excavatum should be examined clinically and perhaps by echocardiography to exclude idiopathic prolapse of the mitral valve; when murmurs are present, a diagnosis of "pseudoheart disease" should not be made before mitral valve prolapse has been excluded.
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PMID:Thoracic skeletal abnormalities in idiopathic mitral valve prolapse. 114 95


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