Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four hundred and ninety-nine surgically excised valves were examined for pathological evidence of myxomatous degeneration. Thirty-six valves (7%) had myxomatous degeneration as a major pathological finding. Fourteen valves (3%) had significant myxomatous degeneration of the pars fibrosa, a finding which we define as "primary myxomatous degeneration". Echocardiographic findings and catheterisation results were correlated with the clinical course, surgical results, and follow-up in these 14 patients. Echocardiograms in 10 of the 11 patients who had them (91%) showed abnormalities suggesting the presence of primary myxomatous degeneration. Echocardiography was more helpful than angiography in diagnosis. The histological pattern of primary mitral myxomatous degeneration appears to be identical to that seen in patients with mitral valve prolapse and five of six patients with mitral lesions had echocardiographic evidence of prolapse. None of the patients with primary myxomatous degeneration of the aortic valve had syphilis of Marfan's syndrome. While the aetiology of primary myxomatous degeneration of cardiac valves is not known, a link to a more generalised disorder is suggested.
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PMID:Primary myxomatous degeneration of cardiac valves. Clinical, pathological, haemodynamic, and echocardiographic profile. 745 45

During the past 2.5 years, 50 Ross operations have been performed at Rigshospitalet in Copenhagen in a broad range of patients with aortic valve disease including children and adults from 6 weeks to 71 years of age. Many patients had complicating conditions including endocarditis (n = 13, eight native, five prosthetic valve), prosthetic valve dysfunction (n = 4), subvalvular obstruction (n = 3) treated by septal myectomy (n = 1) or modified Konno operation (n = 2), ascending aortic aneurysm (n = 2), ventricular septum defect (n = 1), mitral valve disease (n = 6), rheumatic heart disease (n = 4), coronary artery disease (n = 1), and extreme obesity (n = 1). All operations were performed as free-standing total aortic root replacements. The results have been encouraging with low mortality (2%) and no major morbidity. One patient has been reoperated because of autograft insufficiency due to left coronary cusp prolapse and two additional patients have grade 2 autograft insufficiency and are being followed closely. Two patients have developed early pulmonary homograft stenosis, which has required pulmonary homograft replacement. Despite these problems, we are enthusiastic about this operation and believe it may emerge as operation of choice for most patients under 60-65 years of age with aortic valve disease and for patients with prosthetic or advanced native aortic valve endocarditis. With increasing frequency, our choice has been to proceed with a Ross operation, and currently, our only absolute contraindication is Marfan's syndrome. Based on reported recurrent disease in patients with rheumatic valve disease, the autograft should be used with caution for this indication.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Expanding indications for the Ross operation. 758 41

The Prader-Willi syndrome (PWS) with Angelman's syndrome form a pair known above all due to problems of genetic imprinting and uniparental disomy. Both phenomena drew attention to the importance of control of expression of different alleles and their genetic origin. The causes of the two syndromes have not been elucidated unequivocally so far. In case of the PWS, at least, there is the possibility of a gene of the protein carrier of a small nuclear ribonucleic acid described as SNRPN. In case of Marfan's syndrome the responsible gene is the fibrillin gene (FNB1) with the locus on area 15q21. The mentioned gene participates probably also in diseases caused by a change of the vascular wall (aneurysm) and in prolapse of the mitral valves. On the 15th chromosome are several representatives of the family of genes of cytochrome P450 the products of which play a part in the metabolism of exogenous substances, incl. pharmaceutical ones. Their activity is part of the natural sensitivity or resistance to some chemical cancerogens. The postscriptis devoted to the assumed locus of dyslexia DLX1.
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PMID:[The human genome--chromosome 15]. 758 67

The many changes in classification of cardiovascular disease during the twentieth century reflect changing etiology of diseases, clinical comprehension and technological advances. In particular, the etiology of valvular heart disease has changed dramatically in the last five decades. The significant reduction of acute rheumatic fever and its sequelae, and the recognition of non-rheumatic causes of valvular disease are responsible for the metamorphosis in the etiology of valvular disorders. Valvular heart disease can be classified as follows: 1) Heritable-congenital causes of valvular heart disease e.g., floppy mitral valve with mitral valve prolapse, bicuspid aortic valve, and the Marfan syndrome; 2) Inflammatory-immunologic causes such as rheumatic fever, acquired immune deficiency syndrome, endocardial proliferative disorders, and antiphospolipid syndrome; 3) Myocardial dysfunction-ischemic cardiomyopathy, dilated or hypertrophic cardiomyopathy-resulting in valvular heart disease; 4) Diseases and disorders of other organs as causes of valvular heart disease, e.g., chronic renal failure and carcinoid heart disease; 5) Valvular heart disease related to aging: calcific aortic stenosis and mitral annular calcification; 6) Valvular disease following interventions such as valvuloplasty, valve reconstructive surgery and valve replacement; and 7) Valvular disease related to drugs and physical agents, such as chronic ergotamine use, radiation therapy and trauma. In clinical practice the most common causes of mitral regurgitation are floppy mitral valve with mitral valve prolapse, ischemic heart disease, dilated cardiomyopathy and mitral annular calcification, while the most common cause of mitral stenosis is rheumatic fever. The most common causes of isolated aortic regurgitation are bicuspid aortic valve and floppy aortic valve, while the most common causes of isolated aortic stenosis are related to the bicuspid aortic valve and the development of calcific senile aortic stenosis. The most common causes of tricuspid regurgitation are dilated cardiomyopathy, ischemic cardiomyopathy, floppy tricuspid valve with tricuspid valve prolapse and infectious endocarditis. Combined mitral and tricuspid regurgitation occur with heritable connective tissue disorders, dilated or ischemic cardiomyopathy, while the most common cause of mitral stenosis plus aortic regurgitation is rheumatic fever. Statistics obtained from cardiac surgery and necropsy may underestimate the true incidence of certain valvular diseases by selection bias. This is particularly so with valvular disease associated with significant ventricular dysfunction, or in the elderly who may not be surgical candidates, or in cases where the valvular disease is not severe enough to require surgical intervention. Recent advances in hemodynamic and imaging technology allow clinicians to define valvular structure and function and to accurately classify valvular heart disease in clinical practice.
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PMID:Valvular heart disease: the influence of changing etiology on nosology. 800 Jun 16

A 56-year-old female had pure regurgitation in all cardiac valves. Color Doppler echocardiography showed a regurgitant jet in all cardiac valves. The severity of regurgitation due to the prolapse in all valves was moderate. The patient had no history of rheumatic fever, ischemic heart disease, endocarditis or hypertension. Physical characteristics of the patient were neither of Marfan's nor Ehlers-Danlos' syndrome. The etiology of regurgitation in all cardiac valves of this patient may be due to multiple valve prolapse.
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PMID:An adult case with multiple cardiac valve prolapse and regurgitation. 832 22

The purpose of this study was to demonstrate the special features of cardiovascular effects in connective tissue disorders through a group of fifteen cases observed over a period of ten years. The group consisted of eleven cases of Marfan syndrome (or Marfan-like syndrome), two cases of pseudoxanthoma elasticum and two cases of Ehlers-Danlos disease. The cardiovascular lesions were as follows: 1) dissection of the ascending aorta which was confirmed and had been treated surgically in 2 cases; 2) aneurysmal dilatation of the ascending aorta in 5 cases; 3) moderate mitral valve prolapse, which was isolated in 2 other cases; 4) distention of the mitral valve in 2 cases; 5) mitral valve prolapse combined with tricuspid valve prolapse in 1 case; the mitral incompetence was severe and made it necessary to carry out mitral valve replacement; 6) moderate aortic valve prolapse combined with tricuspid prolapse in a case of type-I Ehlers-Danlos disease; 7) Fallot's tetralogy combined with Marfan's syndrome and treated surgically in one case; 8) severe hypertension with abnormalities of the iliac and renal arteries in one case of elastic pseudoxanthoma elasticum. In three cases complications occurred leading to death, extension of the dissection of the abdominal aorta and global recalcitrant heart failure respectively. The outcome in the other 12 cases, with a mean follow-up time of 3 and one half years (range: 3 years to 7 years) was not marked by complications.
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PMID:[Cardiovascular manifestations of hereditary dysplasias of connective tissue]. 851 93

We reported six patients with Marfan's syndrome, studied retrospectively from May 1990 to April 1992 in the department of Cardiology in Dakar. Morphological, cardiovascular, skeletal and ocular abnormalities have been reviewed. All the patients had been evaluated by echocardiography. Prevalence of Marfan's syndrome among congenital heart diseases during this period was 4.8%. The mean age was 27.6 years. The mean height was 1.80 m (range 1.38-2.02 m) for a mean weight of 62.8 kg. All the patients had dolichostenomely and arachnodactyly. Kyphosis or scoliosis was present in 5 cases. Chest deformities (pectus carinatum and excavatum) were present in 5 cases. 5 patients had hyperextensible joints. 5 patients had ocular abnormalities. Cardiac pathology was found in 5: mitral prolapse with insufficiency in 2; mitral prolapse with aortic dystrophy in 2; and isolated dilatation of ascending aorta in an other. One patient with diffuse Marfan's syndrome died of cardiac failure. Our study confirm polymorphic manifestations of Marfan's syndrome and the frequency of cardiac abnormalities which are the major determinants of life-prognosis in these patients. Echocardiography is very useful as a noninvasive method for defining the extent of cardiovascular involvement and following its course, for more appropriate treatment.
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PMID:[Cardiovascular manifestations of Marfan's syndrome apropos of 6 cases]. 865 85

A diffuse mitral valve prolapse was successfully repaired in 2 patients using polytetrafluoroethylene sutures as artificial chordae. In a 16-year-old boy with Marfan's syndrome and in a 56-year-old woman, a total of 11 and ten pairs of polytetrafluoroethylene sutures were used, respectively, to repair a severe mitral valve prolapse. We consider mitral valve repair using polytetrafluoroethylene sutures to be the treatment of choice for a diffuse prolapse of the mitral valve involving both the anterior and posterior leaflets.
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PMID:Extensive use of artificial chordae for repairing diffuse mitral valve prolapse. 878 25

Mitral valve prolapse (MVP) is a commonly diagnosed condition with varied clinical presentations but local data is lacking. In our study, we reviewed 98 patients (54 males, 44 females) with echocardiographic mitral valve prolapse diagnosed between 1991 and 1993 to study the clinical profile and echocardiographic features of patients with this condition in our local population. The mean and median age at presentation/detection were 42 years and 38 years respectively. The majority of the patients were asymptomatic (59%); the rest presented with palpitations (21%), congestive heart failure (4%) and infective endocarditis (5%). On clinical examination, 64 patients had mitral regurgitation (13 patients had both mitral regurgitation murmur and a systolic click), while one or more systolic clicks were heard in another 32 patients. Six patients also had associated Marfan syndrome. 2D echo revealed isolated anterior and posterior leaflet involvement in 55 and 19 patients respectively. Another 24 patients had involvement of both leaflets. Mitral regurgitation was detected on colour Doppler study in 78 patients. Nine patients had associated tricuspid valve prolapse. Of the 98 patients, 8 patients developed flail mitral valve. Four were detected at presentation/diagnosis, while the other 4 were diagnosed incidentally on routine follow-up 2D echo. Of these 8 patients, one developed cardiac failure. The patients had been on follow-up for a mean period of 9 months. During this period, mitral regurgitation progressed in 3 patients resulting in valve surgery. Only 20 patients had arrhythmias detected on ambulatory ECG monitoring, most of them were frequent atrial and ventricular premature beats. No patient was found to have haemodynamically significant arrhythmia. In summary, most patients with MVP had anterior mitral valve leaflet prolapse and mitral regurgitation. Although most patients with MVP are asymptomatic or have minor symptoms, it is associated with significant morbidity.
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PMID:Clinical and echocardiographic features of mitral valve prolapse patients in a local population. 894 49

The ocular findings in patients with homocystinuria due to cystathionine-beta-synthetase (CBS) deficiency are reviewed and a new approach to lens surgery is presented. Lens surgery in CBS deficiency was reported to be accompanied by a high risk of secondary complications like vitreal prolapse and retinal detachment. So far an operative procedure for these patients that allows a complete preservation of the anterior vitreous cortex, the posterior lens capsule and an implantation of an artificial lens is not established. A minimal invasive method of lens surgery was developed in vitro and applied in the case of a 10-year-old patient with an uncorrectable refractive situation due to progressed lens dislocation. Principle steps of the evolved operative procedure are (1) visco-elastic stabilization of the anterior chamber; (2) small (1.5 mm) peripheral capsulorhexis; (3) manual endocapsular aspiration of lens material and removal of lens epithelium; (4) ab externo introduction of a transcleral fixation suture; (5) positioning of the haptic into the ciliary sulcus in the inferior position; (6) resection of the anterior lens capsule; and (7) positioning of the second haptic into the ciliary sulcus. The first patient treated according to this method presented a stable position of the pseudophakos during the follow up of 1 year. Visual acuity improved from a level of 20/200 (O.D.) and 10/200 (O.S.) to 18/20 in both eyes. The surgical procedure presented in this report seems to be useful for dealing with dislocated lenses due to zonular deficiency in patients with CBS deficiency and hopefully with Marfan disease. It allows a minimal invasive removal of the lens, a complete preservation of the anterior vitreous cortex and at the same time a stable fixation of an artificial intraocular lens.
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PMID:Ocular complications and a new surgical approach to lens dislocation in homocystinuria due to cystathionine-beta-synthetase deficiency. 958 34


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