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)

We report the cases of eight children with Marfan syndrome. Seven (87.5%) were diagnosed with cardiopathy, everyone with auscultatory findings. Electrocardiographic patterns were nonspecific. Enlargement of the aortic root was present in two of the cases as seen by thorax x-rays. Echocardiography detected the presence of mitral valve prolapse in 87.5% of the patients (7 cases) and aortic enlargement in 75% of the patients (6 cases). Echocardiography also detected the presence of aortic dysplasia, tricuspid valve prolapse and right and left ventricular hypertrophy. During the follow-up period, no case had cardiac failure. There was no mortality. The aortic enlargement was progressive and was not modified by propranolol treatment. Surgical treatment was not needed. Family history related to this condition was present in a very small percentage (37.5%). We comment on one infantile form of Marfan syndrome with its own phenotype different from that of classical Marfan syndrome.
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PMID:[Marfan syndrome in childhood: cardiovascular manifestations. Echocardiographic changes]. 141 16

Clinical, electrocardiographic and echocardiographic data were collected in a group of 228 patients with autosomal dominant polycystic kidney disease (PKD) and in another group of 146 unaffected members (NPKD) both comprised in a five-generation kindred followed for 10 years, in order to determine the profile and prevalence of cardiovascular derangement of the genetic disease. A family of 181 members was used as a control. The prevalence of left ventricular hypertrophy in the three groups was 24, 14 and 6% respectively (p less than 0.01); after 10 years it increased up to 35, 26 and 13% respectively (p less than 0.05). The evidence of mitral-valve prolapse was more frequent in PKD and in NPKD group (25 and 20% respectively) than in control subjects (2%) (p less than 0.0001). Mitral incompetence was found in 30, 18 and 8% of those groups respectively (p less than 0.002). The large difference in mitral involvement did not change over time. Tricuspid valve prolapse was detected in 5, 4 and 1% of the three groups, respectively (p less than 0.05). A small increase in frequency was found after 10 years only in polycystic kidney disease patients. Regurgitant aortic lesions were present in higher prevalence in PKD (19%) and NPKD (17%) members than in controls (5%) (p less than 0.001). After 10 years they were 23, 20 and 8%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The spectrum of cardiovascular abnormalities in autosomal dominant polycystic kidney disease: a 10-year follow-up in a five-generation kindred. 160 75

This report describes the simultaneous occurrence of mitral valve prolapse (MVP) and systolic anterior motion (SAM) in hypertrophic cardiomyopathy (HC). In 25 patients (aged 7 to 62 years, mean 29), 15 (60%) of whom were male, distal portions of the anterior or posterior mitral leaflets approached or made midsystolic contact with the ventricular septum, whereas the proximal portion of the mitral leaflets showed marked cephalad excursion into the left atrium, 5 to 15 mm beyond the mitral annular plane. Three mitral valves that were available for gross visual inspection were not morphologically typical of patients with primary MVP. Clinical features and natural history (1 to 14 years [mean 6] of follow-up), cardiac dimensions, and distribution of left ventricular hypertrophy defined in the study patients did not appear to differ distinctly from those in the overall referral population of patients with HC evaluated at our institution. Hence, patients with HC may show a striking pattern of mitral valvular motion involving SAM into the left ventricular outflow tract, as well as MVP; this prolapse motion is probably due to anatomic disproportion between the mitral valve and the small left ventricular cavity rather than to the coexistence of 2 separate disease entities. Such patients further define the great diversity evident within the broad clinical spectrum of HC.
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PMID:Simultaneous occurrence of mitral valve prolapse and systolic anterior motion in hypertrophic cardiomyopathy. 199 65

A group of 22 adults with Turner's syndrome, mean age 29.6 years, was subjected to a careful examination by one-dimensional, two-dimensional, pulsed and coloured Doppler echocardiography. The purpose was to assess the incidence and character of congenital and acquired abnormalities of the cardiovascular system which occur within the framework of this defined genetic syndrome. A quite normal echocardiographic finding was recorded in 13 patients, i. e. in 59.1%. In the remainder a wide spectrum of abnormalities was found such as prolapse of the mitral valve (in 13.6%), bicuspid aortal valve with a medium regurgitation (4.5%), hypoplasia of the coronary cusp of the aortal valve (4.5%), dilatation of the ascending aorta with a residual significant stenosis at the site after operation of coarctation of the thoracic aorta (4.5%), subaortal defect of the interventricular septum (4.5%) and slight left ventricular hypertrophy in patients with arterial hypertension (9.1%). Echocardiographic examination in Turner's syndrome makes early diagnosis of abnormalities of the cardiovascular system possible, incl. quantification of the haemodynamic impact. Some of these pathological changes (bicuspid aortal valve, dilatation of the root of the aorta) are for a long time clinically silent but may be nevertheless associated with serious complications. An echographic diagnosis made in time may be of decisive importance for the prevention of complications.
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PMID:[Disorders of the cardiovascular system in Turner's syndrome]. 239 89

Echocardiographic data are reported for 84 diabetes mellitus patients with reference to their age, severity and clinical pattern of diabetes and its treatment. There was left-ventricular hypertrophy, reduced end-diastolic volume and reduced stroke volume, particularly so in moderate and severe diabetes mellitus. In young insulin-treated patients (11%) with labile glycemia, an obvious mitral prolapse and, perhaps, also aortic valve prolapse were detected. This category of patients would frequently exhibit heart rate and conductivity disorders, such as second-degree sino-auricular block and frequent supraventricular extrasystoles.
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PMID:[Study of the functional status of the heart in diabetes mellitus based on echocardiographic data]. 273 17

The study was performed in 33 patients with echocardiographic diagnosis of mitral valve prolapse (PVM), without any other associated heart disease. A 19 derivations electrocardiogram (ECG) was performed a direct inscription 4 channel Samborns 150 machine at 25 and 50 mm/sec. The purpose of the study was determine the alterations in ventricular depolarization and repolarization, and to correlate them with valve prolapse, as well as with cavitary and parietal dimensions, as measured by M mode and/or two-dimensional echocardiography. Left ventricular hypertrophy detected by ECG agreed with the ECO test in 77%; the sensitivity was of 86% and specificity of 67.5%. Left ventricular hypertrophy detected by ECG was not related with the type of prolapse. Ventricular repolarization alteration was very frequent (84.8%). Association of this parameter with initial notch of R in a VF becomes important for diagnosis suspicion (p less than 0.01). When the abnormal repolarization affected the anterolateral wall, posterior valve prolapse was frequent; when the posteroinferior region was the affected one, the prolapse occurred more frequently in both valves. An important correlation (p less than 0.01) was found between left ventricular dilatation detected by ECO and the abnormal ventricular repolarization.
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PMID:[Electro-echocardiographic correlation in mitral valve prolapse]. 295 79

The purpose of this study of 6 cases of Marfan's or Marfan-like syndrome detected in 7077 echocardiographic examinations was to investigate the clinical value of echocardiography. The mean age of the patients was 40 years, and 4 of them (66 p. 100) were female. The diagnosis was based on the 4 criteria of Marfan's syndrome in 1 case, on 3 criteria in 2 cases and on 2 criteria in 3 cases. Four patients were known to have a previous cardiac murmur. Auscultation revealed a systolic murmur of mitral regurgitation in 3 cases (associated with a diastolic murmur of aortic regurgitation in 2 of them), a diastolic murmur of aortic regurgitation in 3 cases and a systolic murmur due to calcified bicuspid aortic valve in 1 case. ECG recorded a normal rhythm in 4 cases, atrial fibrillation in 2 cases of mitral regurgitation, and left ventricular hypertrophy in 3 cases. Chest X-ray showed cardiomegaly in 3 patients and severe kyphoscoliosis in one. Echocardiography visualized dilatation of the ascending aorta, severe (60 mm) in 1 case, in 3 patients; dilatation of the pulmonary artery in 1 patient; pansystolic mitral valve prolapse in 3 patients (associated with aortic and tricuspid valve prolapse in 2 of them after the disease had progressed); isolated aortic valve prolapse due to bicuspid valve in 2 patients; intracardiac calcifications in 3 patients; subaortic septal hypertrophy in 1 patient and calcified incompetent foramen ovale in 1 patient. Aortography performed in 3 patients disclosed an aneurysm of Valsalva's sinuses in 1 case and a mild aortic insufficiency in 2 cases. Two patients underwent cardiac catheterization for severe mitral regurgitation due to mitral valve prolapse requiring valve replacement, which was successfully done. Thus, echocardiography may provide an early diagnosis of Marfan's syndrome, since cardiovascular abnormalities are frequent in infancy. It also ensures a close follow-up of the disorders and it is useful in deciding whether treatment should be medical or surgical. It may detect formes frustes in a family with Marfan's syndrome, and it may define a borderline group of patients: those with Marfan-like syndrome. In these patients the cardiovascular lesions are more preponderant and appear later than in the classical Marfan's syndrome; they are often difficult to differentiate from the lesions of Barlow's syndrome.
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PMID:[Marfan's or Marfan-like syndrome: value of echocardiography]. 356 65

This is a report of a patient with mitral valve prolapse (MVP) and myocardial abnormalities on endomyocardial biopsy in whose relatives hypertrophic cardiomyopathy (HCM) was identified. A 19-year-old woman was admitted to our hospital for evaluation of a heart murmur. A systolic ejection murmur was audible in the third intercostal space at the left sternal border, and a standard 12-lead electrocardiogram showed ST-T wave changes in leads II, III and aVF. Echocardiography revealed prolapse of the anterior leaflet of the mitral valve, but no left ventricular hypertrophy. Endomyocardial biopsy disclosed mild hypertrophy and disarrangement of the myocardium. The family study revealed asymmetrical septal hypertrophy in her mother, who had no history of hypertension. Her younger sister had mild hypertrophy of the interventricular septum on echocardiography, and her histopathological findings suggested a diagnosis of HCM. This case was clinically regarded as MVP, but development of left ventricular hypertrophy as noted in her mother may occur in the future.
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PMID:[Mitral valve prolapse with myocardial disarrangement and familial hypertrophic cardiomyopathy: a case report]. 378 85

Mitral valve prolapse was observed in 26 of 92 animals in a harem breeding colony of rhesus monkeys (Macaca mulatta). The affected animals had a systolic murmur best auscultated over the mitral region with the animal in a sitting position. Mid-to-late systolic clicks were also heard. Phonocardiographic examination also demonstrated systolic murmurs and clicks in six of 16 animals. Twenty-three of the animals were studied by M mode and/or two-dimensional echocardiography. The diagnosis was confirmed in 12 animals that had a murmur during the examination. Electrocardiograms revealed T wave abnormalities in five animals and left or right ventricular hypertrophy in five. Four adult animals that died during the course of the study were confirmed at necropsy as having prolapse of the posterior and/or anterior mitral valve leaflets into the atrium. Analysis of the breeding records suggested that mitral valve prolapse was a dominant genetic trait with an approximate birth incidence of 16% to 20% in the colony. The existence of mitral valve prolapse in a nonhuman primate species provides a unique opportunity to study the disease in an experimental animal.
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PMID:Spontaneous mitral valve prolapse in a breeding colony of rhesus monkeys. 396 16

A 15-year-old boy had a history of a functional systolic murmur with systolic and diastolic murmurs and radiological evidence of left ventricular hypertrophy. The angiocardiogram demonstrated an aneurysm of the membranous septum, bicuspid aortic valve, dilatation of the left subclavian artery and left common carotid artery. Echocardiography showed a holosystolic prolapse of the mitral valve with mitral insufficiency and a bicuspid aortic valve with insignificant incompetence of the aortic valve. Classical TM mode echocardiography did not show an aneurysm of the membranous septum.
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PMID:Septal aneurysm with associated anomalies. 405 68


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