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

This report reviews the clinical features of 80 patients with roentgenographically proved mitral annular calcification. The mean age of the group was 73 years, and there was a 2.5 to 1 female to male ratio. Evaluation for underlying cardiovascular disease revealed six patients with severe calcific valvular aortic stenosis; five patients with hypertrophic cardiomyopathy, 11 with mitral prolapse and 33 with significant arterial hypertension (blood pressure greater or equal to 150/96 mm Hg). Eighty-five per cent of the group (68 of 80 patients) had an underlying cardiac disorder associated with either chronically increased left ventricular systolic pressure or abnormal leaflet motion. Other cardiovascular abnormalities occurring as complications secondary to the mitral ring calcification included subacute bacterial endocarditis (three cases), arterial emboli (five episodes) and high grade atrioventricular block (16 cases). Twelve patients had severe mitral regurgitation; successful mitral valve replacement was carried out in four patients (all with myxomatous mitral tissue). Evidence of diffuse conduction system disease, not limited to the area of the cardiac fibrous skeleton, was found frequently (44 patients). Nine patients had sinus node dysfunction and 35 patients had electrocardiographic evidence of distal intraventricular (fascicular) block. Twenty-one patients eventually required pacemakers for management of symptomatic bradyarrhythmias. Atrial fibrillation was present in 23 patients. In this review it was found that calcification of the mitral annulus is frequently associated with or induces serious cardiovascular disease. Since some of these disorders may be modified by appropriate therapy, calcification of the mitral annulus should no longer be ignored as a benign marker of the elderly heart.
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PMID:Calcification of the mitral annulus: etiology, clinical associations, complications and therapy. 15 99

Among 119 cases of fatal dissecting aneurysm of the aorta, exclusive of those iatrogenically caused or associated with arachnodactyly or aortic stenosis, there were observed 11 cases of congenital bicuspid aortic valve (9%). The ages ranged from 17 to 69 years, five of the patients being 29 years old or younger. Among the latter, three had coarctation of the aorta and one had Turner's syndrome without coarctation. In one of the older patients, aortic insufficiency was present. Hypertension was either established or inferred from cardiac weight in 73% of the cases. In each case, cystic medial necrosis of the aorta was present. Prolapse of valves other than the aortic was observed in 45% of the cases with bicuspid aortic valve. Compared to an estimated incidence of bicuspid aortic valve of about 1 to 2% in the population, the high incidence among subjects with dissecting aneurysm suggests a causative relationship between bicuspid aortic valve and aortic dissecting aneurysm.
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PMID:Dissecting aortic aneurysm associated with congenital bicuspid aortic valve. 63 1

Four adult women with histories of rheumatic fever and clinical findings of mitral stenosis and regurgitation had echocardiograms demonstrating moderately severe mitral stenosis (EF slope less than 20 mm/sec, mean left atrial size 3.0 cm/m2, mean anterior mitral leaflet excursion 25 mm) as well as typical mitral valve prolapse. Three patients underwent cardiac catheterization which confirmed the presence of mitral stenosis, as well as systolic prolapse and excessive scalloping of the mitral valve with no visible mitral calcium and no coronary artery disease. One patient had associated mild aortic stenosis and regurgitation. Two patients underwent mitral valve surgery which revealed anterior and posterior commissural fusion consistent with rheumatic disease and intact chordal apparatus. Both leaflets were large and the anterior leaflets were redundant. There were no vegetations. Pathology revealed myxomatous degeneration of the valve leaflets. In the absence of heavy calcification and thickening, the presence of mitral stenosis with commisural fusion does not exclude the possibility of a redundant mitral valve. When these entities coexist, systolic clicks may be absent.
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PMID:Mitral valve prolapse in rheumatic mitral stenosis. 92 8

Aortic root echocardiograms were recorded from 89 patients whose aortic valves had also been adequately defined by selective angiography or viewed surgically or at autopsy. The eccentricity index (E.I.) of the aortic leaflets was measured at the onset of diastole and an E.I. of 1.3 or greater was taken as abnormal. Of 31 patients with isolated nonobstruced or mildly obstructed bicuspid aortic valves (7 viewed previously at valvotomy and 24 diagnosed radiologically) 23 (74%) had an abnormal E.I. Varying eccentricity occurred in some of these patients. Central leaflet echoes (E.I. of 1.0 to 1.25) were present in the other eight patients. All 14 patients with nonobstructed tricuspid aortic valves had central echoes. Additional multilayered diastolic echoes were found in patients with bicuspid aortic valves as well as in two patients with abnormal tricuspid aortic valves. The valves of 13 patients with aortic stenosis or incompetence were viewed surgically and the E.I. was abnormal in all patients with a bicuspid aortic valve in this group. Aortic leaflet echo findings were not diagnostically helpful in ten patients with tetralogy of Fallot, one of whom had a normal E.I. with a surgically confirmed bicuspid aortic valve. Of 21 patients with VSD only one had a bicuspid aortic valve but six had an abnormal E.I. This false positive sign was related to a high membranous VSD, sometimes with aortic valve prolapse. It is concluded that an E.I. of greater than or equal to 1.3 in the absence of an associated VSD is diagnostic of a bicuspid aortic valve and can be expected to be found in approximately three-quarters of subjects with this abnormality.
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PMID:Echocardiographic assessment of bicuspid aortic valves. Angiographic and pathological correlates. 124 58

A consecutive series of 912 surgically excised aortic valves was evaluated by means of macroscopic and histologic study. Pure aortic stenosis was diagnosed in 203 patients (p.) (22.25%), pure incompetence in 125 (13.72%) and combined dysfunction in 584 (64.03%). The diseases affecting the valves were: a) chronic rheumatic disease (593 p., 65%); b) dystrophic calcifications (214 p., 23%); c) noninflammatory aortic root disease (NIARD) and/or myxomatous infiltration of aortic cusps, floppy aortic valve (FAV) (55 p., 6%) d) infective endocarditis (50 p., 5.5%). Males outnumbered females with a ratio ranging from 2.4 (dystrophic calcific disease) to 1.6 (infective endocarditis). The mean age ranged from 37 +/- 7.5 (NIARD) to 61.2 +/- 6.3 (dystrophic calcific disease). Chronic rheumatic disease was the most frequent cause of stenoincompetence (542 p., 91.4%) while isolated stenosis was prevalently due to dystrophic calcification (172 p., 80.4%). The diseases causing isolated aortic incompetence were (in order of frequency): a) NIARD and/or FAV (55 p., 44%); b) infective endocarditis (50 p., 40%); and c) rheumatic disease (30 p., 16%). The 55 patients with NIARD and or FAV were divided into 3 groups: a) 23 p. with aortic root dilatation and normal cusps; b) 20 p. with aortic root dilatation and FAV; c) 12 p. with FAV but undilated aortic root. Aortic regurgitation was caused by cusp derangement in rheumatic disease (shortening, retraction) and infective endocarditis (perforations, erosions). Cusps diastasis and prolapse were the cause of regurgitation in aortic root dilatation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Surgical pathology of the aortic valve: a morphologic study on 912 surgically excised valves]. 129 12

This is the case of a female patient, 31 years of age with the diagnosis of aortic stenosis, prolapse of mitral valve, aortic insufficiency, with pregnancy. The literature is reviewed and the management of this type of condition is analyzed.
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PMID:[Aortic stenosis and pregnancy: presentation and physiopathology of a case]. 161 4

Twenty-eight consecutive patients underwent aortic valvuloplasty for aortic insufficiency caused by leaflet prolapse. The technique involved triangular resection of the free edge of the prolapsing leaflet, annular plication at the commissure, and resection of a raphe when present in bicuspid valves. Mean age of the patients was 46.8 +/- 14.4 years. Twenty-six (92.7%) were male. Seventy-five percent of the patients had a bicuspid aortic valve; the remaining valves were tricuspid. The extent of aortic insufficiency was 3.6 +/- 0.8 by aortography, 3.1 +/- 0.1 by preoperative Doppler echocardiography, and 3.4 +/- 0.7 by intraoperative Doppler echocardiography. The amount of aortic insufficiency decreased from 3.4 +/- 0.7 to 0.6 +/- 0.5 intraoperatively, immediately after repair (p less than 0.001). Mean transvalvular gradient by echocardiography was 12.9 +/- 6.8 mmHg. There was one death in a patient who had an intraoperative cerebral vascular accident. Mean follow-up was complete at 6.9 months. One patient had a cerebral vascular accident and one patient required reoperation for recurrent aortic insufficiency caused by partial suture line dehiscence. In 15 patients with late echocardiograms, aortic insufficiency did not progress (0.7 +/- 0.6 in the hospital and 0.8 +/- 0.5 late). Aortic valve repair for aortic cusp prolapse effectively eliminates aortic insufficiency without causing aortic stenosis. At early follow-up the repair has been stable.
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PMID:Valvuloplasty for aortic insufficiency. 192 33

This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2:1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticists. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10-40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Spectrum of cardiovascular anomalies in Williams-Beuren syndrome. 845 33

From 1986 to 1988, balloon aortic valvuloplasty was performed in 32 patients with congenital valvular aortic stenosis. The patients ranged in age from 2 days to 28 years (mean +/- SD 8.3 +/- 5.9). One balloon was used in 17 patients and two balloons were used in 15 patients. Immediately after valvuloplasty, peak systolic pressure gradient across the aortic valve decreased significantly from 77 +/- 27 to 23 +/- 16 mm Hg (p less than 0.01), a 70% reduction in gradient. At early follow-up study (4.1 +/- 3.3 months after valvuloplasty), there was a 48 +/- 20.5% reduction in gradient compared with that before valvuloplasty, and at late follow-up evaluation (19.2 +/- 5.6 months), a reduction in gradient of 40 +/- 29% persisted. Echocardiography showed evidence of significantly increased aortic regurgitation in 10 patients (31%) and aortic valve prolapse in 7 patients (22%). There was no correlation between the balloon/anulus ratio and the subsequent development of aortic regurgitation or prolapse. In fact, no patient who showed a significant increase in aortic regurgitation had had a balloon/anulus ratio greater than 100%. It is concluded that balloon aortic valvuloplasty effectively reduces peak systolic pressure gradient across the aortic valve in patients with congenital aortic stenosis. However, subsequent aortic regurgitation and prolapse occur in a significant number of patients, even if appropriate technique and a balloon size no greater than that of the aortic anulus are used.
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PMID:Gradient reduction, aortic valve regurgitation and prolapse after balloon aortic valvuloplasty in 32 consecutive patients with congenital aortic stenosis. 237 24

We describe a family with a high frequency of supravalvular aortic stenosis. The family includes 5 generations and 80 subjects (prospective study in 66, on whom physical examination, ECG, M-mode and two-dimensional echocardiogram were performed, and retrospective analysis of available data in 14). This is the largest family group with this disease studied so far. Thirty-six subjects (45%) were found to be affected. On the basis of the echocardiographic image and of the haemodynamic gradient (when available), three different degrees of supravalvular aortic stenosis were identified. The disease was found to be severe in 8 subjects (22%), moderate in 6 (17%), mild in 13 (36%) and undefined in 8 (22%). In 4 cases multiple pulmonary stenoses were associated with supravalvular aortic stenosis, while in one subject multiple pulmonary stenoses were noted in the absence of aortic abnormalities. In the family we studied, the supravalvular aortic stenosis gene is transmitted with a pattern of inheritance consistent with an autosomal dominant trait with variable expressivity and penetrance (penetrance coefficient = 0.86). A high mortality rate in early childhood was observed, while symptoms and ECG abnormalities were not related to the degree of the stenosis. Furthermore, we found a high rate of mitral valve echocardiographic abnormalities, such as mitral prolapse and systolic anterior motion. The absence of Williams dysmorphic somatic features in the many generations as well as in the large number of patients we studied, appears to exclude the coexistence of Williams and Eisenberg's syndromes in the same family group.
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PMID:[Supravalvular aortic stenosis: clinical and genetic study of a family group]. 280 84


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