[Sudden death in young people with mitral valve prolapse or mitral valve prolapse in young people who died suddenly].
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A 63 year-old woman who had had mitral valve commissurotomy 12 years earlier was seen because of rheumatic mitral stenosis and left brachial paresis due to cerebral embolism. On clinical evaluation, a diastolic rumble was heard over the mitral area, and the echocardiogram revealed a mass attached to the mitral subvalvular apparatus. The patient was operated on, and both the surgical and histologic findings depicted papillary fibroelastoma. This tumor may occur as an isolated lesion or be associated with mitral valve stenosis or other cardiac abnormalities, and it is an important source of emboli. Early echocardiographic diagnosis, followed by surgical excision, may avoid serious complications such as stroke, myocardial infarction, and sudden death.
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To elucidate the mechanism of generation of mitral valve prolapse (MVP) in Marfan syndrome, we studied the relations of the length of the anterior mitral leaflet to the diameter of the mitral ring. In 13 cases of Marfan syndrome associated with MVP (Group I), 21 of isolated MVP (Group II), and 24 controls free from valvular disease (Group C), the maximal length of the anterior mitral leaflet (L) and the end-diastolic and end-systolic diameters of the mitral ring (Rd and Rs) were measured in the long-axis views on two-dimensional echocardiograms. In addition, the measurements were corrected with the body surface area; designated as L(c), Rd(c) and Rs(c), respectively. Furthermore, L/Rd and L/Rs, and the percentage of shortening of the mitral ring in systole, (Rd-Rs)/Rd X 100, were obtained. The body surface area in Group I was greater than that in Group II (p less than 0.05), but there was no significant difference between Groups II and C. The L was obviously different between Groups I and II or C (p less than 0.001, respectively). Similarly, there was a significant difference in L(c) between Groups I and II (p less than 0.02) or C (p less than 0.001), indicating the remarkably long anterior mitral leaflet in Group I. The Rd in Group I and II was greater than that in Group C (p less than 0.01 and p less than 0.02, respectively), while there was no significant difference in the Rd(c) among the 3 groups. There was also no statistical difference in either Rs or Rs(c) among the 3 groups. Both L/Rd and L/Rs in Group I were remarkably higher than those in Groups II and C (p less than 0.001, respectively), whereas these parameter did not differentiate Group II from Group C. (Rd-Rs)/Rd X 100 was higher in Group I than that in Groups II and C (p less than 0.05 and p less than 0.01, respectively), but it was not significantly different between Groups II and C. These results suggested that one of the probable mechanisms of generation of MVP in Marfan's syndrome is the redundant mitral valve resulting from a remarkably long leaflet and increased systolic shortening of the enlarged mitral ring.
The relation between mitral valve opening and transmitral blood flow was investigated by M-mode echocardiography and Doppler ultrasound in 50 normal subjects to allow the measurement of the timing of the end of isovolumic relaxation. Standard parasternal M-mode echocardiograms of the mitral valve to show the onset of cusp separation were recorded with a simultaneous electrocardiogram and phonocardiogram. Pulse wave Doppler ultrasound using both amplitude and spectral analysis was recorded with a transducer at the apex, and the initial diastolic blood flow towards the transducer was taken as the onset of flow. For each subject five cardiac cycles of similar length were measured using the three methods. Isovolumic relaxation could be measured with a high degree of reliability (retest reliability coefficient greater than 0.94). The echocardiographic measurement of isovolumic relaxation ranged from 52 to 82 ms (mean(SD) 67(9) ms). Isovolumic relaxation measured by Doppler was 52-83 ms (mean(SD) 67(9) ms) using amplitude analysis and 54-89 ms (mean(SD) 72(11) ms) using spectral analysis. There was a strong correlation between the echocardiographic isovolumic relaxation and measurements made using spectral analysis (r = 0.93, slope 0.97) and amplitude analysis (r = 0.97, slope 0.98). Therefore in normal subjects the end of isovolumic relaxation can be reliably measured by echocardiographic and Doppler methods, and whereas the amplitude signal is coincidental with that measured by echocardiography that measured by spectral analysis is delayed by approximately 5 ms.
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A simple, effective technique for testing the results of repair and reconstructive procedures on the mitral valve apparatus is described. This technique can be used in the operative setting of combined aortic valve replacement and mitral valve repair where other reported techniques for testing the valve apparatus are rendered unfeasible.
Long term results were compared in 81 operative survivors with MVR using Hancock valve (Hancock group) and 83 using St. Jude Medical valve (SJM group). The cumulative follow-up was 709.0 patients-year (p-y) and 175.2 p-y in the Hancock and SJM groups. Twenty-four percent of the patients in the Hancock group was permanently anticoagulated, while 100% of patients in the SJM group anticoagulated. The 7-year actuarial survival rate including early mortality was 79.3 +/- 4.6% for the Hancock and 93.9 +/- 3.0% for the SJM group (p less than 0.01). The survival rate was significantly higher in the SJM group than that in the Hancock group. The 7-year actuarial event free rate of the valve-related complications in the Hancock and SJM groups were as follows; thromboembolism 88.6 +/- 3.8% vs 95.0 +/- 2.8% (NS), hemorrhage, 94.0 +/- 3.0% vs 98.7 +/- 1.8% (NS), paravalvular leak 92.6 +/- 3.2% vs 97.4 +/- 1.8% (NS), infection 93.9 +/- 3.0% vs 100% (p less than 0.05), valve malfunction 78.0 +/- 5.2% vs 100% (p less than 0.001), overall valve related complications 58.9 +/- 5.9% vs 91.1 +/- 3.5% (p less than 0.001), reoperation for valve-related complication 83.1 +/- 4.6% vs 100% (p less than 0.01). The event free rate of reoperation for valve related complication in the Hancock group was significantly lower than that in the SJM group. The Hancock valve had the acceptable antithrombogenicity, but had the limited long-term durability.(ABSTRACT TRUNCATED AT 250 WORDS)
To evaluate the relationship between mitral valve prolapse (MVP) and autonomic nerve dysfunction, clinical and immunohistochemical studies were performed. I. Clinical study Autonomic function tests and supine bicycle exercise were performed in 60 patients with MVP, 41 under 35 years of age and 19 of 35 years and older, and the results were compared with those of 31 normal controls, 19 under 35 years of age and 12 of 35 years and older. Case with positive response on postural stress and cold pressor tests were more frequent in both MVP groups than those of controls. Plasma nor-adrenaline levels were higher in the MVP groups than in the control groups during exercise. Our clinical observation suggested that autonomic dysfunction, particularly sympathetic hyperactivity, probably is present in MVP irrespective of age. II. Immunohistochemical study In 23 mitral valves (15 of normal controls and eight of MVP), three normal tricuspid, three normal aortic and three normal pulmonic valves, immunohistochemical localizations of S-100 protein, glial fibrillary acidic protein (GFAP) and neurofilament protein (NFP) were examined to detect the distribution of all nerve endings, choline acetyltransferase (ChAT) to detect the distribution of cholinergic nerve fibers, and neuropeptide Y (NPY) and calcitonin gene related peptide (CGRP) to detect the distribution of afferent nerve fibers, using the avidin-biotin peroxidase complex (ABC) method. The distribution of adrenergic nerve fibers was examined by fluorescence with the glyoxylic acid method. In normal valves, S-100 protein was mainly demonstrated in the base and body of the valve cusp. It was very scanty in the tip, and was only found in the coaptation zone that is, the layer of the atrialis or along the boundary between the atrialis and the spongiosa in the atrioventricular valves. The distribution of S-100 protein in the prolapsed mitral valve was the same as that of the normal valve except for the lack of S-100 protein in the degenerated portion. The distribution of GFAP, NFP, ChAT, NPY, CGRP and adrenergic fluorescence were the same as that of S-100 protein in both the normal and prolapsed mitral valves. It is suggested that the above-mentioned anatomical distribution of the nerve in the mitral valve is closely related to the dysfunction of the autonomic nerve system in the condition of mitral valve prolapse, because the body of the mitral valve is rich in nerve endings and this area is most easily influenced by the mechanical stimulation due to abnormal coaptation.
OBJECTIVES: The present study evaluates the long-term course of aortic valve disease and the need for aortic valve surgery in patients with rheumatic mitral valve disease who underwent mitral valve surgery. BACKGROUND: Little is known about the natural history of aortic valve disease in patients undergoing mitral valve surgery for rheumatic mitral valve disease. In addition there is no firm policy regarding the appropriate treatment of mild aortic valve disease while replacing the mitral valve. METHODS: One-hundred thirty-one patients (44 male, 87 female; mean age 61+/-13 yr, range 35 to 89) were followed after mitral valve surgery for a mean period of 13+/-7 years. All patients had rheumatic heart disease. Aortic valve function was assessed preoperatively by cardiac catheterization and during follow-up by transthoracic echocardiography. RESULTS: At the time of mitral valve surgery, 59 patients (45%) had mild aortic valve disease: 7 (5%) aortic stenosis (AS), 58 (44%) aortic regurgitation (AR). At the end of follow-up, 96 patients (73%) had aortic valve disease: 33 AS (mild or moderate except in two cases) and 90 AR (mild or moderate except in one case). Among patients without aortic valve disease at the time of the mitral valve surgery, only three patients developed significant aortic valve disease after 25 years of follow-up procedures. Disease progression was noted in three of the seven patients with AS (2 to severe) and in six of the fifty eight with AR (1 to severe). Fifty two (90%) with mild AR remained stable after a mean follow-up period of 16 years. In only three patients (2%) the aortic valve disease progressed significantly after 9, 17 and 22 years. In only six patients of the entire cohort (5%), aortic valve replacement was needed after a mean period of 21 years (range 15 to 33). In four of them the primary indication for the second surgery was dysfunction of the prosthetic mitral valve. CONCLUSIONS: Our findings indicate that, among patients with rheumatic heart disease, a considerable number of patients have mild aortic valve disease at the time of mitral valve surgery. Yet most do not progress to severe disease, and aortic valve replacement is rarely needed after a long follow-up period. Thus, prophylactic valve replacement is not indicated in these cases.
The advantages of mitral valve repair in mitral regurgitation are well established. The aim of this study was to analyze retrospectively the early and mid-term results of primary mitral valve repair. Between January 1995 and August 2001, primary mitral valve repair operations were performed on 112 patients (76 men and 36 women) with mitral regurgitation; the mean age was 59.8 +/- 16.5 years. Mitral valve repair was performed for rheumatic heart disease (13), degenerative disease (60), infective endocarditis (2), ischemic heart disease (26), congenital heart disease (2), and dilated cardiomyopathy (9). The degree of mitral regurgitation was moderate in 28 patients, moderately severe in 17 patients, and severe in 67 patients, as determined by echocardiography. Left ventricular ejection fraction (mean 41.2% +/- 13.5%) was measured by radionuclide ventriculography. Fifty percent of patients underwent additional procedures (e.g., coronary artery bypass grafting). The mortality rate was 8.9% (10/112) for hospitalized patients, 19.2% (5/26) for ischemic disease, and 5% (3/60) for degenerative disease. Follow-up duration ranged from 0.37 to 84.03 months. All survivors were evaluated with serial echocardiography. At the final follow-up echocardiography of 94 patients without reoperation and 6 patients at the time of reoperation, 84% had no or mild mitral regurgitation. The survival rate at 5 years was 92.68 +/- 3.57% for degenerative disease and 44.47% +/- 10.89% for ischemic disease. The risk of infective endocarditis (1.78%), thromboembolism (1.78%), and anticoagulant-related complications (0.8%) was very low during the follow-up period. The pathophysiological mechanism resulting in mitral regurgitation was the most important determinant of outcome after mitral valve repair. Excellent results were achieved with mitral valve repair for degenerative disease. Because of low morbidity and acceptable mortality, we strongly recommend mitral valve repair for mitral regurgitation due to degenerative disease.
The postoperative regional left ventricular motion of 22 patients with a diagnosis of mitral regurgitation, and who underwent mitral valve replacement with preservation of chordae tendineae, were retrospectively analyzed by cineangiography in the early postoperative period and by multiple-gated cardiac blood pool scintigraphy in the mid-to-late postoperative period. The operation consisted of the division of the anterior leaflet into anterior and posterior segments, the shifting and reattachment of the divided segments to the mitral ring of the respective commissural areas, and the use of a low-profile bileaflet prosthetic valve. Control groups consisted of 28 patients with mitral regurgitation who underwent mitral valve replacement with a conventional technique and 16 patients who underwent mitral valve repair. Compared with the conventional mitral valve replacement group, the radial shortening of the left ventricle of the chordae-preserved mitral valve replacement group was greater at the apical septal, inferoapical, anterobasal, and anterolateral portions, whereas the radial shortening of the repair group was greater than that of the chordae-preserved group only at the inferolateral portion. The ejection fraction of the whole left ventricle was statistically greater in the chordae-preserved group, and also regional ejection fraction of the chordae-preserved mitral valve replacement group was greater at the apical septal, inferoapical, inferolateral, anterobasal, and anterolateral portions than that of the conventional mitral valve replacement group at these portions. On the other hand, the postoperative regional and global motion was identical to that of the mitral valve repair group except at the inferolateral portion. The result of this study supports a concept that maintenance of continuity between the mitral anulus and the papillary muscle has a beneficial effect on postoperative left ventricular performance.
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AIM: The principal techniques for surgical correction of mitral valve regurgitation (MR) were compared, with emphasis on the economic impact. METHODS: In a prospective non-randomized study 225 patients undergoing mitral valve repair were analyzed, 75 had mitral valve plasty (MVP) and 150 had mitral valve replacement (MVR). Patient demographics showed no group differences. RESULTS: Cardiopulmonary bypass time and ischemia time were shorter in the MVP-group, p<0.0001. Hospital mortality was lower after MVP, 2.0% (3/150) compared to MVR, 6.7% (5/75). ICU-stay was shorter in the MVP-group and so was length of postoperative hospital stay, p=0.014. Urgent operation was the only significant risk factor for mortality after MVP. Re-operation, endocarditis, grade IV MR, and NYHA class IV were additional risk factors in the MVR-group. Postoperative improvements of NYHA and mitral valve function were similar in both groups. MVP was more cost effective than MVR (18,050 USD or 20,430 Euro versus 24,824 USD or 28,097 Euro, p<0.001). CONCLUSION: Mitral valve plasty for MR is efficient and associated with shorter CPB and ischemia times as well as length of stay in ICU, together with a lower device cost, which makes MVP more cost effective than MVR.
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To determine mitral valve and extravalvular findings associated with mitral valve prolapse (MVP) in patients with the Marfan syndrome, we compared mitral leaflet and anular dimensions and motion by computerized two-dimensional echocardiography in 53 Marfan patients (28 with M-mode echocardiographic MVP) to those in 48 adults with primary MVP and in 35 normal subjects. Mitral leaflet billowing occurred in 28 of 28 Marfan patients with M-mode MVP versus 24 of 48 with primary MVP (p less than 0.00005), 0 of 25 Marfan patients without M-mode MVP, and 0 of 35 normal subjects (both, p less than 0.0001). Billowing occurred on the first systolic frame in 8 of 28 Marfan-MVP patients, in whom posterior leaflet chordae arose abnormally from the posterior ventricular wall, and in no other subjects. These patients had large mitral valves and normal anular dynamics, whereas the remaining 20 Marfan-MVP patients had increased systolic anular expansion. Marfan-MVP patients were younger than those without MVP (29 +/- 12 vs. 38 +/- 15 years, p less than 0.02) and had lower body mass index (19.8 +/- 2.7 vs. 23.9 +/- 2.9 kg/m2, p less than 0.00005) and systolic blood pressure (120 +/- 20 vs. 133 +/- 20 mm Hg, p less than 0.05), similar to differences between primary MVP and normal subjects in body mass index (21.5 +/- 3.0 vs. 23.9 +/- 4.8 kg/m2, p less than 0.01) and systolic pressure (118 +/- 14 vs. 125 +/- 18 mm Hg, p less than 0.05). Marfan patients with and without MVP had similar arm span, arm span to height ratio, upper to lower segment ratio, and prevalence of ectopia lentis and thoracic bony abnormalities, but arachnodactyly was more frequent in those with MVP (82% and 48%, respectively; p less than 0.02). We conclude that 1) leaflet billowing occurs more uniformly in Marfan patients with MVP than in primary MVP, 2) MVP in Marfan patients may be due to either valve enlargement with distinctively abnormal chordal architecture or abnormal mitral anular distensibility, 3) Marfan patients with MVP have low body weight and systolic blood pressure, similar to primary MVP, and 4) Marfan patients with MVP more commonly have arachnodactyly but otherwise have similar skeletal and anthropometric characteristics to other Marfan patients.
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A "semiopen" diastolic equilibrium position of the mitral valve that is assumed in the absence of transmitral flow has been demonstrated in instrumented dogs. It has been suggested that the papillary muscles and chordae tendineae play an integral role in returning the valve to this position after initial diastolic opening. To determine whether such a model of mitral valve motion is valid in noninstrumented humans without underlying valvular disease, M-mode and Doppler ultrasound studies were performed in 6 subjects who underwent prolonged periods of ventricular diastole and atrial inactivity. After maximal opening, the mitral valve assumed a stable open position in which mean separation of the anterior and posterior mitral leaflets was 52% of maximal separation. This position was maintained for a mean duration of 585 ms, during which time transmitral flow fell to 0 m/s. Peak mitral valve opening preceded the early transmitral flow peak by an average of 42 ms, suggesting an active closure of the mitral valve as flow continued to accelerate. It is concluded that a semiopen equilibrium position of the mitral valve is assumed during prolonged diastolic periods in the absence of transmitral flow and is an accurate model describing diastolic mitral valve motion in noninstrumented humans. The chordae tendineae and papillary muscles may actively participate in the genesis and maintenance of the equilibrium position.