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1.
A R Morales R Romanelli R J Boucek L G Tate R T Alvarez J T Davis 《Human pathology》1992,23(2):129-137
Because of the microscopic features of the affected leaflets in mitral valve prolapse (MVP), myxoid degeneration of the valve is a common pathologic designation applied to this condition. We undertook this study as a means of gaining an insight into the occurrence and prevalence of extravalvular cardiac alterations in hearts with severe MVP. Tissues of 24 hearts with severe myxomatous transformation of the mitral valve as the sole cardiac abnormality were examined. Eighteen of the 24 subjects with severe MVP died suddenly. Only two of these had pathologic evidence of severe mitral insufficiency. Twenty-four normal hearts served as controls. The two groups of hearts came from victims of homicide, suicide, accident, or natural death. Sections of the mitral valve, working myocardium, conduction system, and cardiac nerves and ganglia were studied by routine and special connective tissue and proteoglycan stains. Similar to the findings in severely affected mitral valves, prominent deposits of proteoglycans in neural and conduction tissue readily distinguished hearts with myxomatous valve changes from the control hearts. We conclude that the commonly recognized local derangement of valvular tissue in MVP is but one specific reflection of a more general myxomatous alteration in cardiac connective tissue. 相似文献
2.
Olsen LH Mortensen K Martinussen T Larsson LI Baandrup U Pedersen HD 《Journal of comparative pathology》2003,129(2-3):120-130
Comparable pathological changes in the mitral valve have been described in dogs, pigs and human patients with myxomatous mitral valve disease (MMVD), i.e., primary mitral valve prolapse. The progressive myxomatous changes are probably a response to repeated impact on the leaflets, and endothelial stress or damage probably plays a central role in the pathogenesis. Little, however, is known about the vasoactive substances that mediate the subendothelial changes. The aim of this study was to investigate the expression of nitric oxide synthase (NOS) in canine mitral valve leaflets and to relate the findings to MMVD changes. The mitral valve was taken post mortem from 12 dogs (six males and six females) and a whole valve NADPH (the reduced form of nicotinamide-adenine dinucleotide phosphate) diaphorase (NADPH-d) reaction was performed. Macroscopical (semiquantitative) and microscopical (computer image analysis) evaluations of the staining due to NADPH-d activity were performed at four specific areas of the valve and related to microscopical signs of MMVD and gross signs of thickening or prolapse, or both. Macroscopically, the NADPH-d colour grade was correlated with the degree of MMVD (P=0.01). In addition, endothelial NADPH-d staining intensity was correlated with macroscopical signs of disease (P=0.004) as well as with collagen degeneration (P=0.008) and deposition of mucopolysaccharides (P=0.02). Age, gender and specific area of the valve did not seem to influence the NADPH-d activity. In conclusion, increased NADPH-d activity, suggesting increased NOS expression, was found in areas of the mitral valve with myxomatous changes. This indicates that nitric oxide (NO) may play a role in the pathogenesis of MMVD in dogs. 相似文献
3.
Until recently, the cause of isolated aortic insufficiency (AI) was usually thought to be inflammatory or rheumatic in most cases. However, at our institution we have noted a high prevalence of myxomatous degeneration (MD) in aortic valves removed for AI. In this study we report anatomic observations on valves from 56 consecutive patients with isolated AI undergoing aortic valve replacement surgery. Fifty-six consecutive aortic valves removed at our institution from 1994 to 1996 for isolated AI and/or aortic aneurysm were reviewed. Anatomic features were compared with clinical history and echocardiographic data. The anatomic results were also compared to 22 age-matched control aortic valves obtained at autopsy. In 13/56 cases (23%), a specific valvular cause of AI was determined (infectious endocarditis, seven cases; chronic rheumatic disease, four cases; congenital bicuspid valve, two cases). Of the remaining (idiopathic) 43 cases, 18 (42%) had severe isolated MD defined as >50% expansion of the spongiosa and disruption of the fibrosa by the deposition of acid mucopolysaccharides in the absence of severe calcification, fibrosis, or other pathologic findings. Only 1/22 aortic valves from the autopsy controls had severe MD. Eighteen of the 56 patients also had a clinical history of aortic dilatation/aneurysm of which 12 were confirmed to be dilated by echocardiographic criteria. Of these 12, five (42%) had MD of the aortic valve only, three (25%) had both MD and cystic medial degeneration (CMD) of the aorta, two (17%) had CMD of the aorta only, and two (17%) had no specific diagnosis. Isolated MD of the aortic valve is the most common cause of isolated AI in our patient population. Furthermore, in a subset of non-Marfan’s patients with both AI and dilatation of the aortic root/aortic aneurysm the incidence of MD is even higher (67%). These results suggest that there is overlap between MD and CMD in non-Marfan’s patients and that both entities may be part of a spectrum of a generalized connective tissue disorder. 相似文献
4.
《European journal of medical genetics》2020,63(4):103854
ObjectivePolyvalvularmyxomatous degeneration is a rare clinical condition. A 51-year-old male patient presented at our centre with all four heart valves with myxomatous degeneration and severe mitral and aortic regurgitation due to leaflet prolapse. The patient referred five further family members with valvular heart disease at different stages of presentation. The aim of this study was to investigate the genetic basis of this familial polyvalvularmyxomatous degeneration which was associated with mild dysmorphic facial anomalies and short stature.DesignA detailed family history was recorded. Nine members of the family, affected or not by valvular heart disease, were studied clinically, echocardiographically and by detailed genetic analyses.ResultsSix of the nine family members had echocardiographic features of different degrees of degenerative heart valve disease. In addition, the affected subjects shared similar mild dysmorphic facial anomalies and short stature. Exome sequencing identified a rare heterozygous single nucleotide deletion in the TAB2 gene in all affected family members, which was absent in the unaffected members.ConclusionsA variant in the TAB2 gene is proposed as the cause of syndromic congenital heart disease, displaying congenital myxomatous degenerative heart valve disease, mild dysmorphic fascial anomalies and short stature in this family. 相似文献
5.
Jagdish Butany Pradeep Vaideeswar Vidya Dixit Vidyadhar Lad Annette Vegas Tirone E. David 《Cardiovascular pathology》2009,18(1):11-18
BackgroundAneurysms of the ascending aorta occur as result of intrinsic changes in the aortic wall and have been well documented in patients with bicuspid aortic valve (BAV). In few reported clinical studies, documenting aneurysmal dilatation in unicommissural aortic valves (UAV); there have been no comments on the aortic wall pathology. This study presents the pathological findings of the ascending aorta in patients with UAV.Materials and MethodsThe clinical data from 39 patients with concomitant excision of the UAV and aneurysmal aortic tissue were reviewed. In all cases, the gross features of the valve and aortic segments were noted and submitted for histology. The sections of the aorta were semi-quantitatively graded for the extent of medionecrosis, cystic medial change, fibrosis, and elastic tissue changes (fragmentation/ loss) in the media. The medial alterations were correlated with patient age, gender, and valvular dysfunction, and compared to aneurysmal disease in BAV and three-cuspid aortic valves (TAV) excised over a 3-year period.ResultsAmong 39 patients studied, a majority were males (92.3%), with a mean age at surgery of 39.92 years. Only three patients (7.69%) were above the age of 50 years. Eighteen patients (46.1%) had aortic stenosis with regurgitation. Ascending aorta diameters ranged from 4 to 5.5 cm. The overall pattern of medial changes was nearly the same in all cases of UAV, irrespective of age and nature of valvular dysfunction. Most cases showed mild histological changes, with medionecrosis and fibrosis being the more common and consistent features. However, varying grades of change affected different portions of the media and/or the aortic wall in the same patient. The changes in UAV aortae were comparable to the changes seen in the TAV and BAV, but these differed with the age of onset.ConclusionsThis study demonstrates the presence of medial changes in the ascending aortic tissue in all patients of UAV with aneurysms. These changes, while mild to moderate in degree, likely have a similar pathogenetic mechanism as those seen in BAV disease. The significant difference in age, at the time of surgery, suggests a more rapid progression of the aortic changes. 相似文献
6.
John P Veinot Wendy Prichett-Pejic Jianming Song Geeta Waghray William Parks Thierry G Mesana Marc Ruel 《Cardiovascular pathology》2006,15(1):36-40
BACKGROUND: There is no report to date of stem cells in human cardiac valves. We examined their possible presence, number, and distribution in valves removed at cardiac surgery from patients with a variety of underlying valve pathologies. METHODS: Grossly normal aortic and mitral valves were obtained from live heart transplant patients. Surgically excised valves with rheumatic mitral stenosis, aortic valve age-related degeneration, aortic valve changes of aortoannular ectasia, and mitral valves with myxomatous degeneration were studied. Immunohistochemical and histochemical studies were performed on sequential valve sections, including hematoxylin and eosin, hematoxylin phloxine saffron, Movat pentachrome, toluidine blue, CD31, CD34, and CD117. RESULTS: There were small clusters of CD117-positive cells in the fibrosa and spongiosa of mitral and aortic valves from all groups of valves. Sequential sectioning and staining showed that almost all of these cells were mast cells. However, in the mitral myxomatous valves and the mitral rheumatic valves, there were rare CD117-positive cells that did not have corresponding toluidine blue staining and thus could be valve mesenchymal stem cells. CONCLUSIONS: Most of the CD117-positive cells in normal and diseased adult heart valves are mast cells. These valve cells could play a role in valve pathology and injury. A very small number of possible valve stem cells were also identified. It is unlikely that these valve stem cells are sufficient in number to allow isolation and expansion for tissue engineering purposes. 相似文献
7.
背景:在主动脉置换过程中常遇到瓣环钙化、瓣周囊肿等特殊情况,这时一般应用特殊技术辅助主动脉瓣置换。
目的:观察自体心包补片修补主动脉瓣环辅助主动脉瓣置换治疗钙化性主动脉瓣狭窄并瓣环钙化的临床可行性。
方法:回顾性分析2009年1月至 2012年1月郑州大学第一附属医院42例钙化性主动脉瓣狭窄并瓣环钙化患者的临床资料,并通过统计学软件处理自体心包补片修补主动脉瓣环技术辅助主动脉瓣置换前后的主动脉瓣有效瓣口面积指数、最大跨瓣压差、血流峰值速度、左室射血分数等数据,分析自体心包补片修补主动脉瓣环技术辅助主动脉瓣置换的应用效果。
结果与结论:无置换中死亡病例,置换中主动脉阻断时间为52-88(63.0±18.1) min,体外循环时间为78-122(102.6±25.1) min,置换后1例患者出现急性肾功能衰竭,经床旁血透治疗后治愈。余患者无严重置换并发症。置换后住院天数为7-20(13.6±5.5) d。置换后多普勒超声心动图示:瓣膜功能良好,均未发现主动脉瓣周漏。置换后6个月的主动脉瓣有效瓣口面积指数、最大跨瓣压差、血流峰值速度、左室射血分数均有显著改善,与置换前比较差异均有显著性意义(P < 0.05)。证实对置换适应证合适的特殊换瓣患者,自体心包补片修补主动脉瓣环辅助主动脉瓣置换可取得满意的外科治疗效果,且操作安全简单,是一项可行的技术。 相似文献
8.
Wanda M. Lester MD 《Cardiovascular pathology》1995,4(4):257-264
Myxomatous mitral valve disease is a cause of prolapse of mitral valve leaflet(s) into the left atrium. In this condition there is a loss of collagen from the leaflet and an accumulation of glycosaminoglycan. Complications of myxomatous mitral valve disease include infective endocarditis, mitral regurgitation, sudden death, and stroke. Mitral valve prolapse has been reported to occur in association with many syndromes. In some of these, such as Marfan syndrome, the pathology of the mitral valve has been well described and is that of a myxomatous valve. However, in some of the other disorders associated with mitral valve prolapse, there is no information yet available on the valve pathology, and this would be useful in order to better understand the pathogenesis of myxomatous mitral valve disease. 相似文献
9.
Chr Ladefoged Niels Rohr 《Virchows Archiv : an international journal of pathology》1984,404(3):301-312
Summary The material from 100 consecutive aortic and mitral valve operations has been studied histologically with particular reference to the presence of amyloid deposits. Sixty seven per cent were positive (aortic 88%, mitral 45%).The simultaneous occurrence of calcification of the valves and amyloid degeneration as well as of calcification and hyalinization was significant. Similarly there was significantly more amyloid in the older age groups, as well as a significant correlation between the degree of hyalinization of the valve and amyloid.]Thirty-two patients had previously suffered from rheumatic fever. The heart valves of these patients did not differ histologically from the others, whereas significantly more amyloid was observed in the stenotic mitral valves than in the mitral valves which were insufficient. 相似文献
10.
Luc M Beauchesne John P Veinot Lyall A Higginson Thierry Mesana 《Cardiovascular pathology》2004,13(3):165-167
A patient with a 10-year-old Medtronic Hancock II porcine aortic bioprosthesis developed severe aortic insufficiency. A transesophageal echocardiogram showed a long and mobile mass attached to the bioprosthesis which was consistent with a torn cusp. The patient underwent replacement of the prosthesis with a mechanical valve. Pathological examination showed two subacute tears arising from the same suture buttressing site. These two tears allowed a portion of the valve apparatus to prolapse. 相似文献
11.
B M McManus R S Cassling T J Soundy J E Wilson T D Sears W C Rogler B A Buehler J F Wolford M J Duggan P H Byers 《The American journal of cardiovascular pathology》1987,1(1):55-67
The interaction of elevated blood pressure and aortic metabolism in the genesis of aortic dissection is uncharacterized. A kindred with fatal familial aortic dissection in association with precocious systemic hypertension and in absence of a definable connective tissue syndrome has undergone genealogical, clinical, pathological, and biochemical evaluation. Six family members spanning three generations have died of acute dissection. Five men died at a mean age of 28 years (range 22-34), while the proband's paternal grandmother died at 62 years of age. All were hypertensive. A constellation of subtle clinical features points toward deficient integrity of connective tissues; however, major hallmarks of known connective tissue syndromes including aortic root ectasia or aneurysms are absent. Studies of cultured dermal and aortae fibroblasts of two of the proband's brothers mitigate against Ehlers-Danlos IV syndrome. This family's susceptibility to aortic dissection reflects the synergistic liability of coexistent elevated blood pressure and metabolic abnormalities in the genesis of aortic degeneration. 相似文献
12.
Capelli C Bosi GM Cerri E Nordmeyer J Odenwald T Bonhoeffer P Migliavacca F Taylor AM Schievano S 《Medical & biological engineering & computing》2012,50(2):183-192
Transcatheter aortic valve implantation (TAVI) enables treatment of aortic stenosis with no need for open heart surgery. According
to current guidelines, only patients considered at high surgical risk can be treated with TAVI. In this study, patient-specific
analyses were performed to explore the feasibility of TAVI in morphologies, which are currently borderline cases for a percutaneous
approach. Five patients were recruited: four patients with failed bioprosthetic aortic valves (stenosis) and one patient with
an incompetent, native aortic valve. Three-dimensional models of the implantation sites were reconstructed from computed tomography
images. Within these realistic geometries, TAVI with an Edwards Sapien stent was simulated using finite element (FE) modelling.
Engineering and clinical outcomes were assessed. In all patients, FE analysis proved that TAVI was morphologically feasible.
After the implantation, stress distribution showed no risks of immediate device failure and geometric orifice areas increased
with low risk of obstruction of the coronary arteries. Maximum principal stresses in the arterial walls were higher in the
model with native outflow tract. FE analyses can both refine patient selection and characterise device mechanical performance
in TAVI, overall impacting on procedural safety in the early introduction of percutaneous heart valve devices in new patient
populations. 相似文献
13.
Rajeev Mahimkar Anita Nguyen Michael Mann Che-Chung Yeh Bo-Qing Zhu Joel S. Karliner David H. Lovett 《Cardiovascular pathology》2009,18(5):253-261
IntroductionMyxomatous mitral valve “degeneration” with prolapse (MVP) is the most frequent form of nonischemic mitral valve disease. In myxomatous valves, interstitial cells express extracellular matrix-degrading enzymes and it has been postulated that matrix metalloproteinases (MMPs) contribute to these changes.MethodsWe generated mice with cardiac-specific expression of constitutively active MMP-2 under the control of the α-myosin heavy chain promoter.ResultsThese mice are normal at 4–6 months of age; at 12–14 months the mitral valves and chordae tendineae exhibit severe myxomatous change with echocardiographic MVP. Myxomatous change was also evident to a lesser extent in the aortic valves. Myxomatous changes were heterogeneous and limited to the left side of the heart with major disorganization of collagen bundles within the lamina fibrosa. Alcian blue/PAS-stained valves revealed massive accumulation of acidic glycosoaminoglycans within the lamina spongiosa, consistent with valvular interstitial cell differentiation to a chondrocytic phenotype. Cells with the histologic features of hypertrophied chondrocytes were found within the chordae tendineae and the tips of the mitral papillary muscles.ConclusionThis report demonstrates that increased activity of a single enzyme, MMP-2, within a transgenic context reproduces many of the features of the human MVP syndrome. The cardiac-specific MMP-2 transgenic mouse potentially provides a unique experimental platform for the evaluation of nonsurgical therapies based on the underlying pathophysiology of this disease. 相似文献
14.
We investigated a total of 15 mitral valves with myxomatous degeneration and compared these with normal mitral valves. In normal mitral valves, stromal cells located in the fibrosa and spongiosa showed small bipolar cytoplasmic processes and were found to be positive for CD34, suggesting a close relationship to CD34+ fibrocytes. In cases of myxomatous degeneration, stromal cells showed an altered morphology in that they exhibited multipolar cytoplasmic processes, appeared to be hyperplastic, and were increased in number. This study is the first to report on CD34+ fibrocytes making up the majority of mitral valve stromal cells. Major factors in the development of myxomatous valve degeneration are MMP-9, as well as collagen I and III, which have been reported to be secreted by CD34+ fibrocytes. Therefore, it is likely that CD34+ fibrocytes are involved in the pathogenesis of myxomatous mitral valve degeneration. 相似文献
15.
J P Loehr D P Synhorst R R Wolfe R J Hagerman 《American journal of medical genetics》1986,23(1-2):189-194
Forty patients with fragile X [fra(X)] or Martin-Bell syndrome, confirmed by chromosome analysis, underwent full cardiac evaluation including physical examination, chest film, electrocardiography (ECG), and M-mode and 2-dimensional echocardiography. Thirty-four males and six females were studied. Although all patients were asymptomatic, seven males were found to have mild aortic root dilatation. All seven also had evidence of mitral valve prolapse. Twenty-two (55%) of the study patients had mitral valve prolapse with either a click or murmur heard on physical examination and confirmation by M-mode echocardiography. The frequency of mitral valve prolapse was the same in males and females, but 80% of males older than 18 years had mitral valve prolapse. These findings support the hypothesis of a connective tissue dysplasia in the fra(X) syndrome. 相似文献
16.
Marek Maciejewski Katarzyna Piestrzeniewicz Agata Bielecka-Dabrowa Andrzej Walczak 《Archives of Medical Science》2011,7(6):993-999
Introduction
Patients with implanted bioprostheses are at risk of structural dysfunction which results from the limited durability of biological valves. The aim of this study was to analyse the mechanism of bioprosthesis degeneration and to evaluate the usability of transthoracic and transoesophageal echocardiography in determining the indications for reoperation in 117 patients with a bioprosthesis implanted before 65 years old.Material and methods
The study comprised 117 consecutive patients (M – 27, F – 90, age 48-74 years, 57.5 ±9.5 years) with a bioprosthesis implanted under the age of 65, who were examined in accordance with the accepted protocol and whose complete clinical and echocardiographic documentation was collected. The scheduled echocardiographic examination was performed annually from the 5 year after implantation of the bioprosthesis in patients with a valve implanted over the age of 35 years and from the 1 year after bioprosthesis implantation in patients with a prosthesis implanted at a younger age. Unscheduled echocardiographic examinations were performed only on clinical indications.Results
During the period under observation, due to degeneration of the bioprosthesis 76 patients were reoperated, including 62 patients with mitral bioprostheses. In 88.7% of patients with degeneration of mitral valve bioprostheses, regurgitation was observed. In 69% of patients with aortic bioprostheses, valve dysfunction was the dominant mechanism of stenosis.Conclusions
The most common mechanism of structural dysfunction of a mitral bioprosthesis is regurgitation caused by prolapse or perforation of one of the leaflets. Degeneration of an aortic bioprosthesis usually results in aortic stenosis. In cases of bioprosthesis degeneration connected with stenosis, transthoracic echocardiography was sufficient for the evaluation of valve dysfunction. In the case of bioprosthesis dysfunction accompanied by regurgitation, transoesophageal echocardiography was more informative to decide when the operation should be performed. 相似文献17.
N. Zöllner W. G. Zoller F. Spengel B. Weigold C. K. Schewe 《Journal of molecular medicine (Berlin, Germany)》1991,69(14):633-639
Summary Since abdominal ultrasonography has become a routine diagnostic procedure, increasing numbers of small asymptomatic abdominal aortic aneurysms are detected incidentally. Of 128 patients (108 male, 20 female) with abdominal aortic aneurysms, 96 patients were observed clinically and by repeated ultrasound studies for an average of 3.47 years, adding up to a total observation period of 333 patient-years. Among these 96 patients, 72 had small aneurysms (averaged diameters less than 5 cm). Three of them were lost to follow up. None of the remaining 69 patients died from rupture, 20 died from other causes and 8 patients were successfully operated. Of the patients with a large aneurysm one was lost to follow up. Five patients of the remaining 23 died as a result of rupture, 7 were successfully operated. The average growth rate of small aneurysms was 0.18 cm/year, whereas the larger aneurysms showed a growth rate of 0.28 cm/year (diameter).The survival rate of patients with small aneurysms was 94% after one year, 80% after 3 years, and 73 % after 5 years, indicating that life expectancy is reduced in patients with an aneurysm of the abdominal aorta, but not because of complications of the aneurysm. 相似文献
18.
Mayuko Togashi Koichi Tamura Yukinari Masuda Yuh Fukuda 《Nippon Ika Daigaku zasshi》2008,75(3):138-145
Calcification of the aortic valve leads to stenosis or regurgitation or both. To clarify the mechanism of heart valve calcification, comparative studies using histological and ultrastructural examinations were performed of calcified aortic valves. These valves were obtained at valve replacement surgery from 11 patients with rheumatic aortic valvular disease (RAVD), 10 patients with degenerative aortic valve disease (DAVD), and 10 patients with congenitally bicuspid aortic valves (CBAV). For electron microscopic study, 5 cases were selected from each group. In RAVD, histological examination revealed calcification in a degenerated amorphous area at the center of fibrous thickened regions and in laminar fibrous thickened areas near the valve surface. In DAVD, calcification was observed mainly in the fibrosa near the valve ring. In CBAV, basic pathological changes were similar to those in DAVD; however, additional severe calcification of the raphe was observed, if the raphe was present. Ultrastructural examinations showed deposition of electron-dense materials in two patterns in all three groups; one pattern was observed in the interfibrillar spaces of collagen fibrils, and the other pattern was widespread macular deposition unrelated to the preexisting structure. In RAVD, microfibril-like fibrillar structures were found in the areas of deposition of electron-dense materials. These findings suggest that newly formed connective tissue degraded and became necrotic because of nutritional deprivation, especially in the thickened central area, causing calcium deposition. In DAVD and CBAV, numerous lipid vacuoles were found in the electron-dense deposition areas similar to lipid deposition in aortic atherosclerosis. Localized calcium deposition in the fibrosa suggests that the stress of valvular motion and pressure load induces sclerotic changes with the degeneration of collagen fibers, providing a core for calcification. In CBAV, the raphe was the main location of calcification, wherein spiraled collagen fibrils were observed. Increasing the hemodynamic load with abnormal structure might influence calcification. The ultrastructural pattern of calcification of the valve is common; however, additional findings suggest that the cause and mechanism are different in each type of heart valve disease. 相似文献
19.
In this study, 100 consecutive, surgically excised, mitral valves were examined pathologically. The valves were classified according to primary conditions that resulted in valvular malfunction. Rheumatic mitral valvular diseases (stenosis and/or insufficiency) accounted for 54% of the cases. Myxomatous changes (prolapse) were present in 32 cases. Fifty-nine percent (19 cases) of those cases with myxomatous changes also had chordal rupture. Four of the cases had papillary muscle rupture, and in seven cases, papillary muscle dysfunction occurred. In one case bacterial endocarditis was observed on a previously normal valve. In one case the pathology of valvular. In one case the pathology of valvular changes was indeterminant. Lupus erythematosus was diagnosed in one patient, and mitral valve insufficiency may have resulted as a complication. 相似文献
20.