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myxoid degeneration of the mitral valve

S Gasser, H Reichenspurner, E Girdauskas
BACKGROUND: Myxomatous mitral valve prolapse is a common cardiac abnormality. Morbus Barlow is characterized by excess myxomatous leaflet tissue, bileaflet prolapse or billowing, chordae elongation and annular dilatation with or without calcification. Extensive myxoid degeneration with destruction of the normal three-layered leaflet tissue architecture is observed histologically in such patients. Autosomal dominant inheritance with an age and sex-dependent expression has long been recognised...
February 27, 2018: BMC Cardiovascular Disorders
Olga C G Stam, Mat J A P Daemen, Jan Willem van Rijswijk, Bas A J M de Mol, Allard C van der Wal
INTRODUCTION: Intraleaflet hemorrhage (ILH) has been reported to occur in calcified degenerated aortic valves. At present, no such information is available for mitral valves or for other types of valvular disease. We examined the prevalence, age, and potential source of ILH in a consecutive series of surgically removed aortic and mitral valves, and related the findings to specific types of heart valve pathology. METHODS: A total of 105 aortic (n=85) and mitral (n=20) valves were retrieved from 100 symptomatic patients...
September 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Lucio Díaz-Flores, Ricardo Gutiérrez, Lucio Díaz-Flores, Miriam González Goméz, Francisco J Sáez, Juan F Madrid
We consider CD34+ stromal cells/telocytes (CD34+ SC/TCs) in normal and pathological conditions. These cells are involved in organisation and control of the extracellular matrix, structural support, creation of microenvironments, intercellular communication, neurotransmission, immunomodulation and immunosurveillance, inhibition of apoptosis, and control, regulation and source of other cell types. CD34+ SC/TCs are widely reported in the origin of interstitial cells of Cajal and in regeneration in the heart, skeletal muscle, skin, respiratory tree, liver, urinary system and the eye...
July 2016: Seminars in Cell & Developmental Biology
Lucio Díaz-Flores, Ricardo Gutiérrez, Maria Pino García, Miriam González, Lucio Díaz-Flores, Juan Francisco Madrid
This review outlines the role of CD34+ stromal cells/telocytes (CD34+ SC/TCs) in repair and considers the following issues. Firstly, the conceptual aspects of repair, including regeneration and repair through granulation tissue (RTGT) as two types of repair, RTGT stages (inflammatory, proliferative, and remodeling), and tissue in repair as a substrate to assess the in vivo behavior of activated CD34+ SC/TCs. Subsequently, current knowledge of CD34+ SC/TCs, such as identification, characteristics, and functions, as well as possible stages (quiescent and activated) are taken into account...
2016: Current Stem Cell Research & Therapy
Stefania Rizzo, Cristina Basso, Elisabetta Lazzarini, Rudy Celeghin, Adolfo Paolin, Gino Gerosa, Marialuisa Valente, Gaetano Thiene, Kalliopi Pilichou
AIMS: Dysregulation of the transforming growth factor beta (TGF-β) 1 pathway has been associated with either syndromic or isolated mitral valve (MV) prolapse due to myxoid degeneration (floppy MV). The activation of Smad receptor-mediated intracellular TGF-β pathway and its effect on adherens junction (AJ) molecular pattern of activated valvular interstitial cells (VICs) in MV prolapse are herein investigated. METHODS: Floppy MV leaflets were obtained from 30 patients (24 males, mean age 55...
November 2015: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Heena M Desai, Gayathri P Amonkar
Mitral valve prolapse (MVP) is usually asymptomatic, but can be associated with complications such as infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. It has been very rarely reported to occur in association with other valvular involvement. A 55-year-old male patient was brought dead and at autopsy the mitral valve orifice was stenotic and the leaflets were enlarged, myxoid and bulging suggestive of MVP and chordae tendinae were thickened, stretched and elongated. Similar changes were seen in the tricuspid valve...
April 2015: Indian Journal of Pathology & Microbiology
William C Roberts, Travis J Vowels, Jong M Ko, Robert F Hebeler
OBJECTIVES: The aim of the study is to describe gross and histological features of operatively excised portions of mitral valves in patients with mitral valve prolapse (MVP). BACKGROUND: Although numerous articles on MVP (myxomatous or myxoid degeneration, billowing or floppy mitral valve) have appeared, 2 virtually constant histological features have been underemphasized or overlooked: 1) the presence of superimposed fibrous tissue on both surfaces of the leaflets and surrounding many chordae tendineae; and 2) the absence of many chordae tendineae on the ventricular surfaces of the leaflets as the result of their being hidden (i...
April 29, 2014: Journal of the American College of Cardiology
R A Norris, R Moreno-Rodriguez, A Wessels, J Merot, P Bruneval, A H Chester, M H Yacoub, A Hagège, S A Slaugenhaupt, E Aikawa, J J Schott, A Lardeux, B S Harris, L K Williams, A Richards, R A Levine, R R Markwald
Myxoid degeneration of the cardiac valves is a common feature in a heterogeneous group of disorders that includes Marfan syndrome and isolated valvular diseases. Mitral valve prolapse is the most common outcome of these and remains one of the most common indications for valvular surgery. While the etiology of the disease is unknown, recent genetic studies have demonstrated that an X-linked form of familial cardiac valvular dystrophy can be attributed to mutations in the Filamin-A gene. Since these inheritable mutations are present from conception, we hypothesize that filamin-A mutations present at the time of valve morphogenesis lead to dysfunction that progresses postnatally to clinically relevant disease...
July 2010: Developmental Dynamics: An Official Publication of the American Association of Anatomists
M Dupuy, B Marcheix, E Grunenwald, Z Dickson, C Cron, D Chauveau
BACKGROUND: Wegener's granulomatosis (WG) is a granulomatous disease that can affect many organ systems. The most frequently involved organs include the upper and lower respiratory tract as well as the kidney. Cardiac involvement is rare. METHODS: We report the case of a patient with grade 4 mitral insufficiency associated with severe WG. RESULTS: Surgical analysis of the mitral valve revealed perforation of the anterior leaflet without evidence of endocarditis...
June 2009: Annales de Cardiologie et D'angéiologie
F Kyndt, S Le Scouarnec, P Jaafar, J-P Gueffet, A Legendre, J-N Trochu, V Jousseaume, A Chaventré, J-J Schott, H Le Marec, V Probst
Valvular dystrophies due to myxoid degeneration are common and potentially serious cardiac pathologies. They constitute a heterogeneous group of which the most usual is idiopathic mitral valvular prolapse (Barlow's disease). The majority of mitral valvular prolapses are sporadic, but there are several familial forms. Transmission is usually autosomal dominant with incomplete penetrance and variable expression. The first chromosomal location to be identified was on the 16p11-13 chromosome. Since then, two other loci have been identified on the 11p15...
December 2007: Archives des Maladies du Coeur et des Vaisseaux
Tomoki Hanada, K Shimizu, R Murakami, T Ota, S Okada, S Inoue, K Hiroe, Y Higaki, T Oda
An 82-year-old man was referred to our hospital because of progressive heart failure. He had Parkinson's disease and had been treated with cabergoline during the preceding 4 years and 8 months. Echocardiography revealed severe mitral regurgitation through retracted mitral leaflets with incomplete coaptation. Heart failure persisted despite pharmacologic therapy, so the mitral valve was surgically replaced with a biological valve. Histologic analysis showed fibrous thickened mitral chordae with myxoid degeneration...
October 2007: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Syed Saghir, Tom D Ivey, Dean J Kereiakes, Wojciech Mazur
We report a case of mitral valve aneurysm in a 30-year-old man presenting with fatigue, malaise, and fever 10 weeks after emergent aortic valve replacement for endocarditis. The transthoracic echocardiogram demonstrated perivalvular aortic regurgitation, but no abscess cavity was defined. Cardiac magnetic resonance imaging (MRI) revealed a mitral valve leaflet aneurysm. Both aortic and mitral valves were replaced with mechanical prostheses. Pathology of the excised mitral valve showed a focally hemorrhagic aneurysm of the anterior leaflet with myxoid degeneration and focal calcification...
2006: Reviews in Cardiovascular Medicine
John W Brown, Mark Ruzmetov, Mark D Rodefeld, Mark W Turrentine
BACKGROUND: Pulmonary autograft mitral valve replacement (PA-MVR) was introduced clinically by Ross in 1967, but has been rarely utilized in North America and Europe. The aim of this study is to review our early experience with PA-MVR. METHODS: Since June 2002, 8 patients (7 female and 1 male) between 12 to 46 years of age with outgrown mechanical valves (n = 3) failed MV repair for rheumatic or congenital MV disease (n = 4) and irreparable bileaflet myxoid prolapse (n = 1), underwent PA-MVR...
February 2006: Annals of Thoracic Surgery
Gary R Cooper, Edward D Staples, Kenneth A Iczkowski, Cornelius J Clancy
Comamonas testosteroni has rarely been implicated as a human pathogen. We report a case of infectious endocarditis due to this organism, occurring in a 49-year-old man. The posterior leaflet of the mitral valve contained a 1 x 1 cm vegetation and showed myxoid degeneration and acute inflammation. The patient had no evidence of reinfection after 32 months. The infection was almost certainly community acquired, as is usually true for this organism.
May 2005: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
M Ba, J M Dangou, O Diarra, Y Thiongane, C S Boye, M Ndiaye
Authors record a preliminary study led so as to develope techniques of preparation of the cardiac homograft valves. Eight human hearts have been appropriated in the course of medico-legal autopsy. Under sterile hodd, the heart was dissected, aortic and pulmonary valves as well as a mitral valve fragment were collected for a total of 24 valvular levies. After sterlization in an antibiotic solution, valves were preserved at 4 degrees C for the mitral fragment specimen of fresh allograft and to - 196 degrees C in Nitrogen liquidates for the pulmonary and aortic valves...
2001: Dakar Médical
Joseph F Nasuti, Paul J Zhang, Michael D Feldman, Terri Pasha, Jasvir S Khurana, Joseph H Gorman, Robert C Gorman, Jagat Narula, Navneet Narula
BACKGROUND: Unlike myxomatous degeneration in Marfan syndrome, which has been reported to result from a mutation in the gene that codes for the extracellular structural protein fibrillin, no specific molecular abnormality has been documented to be the underlying cause of myxomatous degeneration in mitral valve prolapse syndrome (MVPS). The present study examined the distribution of fibrillin and other extracellular matrix proteins in patients with isolated MVPS. METHODS: Mitral valve leaflets from 7 MVPS patients and 5 rheumatic heart disease (RHD) patients were characterized immunohistochemically for fibrillin, elastin, collagen I, and collagen III distribution, and compared with five normal mitral valves...
February 2004: Annals of Thoracic Surgery
J E Barber, N B Ratliff, D M Cosgrove, B P Griffin, I Vesely
BACKGROUND AND AIM OF THE STUDY: Chordal rupture is the most common reason for severe mitral regurgitation requiring surgery. The features that predispose myxomatous chordae to rupture, however, have not been studied. Thus, the physical and mechanical properties of normal and myxomatous mitral valve chordae were measured. METHODS: Chordae from 24 normal and 59 myxomatous mitral valves were cut into 10 mm-long segments and mechanically tested to measure extensibility, modulus, failure stress, failure strain, and failure load...
May 2001: Journal of Heart Valve Disease
P Schroeyers, F Farhat, F Van Praet, Y Vermeulen, H Vanermen
Although conventional right thoracotomy is a well-known alternative for mid-sternotomy to access the left atrium, a combined video-assisted mitral and left anterior descending (LAD) approach via Port-Access has not yet been described. We report the case of a 68-year-old patient with severe mitral valve regurgitation due to myxoid degeneration associated with a critical LAD coronary artery stenosis. A totally video-assisted 'true' Port-Access (i.e. no costal retraction) mitral valve repair and direct coronary artery bypass were performed in combination, without complications...
November 2000: Journal of Heart Valve Disease
K B Nolte
A 26-year-old woman collapsed and died suddenly while dancing. Autopsy findings included the cutaneous lesions of pseudoxanthoma elasticum (PXE), a rare genetic disease with autosomal dominant and recessive inheritance patterns. Pathologic findings of PXE (degenerated elastic fibers) were seen in the stenotic epicardial coronary arteries, the intramyocardial arterioles, the subendocardium, the mitral valve, and the blood vessels of other viscera. The mitral valve was slightly myxoid. Intramyocardial arteriolar involvement has not been previously described in PXE...
August 2000: Human Pathology
J N Trochu, F Kyndt, J J Schott, J P Gueffet, V Probst, B Bénichou, H Le Marec
OBJECTIVES: The purpose of this study was to describe the phenotypic characteristics of an inherited myxomatous valvular dystrophy mapped to Xq28. BACKGROUND: Myxomatous valve dystrophies are a frequent cause of valvular diseases, the most common being idiopathic mitral valve prolapse. They form a group of heterogeneous diseases difficult to subclassify. The first mapping of the gene for a myxoid valvular dystrophy to Xq28 allowed investigation of the phenotype of affected members in a large family and characterization of the disease...
June 2000: Journal of the American College of Cardiology
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