myxoid degeneration of the mitral valve

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...

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...

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...

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...

In this study, 872 heart valves surgically excised from 810 patients during a period of 5 years (1994 through 1998) were examined pathologically. There was a predominance of aortic (506 patients) versus mitral valves (246 pts.). While aortic valves came more often from men (364) than from women (142), in mitral valves the M:F ratio is 82/164. Isolated calcific aortic stenosis appeared as the most frequent valvular disease (418 pts.), with predominance of its sclerotic-senile type (238 pts.). Mitral stenosis (185 pts...

A right thoracotomy is a well-known alternative for midsternotomy to have access to the left atrium. The Port-Access (Heartport, Inc, Redwood City, CA) approach is an invaluable option to avoid cracking of ribs and cartilage. EndoCPB (Heartport, Inc) and Endo-Aortic Clamp (Heartport, Inc) allows installation of the extracorporeal circulation and cardiac arrest from the groin. Videoassistance and shafted instruments help the surgeon to perform the surgery through a 5 x 2-cm port and fulfill the main goals of minimally invasive cardiac surgery, comfort, cosmesis, and fast rehabilitation...

The aim of this study was to report the prevalence of degenerative and early inflammatory lesions and to discuss some histological aspects of normal and pathological mitral valve leaflets in healthy, normally slaughtered pigs. Mitral valves were collected from 112, 6 month-old, pigs reared under different conditions. Histological examination revealed the presence of myxoid degeneration in 29.5% of the valves with no significantly different prevalence according to rearing conditions; fibrosis was present in 12...

Cor triatriatum is a rare cardiac anomaly which can be congenital or acquired in origin. Congenital cor triatriatum is due to an alteration of the common pulmonary vein resorption and therefore the left atrium is divided into two chambers, a proximal one, in communication with the pulmonary veins, and a distal one, in communication with the mitral valve orifice. The diagnosis is usually made at birth, but in rare cases, when the communication between the two chambers is wide and the patient is asymptomatic, the lesions may be diagnosed incidentally during a routine echocardiographic examination...

Myxoid heart disease is frequently encountered in the general population. It corresponds to an etiologically heterogeneous group of diseases, idiopathic mitral valve prolapse (IMVP) being the most common form. A rarely observed form of myxoid heart disease, X-linked myxomatous valvular dystrophy (XMVD), is inherited in an X-linked fashion and is characterized by multivalvular myxomatous degeneration; however, the histopathological features of the mitral valve do not differ significantly from the severe form of IMVP...

Ventricular electrical instability and sudden death in mitral valve prolapse (MVP) have been related to mitral valve regurgitation and left ventricular dysfunction, autonomic nervous system abnormalities, or underlying cardiomyopathy. The aim of the present study was to assess the frequency, nature and pathophysiologic significance of histologic myocardial abnormalities in young patients with apparently isolated MVP and sudden cardiac death. Among 163 cases of sudden cardiovascular death in young people, MVP was the only cardiac pathology found at postmortem gross examination in 17 (10%) (12 females and 5 males) aged 14 to 35 years, mean 24...

Pig endocardiosis is a pathological process affecting cardiac valves that is characterised by the accumulation of glycosaminoglycans (GAG) in the extracellular matrix. To investigate the involvement of GAG in the condition, the morphology of the mitral valves from 23 affected pigs and seven normal controls was studied and qualitative and quantitative biochemical analyses of GAG were made. Gross and histopathological lesions were characterised by valve enlargement, collagen disorganisation and myxoid degeneration...

Indications for specific valve types, whether they be mechanical or bioprosthetic, have been rigorously defined in recent years. Candidates for bioprosthetic valves would be: older patients, patients who are noncompliant or have contraindications to anticoagulation, patients in normal sinus rhythm, and young females desirous of future pregnancy. Recent data suggest that patient age and presence of coronary artery disease may significantly alter the indications for mechanical or tissue valves. There is mounting evidence that patients without coronary disease in their 60s and 70s who require valve replacement are better served with a mechanical prosthesis...

Two degenerations of the mitral valve apparatus, namely myxoid degeneration of the mitral cusps and necrosis with subsequent calcification of the annulus fibrosus, are extensively reviewed and discussed. Both lesions are mainly noticed in elderly patients, with the possible exception of systemic dystrophies of the connective tissue associated with metabolic disorders. The clinicopathological correlations of several cases are reported, with special emphasis on modern diagnostic procedures and their wide anatomic spectrum...

Idiopathic mitral valve prolapse due to severe myxoid degeneration may be associated with aortic and/or tricuspid valve disease of the same aetiology. These localisations, which usually give rise few symptoms, can be detected by echocardiography. Sometimes, however, they may give rise to serious valvular regurgitation requiring surgical correction at each diseased valve. The authors report two cases of "floppy" mitral valve prolapse, the first requiring mitral and tricuspid, and the second, mitral and aortic, valve replacement...

Current knowledge concerning idiopathic prolapse of the mitral valve is illustrated. The histopathological cause is myxoid degeneration of the mitral cusps, which sometimes extends to the tendinous cords, the valve implant ring, and the apex of the papillary muscles. Primary damage to these structures, whose intactness is essential for correct closure of the ostium, causes protrusion of the ventricular cusps into the left atrium during ventricular systole (i.e. prolapse). The reason for this degeneration is not known...

A clinico-pathologic study was performed in 25 patients undergoing aortic valve replacement because of regurgitation, caused by myxoid degeneration of the valve leaflets. Associated cardiac anomalies were floppy mitral valve (2 cases), floppy mitral valve and idiopathic hypertrophic subaortic stenosis (1), left atrial myxoma (1), and aortic coarctation at the isthmus (1). Three patients died (2 immediately and 1 on the 30th postoperative day). Pathological studies of the explanted valves showed deformities characterized by redundant thin leaflets which appeared soft and gelatinous...

Clinical and pathologic data were reviewed in 55 patients who had valve replacement for pure aortic regurgitation (AR) during a 6-year period. The clinical histories established the cause for AR in 34 cases: 11 rheumatic, 13 infective endocarditis, 4 congenital, 4 associated with aortic aneurysms and 2 the Marfan syndrome. In the valves from the other 21 patients, 13 had myxoid degeneration, defined as significant disruption of the valve fibrosa and its replacement by acid mucopolysaccharides and cystic change...

Five cases of degenerative mitral incompetence due to rupture of the chordae tendinae in patients over 70 years of age were reviewed to determine the clinical features of this pathology which is not rare in elderly patients. Chordal rupture usually involves the posterior leaflet and is a sign of generalised disease of the mitral apparatus of two main types: myxoid infiltration or pellucid degeneration. Although the clinical syndrome of rupture is rare (10 p. 100), the mechanism of the mitral regurgitation can be identified by 2D echocardiography with a sensitivity of 92 p...

This study concerns 51 cases of mitral valve prolapse demonstrated on bidimensional 4 cavities sonography according to Gilbert's criteria and aims to determine the frequency of the associated valvular involvement, anatomically with sonography and functionally with the Doppler test. Sonography demonstrates a tricuspid valve prolapse in 79 p. cent of the cases and an aortic valve prolapse in 10 p. cent. The Doppler test demonstrates a tricuspid leakage in 52 p. cent of the cases, a pulmonary leakage in 62 p. cent, and an aortic leakage in 18 p...

Severe aortic and mitral valvular insufficiency developed in a 52-year-old woman with systemic lupus erythematosis after previous treatment with corticosteroids, splenectomy, and cyclophosphamide for immune thrombocytopenia and hemolytic anemia. After aggressive management of the thrombocytopenia, simultaneous prosthetic replacement of both valves was satisfactorily performed; pathologic examination revealed myxoid degeneration. The patient was doing well with excellent valve function 26 months after the operation...