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Mitral valve prolapse: from syndrome to disease.

Mitral valve prolapse (MVP) is due to a heterogeneous group of conditions that may affect the mitral valve or the mitral valve apparatus. Although MVP may progress later in life to frank mitral insufficiency requiring mitral valve repair or may predispose to bacterial endocarditis, in most cases it is a benign, idiopathic condition without serious consequences. However, many investigators have documented that MVP is often associated with a constellation of signs and symptoms, which appear to constitute a distinct syndrome. These associated findings include autonomic dysfunction, frequent complaints of chest pain, palpitations, orthostasis, fatigue, dyspnea on exertion and anxiety. Although the risk of significant myocardial dysfunction or bacterial endocarditis appears to be related to patient sex, age and the severity of valvular prolapse and insufficiency, there appears to be little or no relations between the extent of prolapse and the degree of autonomic dysfunction or the severity of symptoms of chest pain, palpitations, dyspnea on exertion and anxiety. The development of uniform diagnostic standards for mental disorders has helped to make it possible to identify several related entities, including generalized anxiety disorder, panic disorder and agoraphobia; patients with these disorders frequently somatize their anxiety and complain of many symptoms which may be seen in patients with MVP. Although several studies have reported an increased frequency of MVP in patients with anxiety disorders, recent studies suggest that the conditions are not linked. Iatrogenic cardiac neurosis is common in both groups of patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Anxiety↗

Case report: outer sheath rupture may precede complete chordal rupture in fibrotic mitral valve disease.

Rupture mechanics of mitral valve chordae have been difficult to elucidate because most surgical repairs and pathological examinations are performed after the rupture. In an excised anterior leaflet from a fibrotic mitral valve, chordae were observed in an initial phase of rupture. Microscopic sections showed that thinned, nearly ruptured chordal segments were actually chordal cores, containing highly aligned collagen fibers. The outer sheath of elastic fibers, disorganized circumferentially oriented collagen fibers, and endothelial cells that normally surrounds the collagen core apparently had retracted to the extreme ends of the thinned segment, resulting in a bulbous shape, as noted in the chordal rupture literature. In conclusion, these new observations lead us to propose that the rupture of mitral valve chordae is not spontaneous, but may occur over time. The failure of the outer sheath may represent the first phase in a slow, two-part process leading to eventual chordal rupture.

Chordae Tendineae↗

Mitral valve prolapse: an atypical variation of the anatomy.

BACKGROUND: The mitral valve apparatus is a complex structure composed of an annulus, mitral leaflets, chordae tendinae, papillary muscles and left ventricular, atrial and aortic walls. Deficiency or degeneration of one or more of these structures may result in dysfunction of the valvular apparatus leading to mitral valve prolapse during systole. OBJECTIVE: To review the chordal attachment of surgically resected mitral valve tissue from cases of mitral valve incompetence. METHODS: The clinical and morphological features of 135 surgically resected posterior mitral valve leaflet specimens were reviewed for mitral valve prolapse from January 1999 to June 1999. Four mitral leaflets removed either surgically or at autopsy served as controls. RESULTS: Excised posterior mitral leaflets from two patients had segments containing free margin and rough zone chordae that lacked attachment to larger 'strut' or 'stem' chordae or to either papillary muscle. These chordae were observed in otherwise normal mitral valves with adjacent areas that had normal chordal arrangements. Such chordae of the posterior leaflet were termed 'atypical chordae tendinae'. There was no good gross or histological evidence of chordal rupture, and this atypical arrangement appears to be congenital in origin. Grossly and histologically, these atypical variants otherwise exhibited characteristics similar to those of leaflet tissue from typical cases of mitral valve prolapse, with shiny, grey-white and "edematous" tissue that contained increased mucopolysaccharides in the zona spongiosa and chordae tendinae. CONCLUSIONS: This review found atypical attachment of mitral valve chordae tendinae such that isolated segments of the posterior leaflet congenitally lack chordal support, likely leading to mitral valve prolapse. Review of the literature suggests that this is a heretofore unreported cause of mitral valve incompetence.

Adult↗

Mitral valve repair.

Diseases that affect the mitral valve include mitral regurgitation, mitral stenosis, rheumatic heart disease, and cardiomyopathy. The results of diagnostic procedures are used to identify and confirm mitral valve disease, evaluate the patient's anatomy, and determine the severity of the disease. After the patient is prepared for surgery, the surgeon performs an intraoperative transesophageal echocardiogram and the patient is placed on cardiopulmonary bypass. A repair procedure (eg, annuloplasty, slidingplasty, chordal repair/transfer/replacement, valve replacement) is performed depending on the patient's specific anatomical abnormalities.

Heart Valve Diseases↗

Percutaneous mitral valve repair procedures.

Percutaneous mitral valve procedures are under investigation as a novel method to treat mitral valve insufficiency with a catheter-based, closed heart, non-surgical approach. The technical opportunities, the expectations for the future and the implications for the conventional surgery are discussed.

Cardiac Catheterization↗

Technique for repair and replacement of the mitral valve.

Whenever possible, the patient's own mitral valve mechanism should be preserved. Successful mitral valve repair offers excellent benefits in terms of hemodynamic function, clinical improvement, and longevity. Open mitral commissurotomy or valvuloplasty for localized defects or ruptured chordae tendineae constitutes our best reparative efforts. Today, mitral valve replacement can be accomplished with less than a 5 per cent operative mortality, but should be reserved for patients who are not in desperate terminal condition. In our experience, aggressive tactics undertaken at the endstage of the disease have had little or no long-term success. At the Cleveland Clinic, isolated mitral valve repair or replacement is performed under normothermic cardiopulmonary bypass and anoxic arrest. Generally, the valve is exposed through an atriotomy behind the interatrial groove. Valvular replacement is accomplished by interrupted suture technique, seating the prosthesis at the level of the annulus or below it. Risk is influenced mainly by the chronicity of the valve dysfunction. Patients who have not yet reached a Functional Class IV status or sustained massive cardiomegaly and low cardiac output fare better in both early and late follow-up periods.

Heart Valve Prosthesis↗

Prosthetic mitral valve replacement: late complications after native valve preservation.

Preservation of the mitral valve leaflet and tensor apparatus during valve replacement is believed to maintain left ventricular performance. The routine use of this technique may lead to left ventricular outflow or inflow obstruction as illustrated in the present report. We recommend mobilization or excision of the anterior mitral valve leaflet and preservation of the posterior leaflet if replacement of the valve is contemplated for incompetence.

Aged↗

Increased NADPH-diaphorase activity in canine myxomatous mitral valve leaflets.

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.

Animals↗

Mitral valve prolapse. Comparison between valvular repair and replacement in severe mitral regurgitation.

BACKGROUND: The aim of this study was to analyse long term results of mitral valve repair of degenerative mitral regurgitation compared to valve replacement. METHODS: A hundred-twenty-five consecutive patients with severe mitral valve insufficiency who underwent cardiac surgery from January 1987 to December 1995 were included in the study. Mean age was 55+/-16 years (77 males, 48 females). Mitral repair was performed in 62 patients and mitral valve was replaced in 63 patients. Mean follow-up was 5 years. The repair procedures were based on quadrangular resection of the posterior leaflet, chordal replacement and transposition. Annuloplasty was performed in 100% of cases. The technique of valve replacement was conventional with complete excision of the valve in the majority of cases. RESULTS: Operative mortality following valve repair was 1.6%, no death occurred in the prosthesic group. In the repair group overall survival and re-operation rate were respectively 95.2% and 6.5%, while in the replacement group were 93.7% and 7.9%. No endocarditis and thromboembolic accidents were observed following valvuloplasty, while in the prostheses 6.3% of patients had endocarditis and 1.6% had a thromboembolic event. Mild or moderate left ventricular dysfunction was present in 5 patients after valvuloplasty and in 9 patients with prostheses. CONCLUSIONS: Considering these results we conclude that, in patients with severe degenerative mitral insufficiency, mitral valve repair is warranted whenever it is possible. The advantages given by maintaining the native valve suggest that surgery should be considered in asymptomatic patients before the occurrence of the left ventricular dysfunction.

Aged↗

Comparison of accuracy of transesophageal versus transthoracic echocardiography for the detection of mitral valve prolapse with ruptured chordae tendineae (flail mitral leaflet).

The accuracy of transesophageal echocardiography was compared with that of transthoracic echocardiography in the detection of ruptured chordae tendineae (flail mitral leaflet) in 27 patients with mitral valve prolapse (MVP) who underwent valve repair or replacement for mitral regurgitation. Confirmation of the presence of ruptured chordae resulting in a flail leaflet was available at surgery in all cases. The echocardiographic studies were read blindly by 2 independent observers with any differences resolved by a third. Mean (+/- standard deviation) age was 63 +/- 13 years. Men (n = 20) outnumbered women (n = 7) (p less than 0.02), and tended to be younger (p = 0.06). Flail leaflets were identified in 20 of 27 patients. In 1 patient, both leaflets were involved and in the remaining 19 patients posterior leaflets (15 patients) were more frequently affected than anterior leaflets (4 patients). Transesophageal echocardiography correctly identified all 20 patients with flail leaflets, but 1 false positive study occurred among the 7 patients without a flail leaflet. In contrast, transthoracic echocardiography identified only 12 of 20 patients with flail leaflets, with no false positive studies. Transesophageal echocardiography was more accurate, correctly classifying 26 of 27 (96%) cases versus 19 of 27 (70%) by the transthoracic approach (p less than 0.01). This study suggests a higher incidence of chordal rupture to the posterior leaflet in patients with MVP and demonstrates improved accuracy of transesophageal over transthoracic echocardiography in the detection of flail leaflets.

Aged↗

[Progressive subaortic stenosis caused by accessory mitral valve tissue].

Accessory mitral valve tissue is a rare cause of subaortic obstruction. The reported case correspond to a two days old patient diagnosed by 2D-Echocardiography. Serial doppler flow analysis showed progressive left ventricular outflow obstruction. A cardiac catheterization at the age of 9 months confirmed the obstruction without defining the cause. Five months later, transesophageal echocardiography clearly defined the intraventricular connections of the accessory mitral tissue. At the age of 18 months the patient suffered from cardiac failure and underwent surgery for removing the accessory tissue, assisted by intraoperative transesophageal echocardiography pre and post by-pass. Follow-up echocardiography at 25 months showed no left ventricle outflow obstruction and the patient is now asymptomatic.

Aortic Valve Stenosis↗

Comparison of cleft and papillary muscle position in cleft mitral valve and atrioventricular septal defect.

Differentiation of cleft mitral valve from atrioventricular septal defect (AVSD) is surgically important because of different conduction pathways. The purpose of this study was to find echocardiographic markers for differentiating cleft mitral valve from AVSD. We examined 11 children with cleft mitral valve, 11 children with AVSD, and 11 normal children. We defined a fixed reference point (0 degrees of arc) at the medial junction of the right ventricular free wall with the posteroinferior ventricular septum. Left ventricular papillary muscle and cleft position was measured clockwise in degrees of arc from 0 degrees around a point representing the center of the left ventricle in the parasternal short-axis views. Mural leaflet size was expressed by the arc between the bases of the papillary muscles. Papillary muscle position in cleft mitral valve was similar to that in normals (anterolateral ventricular papillary muscle:cleft mitral valve 194 +/- 10 degrees, normals 191 +/- 8 degrees; posteromedial ventricular papillary muscle:cleft mitral valve 329 +/- 10 degrees, normals 329 +/- 14 degrees). In AVSD, both left ventricular papillary muscles originated closer to each other and were rotated counterclockwise (anterolateral papillary muscle: 182 +/- 7 degrees; posteromedial papillary muscle: 287 degrees +/- 17 degrees). The mural leaflet size was similar in cleft mitral valve and in normal children. In AVSD, the mural leaflet was significantly smaller than in cleft mitral valve and in normals. The cleft position did not permit distinction between cleft mitral valve and AVSD. In conclusion, echocardiographic measurements of papillary muscle position and mural leaflet size permit differentiation of cleft mitral valve from AVSD.

Child↗

[Mitral valve prolapse].

It is thought that mitral valve prolapse is the most commonly diagnosed valvular heart disease and affects 3 to 5% of the community. Mitral valve prolapse is commonly a benign disease and the prognosis is good. But it is understood that mitral valve prolapse causes sudden death rarely. As etiologies of their sudden deaths ventricular arrhythmia, mitral valve regurgitation et al. are known. Mitral valve prolapse is brought as the reason of sudden deaths in athletes not a little. Therefore, it is important to follow up them by exercise tolerance test, Holter ECG, ultrasoniccardiography, and chest auscultation et al. and the prescription of beta-blockade et al. and the insertion of implantable cardioverter defibrillator are considered. Moreover, it is important to decide right or wrong of exercise practice.

Death, Sudden, Cardiac↗

Stentless mitral valve replacement using the quattro valve.

This study analyzed early clinical results after stentless mitral valve (Quadrileaflet Mitral Valve (QMV)/Quattro) implantation. A total of 28 patients have received a Quattro valve since August 1997. Patient age was 69+/-8 years; the underlying disease was mitral incompetence (14) and stenosis (13). Preoperative New York Heart Association functional class was 3.2+/-0.4 and cardiac index was 1.8+/-0.6. Mean cross-clamp duration was 58+/-19 minutes. Twenty patients received a 28-mm prosthesis, and eight patients received a 30-mm prosthesis. Four patients had myocardial revascularization, two had tricuspid valve repair, and six had radiofrequency ablation therapy to restore sinus rhythm. Mortality (1) was nonvalve related. Reoperation was necessary in two patients for posterior paravalvular leakage (1) and for functional stenosis (1). Echocardiography showed satisfactory hemodynamic function. By stentless mitral valve implantation the annuloventricular continuity is preserved to stabilize left ventricular function. The Quattro valve resembles native mitral valve function and is well suited for mitral valve replacement.

Aged↗

Mitral valve prolapse and body habitus in children.

Mitral valve prolapse has generally been associated in adults with a thin body habitus. However, prior studies used biased samples or limited anthropometric measures. In addition, no information has been available on the subjective assessment of body habitus and diagnosis of mitral valve prolapse, especially in children. We conducted a cross-sectional study on 813 children with uniform assessment of anthropometric measures and mitral valve prolapse. Consistent with research conducted on adults, those subjects with mitral valve prolapse were lighter, thinner, and had, on average, lower values for several, quantifiable anthropometric parameters with the exception of height. However, the subjective assessment showed that while the assessment did not differ by diagnosis, those subjects with mitral valve prolapse were never described as fat. These data support an association between mitral valve prolapse and slender body habitus and extends it to children, thus underscoring the clinical importance that a thin body habitus may be a marker for mitral valve prolapse throughout the age span. This association may partly explain the observed genetic distribution of mitral valve prolapse.

Body Constitution↗