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Mitral valve prolapse.

Mitral valve prolapse is a condition that is being recognized with increased frequency. It is not known whether its incidence is increasing, or whether we are better able to diagnose it today. In the idiopathic or familial variety, the mitral valve pathology is almost always that of myxomatous degeneration. Some authors have suggested the presence of a cardiomyopathy because of significant left ventricular dysfunction in many cases. Idiopathic prolapse occurs predominantly in females, often at a young age, and may be associated with chest pain, dyspnea, fatigue, presyncope, syncope, and/or sudden death. The clinical findings are variable and typically consist of a nonejection click and/or late systolic murmur, heard best at the cardiac apex. Diagnosis can be confirmed by echocardiography and/or ventricular cineangiography, the latter permitting accurate recognition of the anatomy of the prolapsed leaflets. The complications of infective endocarditis, severe mitral insufficiency, and life-threatening ventricular arrhythmias represent the major problems of management. It is important to distinguish the idiopathic form of mitral valve prolapse from that due to coronary artery disease and to realize that mitral valve prolapse may occur in Marfan's syndrome, Turner's syndrome, or in association with secundum atrial septal defect or ruptured chordae tendineae. Typical clicks and/or murmurs have also been described in patients with a history of rheumatic fever and in hypertrophic cardiomyopathy. Although much descriptive knowledge has accumulated over the past 15 years, many unanswered questions remain regarding the idiopathic type of prolapse. What is the nature and cause(s) of myxomatous degeneration? What is the relation of the valve pathology to the left ventricular dysfunction? What is the relation of both of these factors to disabling chest pain, electrocardiographic changes, and life-threatening arrhythmias? Hopefully, answers to these and other important questions regarding mitral valve prolapse will be forthcoming.

Electrocardiography

Mitral valve prolapse.

Mitral valve prolapse is a relatively common condition in the general population. The syndrome appears more common in females, and is often associated with a family history. Patients may be asymptomatic or may present with a variety of symptoms ranging from mild chest aches and anxiety to severe angina-like chest pain, palpitations and dizziness. The common auscultatory features include mid-systolic clicks and a late systolic murmur, either alone or in combination. The wide spectrum of symptoms and signs may be explained by ventriculovalvular disproportion, where either the ventricle is too small for the valve, or the valve is too large for the ventricle. The long-term prognosis is very good; severe mitral regurgitation can occasionally develop, but both sudden death and bacterial endocarditis are rare. No treatment is required for asymptomatic patients, beyond antibiotic cover for dental procedures and surgery.

Adolescent

Conservative management of the prolapsed mitral valve.

Prolapsed leaflet is the result of ruptured chordae, elongated chordae, or ruptured papillary muscle. Various techniques adapted to each of these lesions were developed, and repair of 213 prolapsed mitral valves was performed between 1969 and 1977. There were 109 patients with ruptured chordae treated by quadrangular resection of the prolapsed leaflet; 103 patients with elongated chordae were treated by either a "sliding plasty" of the papillary muscle or a "shortening plasty" of the chordae; and 1 patient with ruptured papillary muscle was treated by reimplantation. The great majority of patients had an associated annular dilatation or deformation requiring the use of a Carpentier ring to remodel the annulus and reinforce the repair. The operative mortality was 4% and the late mortality, 3%. There were 6 reoperations, 3 of which occurred within 1 year. Thromboembolic complications occurred in only 1 patient (0.5%), even though the majority of patients received no anticoagulation treatment. Actuarial curves demonstrated a 91% survival at 8 years.

Adult

Mitral valve prolapse due to coronary artery disease.

Controversy exists concerning the etiologic role of coronary artery disease in the prolapsing mitral valve leaflet syndrome. A 35 year old man with progressive coronary artery disease is described. Auscultation before and after his first myocardial infarction revealed only a fourth heart sound; subsequently left ventricular cineangiography demonstrated normal anatomy and function of the mitral valve, despite extensive wall motion abnormalities. Six months later he experienced another myocardial infarction after which the typical mid-systolic click, late systolic murmur of mitral valve prolapse developed. A second left ventricular cineangiogram at this time revealed mid-systolic mitral valve prolapse and mitral regurgitation. This patient's course indicates that myocardial damage from coronary artery disease can cause mitral valve prolapse in patients with preexisting redundant mitral valve tissue.

Adult

The echocardiographic association of mitral valve prolapse and mitral anulus calcification.

Mitral anulus calcification (MAC) and mitral valve prolapse (MVP) are frequently diagnosed conditions. We studied two patients with mild or moderate mitral regurgitation who demonstrated both MAC and MVP on angiography and echocardiography. M-mode echocardiography is probably the definitive test for confirming the presence of MVP. Echocardiography is moderately sensitive in the diagnosis of cardiac calcification, such as MAC, but M-mode echocardiography may not detect the MAC in the majority of patients with both MVP and MAC demonstrated by angiography.

Calcinosis

Mid-systolic click and mitral valve prolapse following mitral commissurotomy.

Five patients with rheumatic mitral stenosis were observed to have mid-systolic clicks with murmurs of mitral regurgitation at various intervals after mitral commissurotomy. In two patients echocardiography showed an unusually rapid posterior deflection of the mitral valve coinciding exactly with a systolic nonejection click. It is speculated that the shortened, fused chordae tendineae, compromised by mitral commissurotomy, rigidly hold the valve leaflets fixed at the onset of systole. During systole, ventricular conformational changes, in the face of marginal coaptation of thickened and fibrotic mitral leaflets, allow the mitral valve to be forced abruptly towards the left atrium with great velocity. This is manifested by a loud systolic click and, in some patients, a near vertical posterior systolic deflection of the mitral valve on the echocardiogram. The systolic click may occur without echocardiographic or angiographic evidence of mitral valve prolapse. Unusually loud mid-systolic clicks can be heard in patients with rheumatic heart disease after mitral commissurotomy and may be accompanied by a distinctive echocardiographic appearance of the mitral valve.

Adult

[Echocardiographic abnormalities in the mitral valve prolapse syndrome].

Eight patients with prolapsed mitral valve syndrome, with a mid - or - end - diastolic click or murmur, underwent echocardiographic examination using ultrasound. Examination of the displacement of the valves by the "time motion" method showed all cases to have an abnormal recoil; in 6 cases this occurred in mid or late systole, and in two from the beginning of systole. The recorded amplitude of the pathological displacement, which gives the systolic tracings a domed appearance, and the consistancy with which it could be recorded (irrespective of the incidence of the ultrasonic waves), seem to afford a method for evaluating the degree of prolapse which, in most cases, involves both the valves. The authors compare their results with those which have already appeared in the literature, and emphasise the very real contribution which echocardiography can make in the diagnosis and assessment of the prolapsed mitral valve syndrome.

Adolescent

Refractory ventricular arrhythmias in a patient with mitral valve prolapse. Successful control with mitral valve replacement.

A 35-year-old woman with proven mitral valve prolapse developed life threatening ventricular arrhythmias which were refractory to medical therapy. She had one episode of "cardiac arrest" presumably due to ventricular tachycardia or possibly ventricular fibrillation, and was successfully resuscitated with closed chest compression. Mitral valve replacement resulted in dramatic control of the ventricular arrhythmias. Over a period of three years following the operation, she has been able to resume an active life with occasional ventricular premature beats and no further episodes of ventricular tachyarrhythmias.

Adult

Altered collagen composition in a prolapsing mitral valve with ruptured chordae tendineae.

A patient presented with mitral valve prolapse, ruptured chordae tendineae, severe mitral regurgitation and congestive heart failure. Pathologic studies demonstrated myxomatous changes of both mitral leaflets. Collagen analysis of the valve and of the chordae tendineae revealed the presence of type I collagen in normal quantities and a striking absence of type III and AB collagens. The altered collagen in this patient's mitral valve probably contributed to the development of his mitral valve disorder.

Chordae Tendineae

Mitral valve prolapse in rheumatic mitral stenosis.

Four adult women with histories of rheumatic fever and clinical findings of mitral stenosis and regurgitation had echocardiograms demonstrating moderately severe mitral stenosis (EF slope less than 20 mm/sec, mean left atrial size 3.0 cm/m2, mean anterior mitral leaflet excursion 25 mm) as well as typical mitral valve prolapse. Three patients underwent cardiac catheterization which confirmed the presence of mitral stenosis, as well as systolic prolapse and excessive scalloping of the mitral valve with no visible mitral calcium and no coronary artery disease. One patient had associated mild aortic stenosis and regurgitation. Two patients underwent mitral valve surgery which revealed anterior and posterior commissural fusion consistent with rheumatic disease and intact chordal apparatus. Both leaflets were large and the anterior leaflets were redundant. There were no vegetations. Pathology revealed myxomatous degeneration of the valve leaflets. In the absence of heavy calcification and thickening, the presence of mitral stenosis with commisural fusion does not exclude the possibility of a redundant mitral valve. When these entities coexist, systolic clicks may be absent.

Aged

Psychiatric implications of the mitral valve prolapse syndrome (MVPS).

The mitral valve prolapse syndrome (MVPS) is an infrequent outcome of mitral valve prolapse, a common and usually benign structural abnormality. Psychiatric patients often present with symptoms indistinguishable from MVPS, knowledge of which is thus essential for differential diagnosis. Even when prolapse of the mitral valve is detected, the symptoms may be variously determined by both physical and emotional factors. Four illustrative case histories are presented.

Adult

Migraine and the mitral valve prolapse syndrome.

We believe there is a significant association between migraine and the prolapse mitral valve syndrome. Propranolol is the drug of choice in these patients for the treatment and prevention of migraine. Increased platelet aggregability may be the common pathophysiologic mechanism as relates to emboli from the valve and possibly in strokes related to migraine. Recognition of the association of the two syndromes will result in appropriate subacute bacterial endocarditis prophylaxis for patients at risk as well as prevention of improper medication to those patients with migraine who are at risk for stroke.

Adolescent

Pseudomonas maltophilia bacteremia associated with a prolapsed mitral valve.

A 53-year-old man had had recurrent episodes of transient visual loss, malaise and a heart murmur. Blood cultures repeatedly grew Pseudomonas maltophilia, a frequent opportunistic pathogen, and echocardiogram documented mitral-valve prolapse. The risk of bacterial endocarditis is stressed.

Heart Valve Diseases

Echocardiographically determined mitral valve prolapse in male patients.

Mitral valve prolapse syndrome is a common clinical entity that may occur as an isolated condition or in association with other cardiovascular diseases. In this retrospective study, 41% (23/56) of the male patients with echocardiographically diagnosed mitral valve prolapse had an additional form of cardiovascular disease.

Adolescent

[Variable aspects in mitral valve prolapse. Echographic and phonocardiographic studies of 68 cases (author's transl)].

Previous parallel echocardiographic-angiocardiographic studies have proven specificity and sensitivity of ultrasonography in diagnosing prolapse of the mitral valve. Echocardiograms and phonocardiograms from 68 patients with mitral valve prolapse were studied. Echocardiograms suggestive of mitral valve prolapse were obtained in 66 patients. The typical abnormality consisted of an abrupt posterior displacement of the mitral valve leaflets, either late systolic or pansystolic. Moreover a new pattern not previously described was observed, i.e. an abrupt isolated early systolic posterior motion of the mitral leaflets ("early prolapse"). Phonocardiography, on the other hand, showed a variable spectrum of acoustic findings: non-ejection systolic click and/or late systolic murmur, pansytolic murmur, early systolic click,ejectioarly systolic click, ejection murmur, in variable association, and finally no abnormal sound at all ("silent prolapse"). The authors conclude that mitral valve prolapse may be suspected, whatever the auscultatory finding. Echocardiography provides a useful, non invasive method for detecting those forms which also present an uncommon auscultatory pattern.

Adolescent

[Echocardiographic evaluation of left ventricular function in mitral valve prolapse].

Echocardiographic assessment of left ventricle functions was performed in 52 cases with idiopathic prolapse of mitral valve, confirmed on the base of the simultaneous presence of the well known auscultatory, phonocardiographic and echocardiographic criteria. Mitral prolapse was established not to be a homogenous group as regards left ventricle function. The majority of the cases (80.7%) had normal EchoC-indices for the pump and total and local contraction function of left ventricle. The cases without (25%) or with light stage (55.7%) mitral regurgitation are included here. In the presence of hemodynamic significant mitral insufficiency -- 13.5% of the cases. EchoC-manifestations for volume left-ventricle loading were found -- enlarged telediastolic dimension and volume of the left ventricle cavity, light hypertrophy and hypercontractility of the interventricular septum and/or the unattached posterior wall of left ventricle, enlarged left ventricle, increased velocity of EF-segment of the anterior mitral cusp. As a rule, a moderate mitral regurgitation is concerned in those cases. In a small number of cases with mitral prolapse (5.8%) EchoC-manifestations for a light to moderate pump and general and/or local contraction function of left ventricle was established without clinical data for a stasis in left ventricle cardiac insufficiency. Grounds exist to admit that in the last cases, a rather not severe local or more diffuse lesion of the left ventricle myocardium is concerned, its relationship with the mitral valve prolapse remaining obscure.

Adult