Value of echocardiographic findings in predicting cardiovascular complications in infective endocarditis.

Echocardiography allows the detection of vegetations and estimation of valvular dysfunction in patients with infective endocarditis. The value of echocardiographic findings in predicting cardiac and other vascular complications in infective endocarditis is not well understood. Identification of high-risk patients and early surgery may improve their prognosis. The authors reviewed echocardiographic findings and related them to the development of congestive heart failure, systemic embolism, and the need for surgery or the risk of death without surgery in patients with infective endocarditis. There were 125 episodes of endocarditis in 114 patients (84 episodes [67%] in men) with a mean age +/- standard deviation of 37 +/- 7 years. Vegetations were detected by echocardiography on at least 1 valve in 87 episodes (70%); on the mitral valve in 36 episodes (29%); on the aortic valve in 21 episodes (17%); and on the tricuspid valve in 45 episodes (36%). Severe aortic regurgitation was present in 9 episodes (7%) and severe mitral regurgitation in 4 instances (3%). In 12 of 21 episodes (57%) of vegetations on the aortic valve compared with 15 of 104 patients (14%) without vegetations on the aortic valve (p < 0.001), and in 8 of 9 instances (89%) of severe aortic regurgitation compared with 19 of 116 episodes (16%) without severe aortic regurgitation (p<0.00001), the patients developed congestive heart failure. In 18 of 55 episodes (33%) of vegetations on the aortic/mitral valve compared with 17 of 70 episodes (25%) without vegetations on the aortic valve/mitral valve (p = NS), the patients developed systemic embolism. In 13 of 21 episodes (62%) of vegetations on the aortic valve compared with 19 of 104 episodes (19%) without vegetations on the aortic valve (p < 0.001), and in 8 of 9 episodes (89%) of severe aortic regurgitation compared with 24 of 116 episodes (21%) without severe aortic regurgitation (p < 0.00001), the patients either had surgery or died without surgery. Echocardiographic findings do not reliably predict the risk of systemic embolism in patients with infective endocarditis. Vegetations on the aortic valve and severe aortic regurgitation detected by echocardiography predict a high risk of developing congestive heart failure, and for the combined outcome of requiring surgery, or dying without surgery in infective endocarditis. Early surgery may improve the outlook for survival of these patients.     

Mitral valve prolapse: definition and implications in athletes

Mitral valve prolapse is probably the most common cardiac valve disorder, affecting approximately 5% of the population. Although it is genetically determined, its clinical manifestations do not usually become evident before adulthood. In the setting of a cardiology referral center, a mitral valve prolapse syndrome, consisting of nonspecific symptoms, repolarization changes on the electrocardiogram and arrhythmias, has been identified. However, doubt has recently been expressed about the existence of such a syndrome. The prognosis of mitral valve prolapse is generally favorable but infrequent complications do occur and include transient ischemic attacks, progression of mitral regurgitation with or without ruptured chordae tendineae, infective endocarditis and sudden death. The symptoms and the complications are not usually related to physical activity. A permissive attitude toward participation of patients with mitral valve prolapse in competitive athletics is probably warranted; however, it would appear reasonable to disqualify athletes with mitral valve prolapse in the following circumstances: history of syncope; disabling chest pain; complex ventricular arrhythmias, particularly if induced or worsened by exercise; significant mitral regurgitation; prolonged QT interval; Marfan's syndrome; and family history of sudden death.     

Mitral valve dysfunction in the Marfan syndrome: Clinical and echocardiographic study of prevalence and natural history

Although mitral regurgitation and fibromyxomatous thickening of the mitral leaflets have long been recognized as such, mitral valve prolapse has only recently been added as one of the pleiotropic features of the Marfan syndrome, the prevalence, age of onset, and natural history of mitral valve dysfunction in this condition are uncertain. Therefore, all patients in one clinic who met strict diagnostic criteria for the Marfan syndrome and who had clinical and echocardiographic examinations before age 22 years were reviewed. Of the 166 patients (84 males, aged 11.9 ± 0.6 years [mean ± SEM]; and 82 females, 11.0 ± 0.6 years), 52 percent had auscultatory and 68 percent had echocardiographic evidence of mitral valve dysfunction, generally mitral valve prolapse. Prevalence did not differ between the sexes. Follow-up in 115 patients averaged five examinations over a mean of four years; 17 percent were followed for more than six years. Criteria for progression of mitral valve dysfunction were: (1) on auscultation, the appearance of new systolic clicks or apical systolic murmurs, a mitral regurgitant murmur increased by two grades, or appearance of congestive heart failure not due to aortic regurgitation; and (2) on echocardiography, the new appearance of mitral valve prolapse or abnormally increased left atrial dimension. Nearly half the patients met at least one criterion and one quarter had both auscultatory and echocardiographic evidence of progressive mitral valve dysfunction. Twice as many females demonstrated worse mitral valve function with time. Eight of the 166 patients either died as a result of mitral valve dysfunction or required mitral valve replacement. Severe mitral regurgitation developed in an additional 15 patients. Rupture of chordae tendineae was uncommon. Antibiotic prophylaxis was routine, and no cases of bacterial endocarditis of the mitral valve occurred. These results suggest that mitral valve dysfunction is extremely common in young patients with Marfan syndrome and usually presents as mitral valve prolapse. Serious mitral regurgitation develops in one of every eight patients by the third decade. Thus, the prevalence and natural history of mitral valve prolapse in the Marfan syndrome appear distinct from mitral valve prolapse associated with other conditions, including idiopathic or familial mitral valve prolapse.     

Valvular heart disease and systemic lupus erythematosus: therapeutic implications

Valvular involvement is the most encountered form of heart disease in systemic lupus erythematosus (SLE). Immunoglobulin and complement deposition in the valvular structure will subsequently lead to Libman-Sacks vegetations, valve thickening, and valve regurgitation. Valvular stenosis is rarely seen. Involvement of the mitral valve is most frequently encountered. Valve disease for most patients is mild and asymptomatic, but patients in whom severe mitral regurgitation develops will present with symptoms of congestive heart failure. A heart murmur will be heard in almost all patients with moderate or severe regurgitation. Transesophageal echocardiography is the most sensitive method to detect the valvular involvement. The valvular changes, the hemodynamic status, or the symptomatology have been shown to progress, remain stable, or sometimes improve. Severe regurgitation, infective endocarditis, and thromboembolic events (mostly stroke or transitory ischemic attacks) are complications of valvular involvement in SLE. In treatment of these patients, prophylaxis of infectious endocarditis, selective antiaggregant and anticoagulant medication, and valve replacement are currently offered. The role of corticosteroid treatment is still unclear in the outcome of SLE valvulopathy.     

The pathology of mitral valve prolapse

The gross criteria for diagnosing prolapsing mitral valve are: 1. interchordal hooding of the involved leaflets, 2. hooding or doming of leaflets towards the left atrium, 3. elongation of the involved leaflets resulting in an increase in valve area, 4. dilatation of the valve annulus in patients with severe mitral regurgitation. The posterior leaflet is most frequently affected. The involved leaflets, in general, are thickened, soft, greyish white and have a smooth atrial surface. Chordae tendineae are described as elongated, tortuous and attenuated or thinned. Deviations from normal chordal insertion have recently been observed which possibly appear to represent the underlying abnormality. Microscopic findings include significant thickening of the spongiosa and the fibrosa, changes in dense collagen fibers in the atrialis layer, occasionally, with fibrin platelet deposits. Histochemical characterization of changes in the spongiosa may also be helpful in the diagnosis. Ultrastructurally, there may be changes in collagen and elastic fibers as well as myxoid areas. On comparison of findings in surgically-removed mitral valves with those of control specimens from autopsy patients with no cardiac abnormalities, the length of the anterior and posterior leaflet as well as the annular ring diameter was larger in the valves with prolapse. Two-dimensional echocardiography accurately assessed leaflet length when compared to morphologic measurements, however, the annular diameter during systole or diastole was smaller. In patients with mitral regurgitation requiring surgery, mitral valve prolapse is the most common cause. Annular ring dilatation and chordae tendineae rupture appear to contribute substantially to incurrence of the mitral regurgitation. The heart weight is increased in the majority of patients with symptomatic mitral valve prolapse but normal, however, in those without symptoms. The most frequent complication of mitral valve prolapse is mitral regurgitation with or without congestive heart failure. Patients with redundant leaflets may be at high risk of sudden death. Young women with abnormal resting ECG, prolonged Q-T interval, family history of sudden death or complex ventricular arrhythmias may also be at a greater risk of sudden death. The incidence of infective endocarditis appears higher in those with redundant than in those with nonredundant valves. The incidence of cerebral ischemic events is low.(ABSTRACT TRUNCATED AT 400 WORDS)     

Complications of mitral valve prolapse. Disproportionate occurrence in men and older patients

To determine factors influencing the strength of association between mitral valve prolapse and mitral regurgitation, ruptured chordae tendineae, and infective endocarditis, the prevalence of mitral prolapse in patients with disease was compared with both clinical and population control groups. The prevalence of mitral valve prolapse was 4 percent among population and clinical control groups (eight of 196 and 84 of 2,146, respectively) and was significantly higher (p less than 0.001) in patients with endocarditis (11 of 67, 16 percent), mitral regurgitation (17 of 31, 55 percent, and ruptured chordae (27 of 43, 63 percent). Odds ratios for complications in persons with mitral valve prolapse ranged from 4.6 for endocarditis to 41.4 for ruptured chordae in overall analyses, and from 6.8 for endocarditis to 53.0 for ruptured chordae based on age- and sex-matched case-control triplets (p less than 0.001 for each). All complications occurred disproportionately in men with mitral valve prolapse, in whom odds ratios ranged from 2.5 to 7.4 compared with an additional control group of unselected subjects with mitral valve prolapse. Compared with this control group, patients with mitral valve prolapse and endocarditis were slightly more likely to have a previously known heart murmur (odds ratio 3.2, difference not significant) but significantly more likely to have murmurs at the time of evaluation (odds ratio 8.5, p less than 0.01). Patients with mitral valve prolapse and mitral regurgitation and ruptured chordae tendineae were also significantly older than the unselected subjects with mitral valve prolapse (48 +/- 14 and 55 +/- 16 versus 38 +/- 14 years, p less than 0.005 for both). The concentration of risk of endocarditis in men with mitral valve prolapse and patients with antecedent murmur suggests that antibiotic prophylaxis is warranted in these groups but not in women without a murmur of mitral regurgitation.     

Prognostic significance of valvular regurgitation in patients with infective endocarditis

PURPOSE: Doppler ultrasound is a sensitive modality for detecting and quantitating valvular regurgitation in patients with infective endocarditis. Because valvular regurgitation leads to heart failure, we evaluated the prognostic significance of Doppler-detected valvular regurgitation in patients with endocarditis who had not yet developed clinical heart failure. PATIENTS AND METHODS: We reviewed the medical records of 65 patients with a clinical diagnosis of infective endocarditis from May 1985 to March 1990. A total of 49 patients were included in the study: 33 patients with native valve endocarditis and 16 patients with prosthetic valve endocarditis. The initial Doppler echocardiogram was examined in these patients to determine the presence and degree of valvular regurgitation. RESULTS: Significant (moderate to severe) valvular regurgitation was detected in 23 (47%) patients. The presence or absence of significant valvular regurgitation did not predict the development of congestive heart failure, the need for surgery, or death (p = NS). The development of congestive heart failure was significantly associated with the need for surgery (p less than 0.0001) and death (p less than 0.05). CONCLUSION: We conclude that the detection of significant valvular regurgitation in patients with infective endocarditis who have not yet developed heart failure is not predictive of future complications nor does the absence of significant valvular regurgitation identify a group of patients with a more favorable prognosis. In our series, patients who developed congestive heart failure had a significantly higher incidence of surgery and death. Therefore, decisions regarding clinical management in patients with infective endocarditis should not be made solely on the presence or absence of echocardiographically detected valvular regurgitation.     

Mitral valve billowing and prolapse—an overview

Three decades after it was demonstrated that nonejection systolic clicks and late systolic murmurs have a mitral valve origin and that a specific syndrome is associated with the primary degenerative mitral lesion, numerous questions remain unanswered. A principal cause of confusion is the use of the term ‘prolapse’, which essentially implies a pathological state, in many patients with minimal evidence of a mitral valve anomaly. It should be recognised that no specific feature, whether evaluated by high standard echocardiography or indeed by careful morphological and histological examination, can be defined which distinguishes a normal variant from a pathological valve. There is a grandation from the normal billowing doring ventricular systole of mitral leaflet bodies to marked billowing. With advanced billowing or floppy leaflets, failure of leaflet edge apposition supervenes (true prolapse). This is functionary abnormal and allows mitral regurgitation. Prolapse in turn may progress to a flail twfltT and hence gross regurgitation. Relatively rare complications of this degenerative mitral valve anomaly include systemic emboli, infective endocarditis, arrhythmias and, arguably, autonomic nervous system abnormalities. An attempt is made to ctarify the management of some symptoms and other aspects of mitral prolapse - including rheumatic anterior leaflet prolapse (without billowing) which remains prevalent in South Africa and Third World countries.     

Perforated mitral valve aneurysm associated with Libman-Sacks endocarditis

The perforation of a mitral valve aneurysm is a rare disease which induces acute mitral regurgitation and is usually induced by infective endocarditis; however, in this case report, acute heart failure was caused by a perforated mitral valve aneurysm that was speculated to be due to Libman-Sacks endocarditis with systemic lupus erythematosis and secondary anti-phospholipid syndrome. Mitral valve plasty was performed and thereafter heart failure improved.     

Echocardiographic and clinical correlations in infectious endocarditis of the left heart

The echocardiographic features were correlated with the clinical findings and outcome in 35 patients with aortic and/or mitral valve endocarditis. There were 26 males and 9 females with a mean age of 38 years. The infection involved native valves in 27 patients and prosthetic valves in 8 patients. Echocardiographically, fourteen patients had involvement of native aortic valve. All patients in this group required surgical intervention, nine patients during antimicrobial therapy. Congestive heart failure was the clinical indication for valvular replacement. A patient died immediately after surgery from low cardiac output syndrome. Six patients had echocardiographic evidence of aortic and mitral valves involvement. A patient in this group expired before surgery, five underwent surgery because of progressive heart failure (aortic or aortic and mitral valves replacement). Seven patients showed lesions on native mitral valve (6 in this group had prolapse syndrome). A patient died from cerebrovascular embolus, two underwent surgery because of persistent infection and embolic events, four were successfully treated with medical therapy. Among patients with prosthetic valve endocarditis, four showed signs of valvular dehiscence and required surgical intervention, during antimicrobial therapy, from congestive heart failure; one patient expired from recurrent infection. The pathological findings correlated well with echocardiographic findings. Conclusions: in IE the localization of lesions by echo has prognostic significance: most patients with aortic valve or aortic and mitral valves endocarditis require early surgical intervention because of congestive heart failure. On the contrary, mitral valve involvement carries a better prognosis, requiring less frequently valvular replacement; the patients with echocardiographic signs of prosthetic valve dehiscence require urgent intervention.(ABSTRACT TRUNCATED AT 250 WORDS)     

Mitralklappenprolaps: Identifikation von Patienten mit erhöhtem Risiko und therapeutisches Management

Mitral valve prolapse shows a wide spectrum from a benign anatomic variant to a progressive disease with severe cardiovascular morbidity and mortality. Echocardiography is the most important tool for diagnosis and risk stratification. Predictors for high risk are significant thickening of mitral leaflet of > 5 mm ("classic" prolapse), moderate to severe mitral regurgitation and reduced left ventricular function. These patients have an increased risk of infective endocarditis, cerebral ischemia and sudden cardiac death. Because of the risk for the development of severe mitral regurgitation requiring surgery short follow-up intervals are necessary.In mitral prolapse syndrome cardiac clinical signs (palpitation, rhythm disorders, syncope, etc.) are associated with a prolapse that can be treated symptomatically with drugs after exclusion of other causes and significant mitral regurgitation requiring surgery.     

Posterior mitral leaflet aneurysm - A rare cause of mitral regurgitation

Posterior mitral leaflet aneurysm is a very rare complication of infective endocarditis. A 28-year-old athlete got admitted with fever, congestive heart failure and multi-organ dysfunction syndrome. The echocardiogram showed large aneurysm of the posterior mitral leaflet with severe mitral regurgitation. Patient succumbed to refractory heart failure.     

Mitral leaflet billowing and prolapse: its prevalence around the world

Primary mitral leaflet billowing, or so-called mitral valve prolapse, has become the most common valve anomaly in the United States and is also frequently found throughout the world. Its prevalence varies from less than 1% to 38%, differing not only between countries but also within the same country. The prevalence depends on whether the study is clinical or echocardiographic, based on autopsy or surgical material, or of hospital or non-care-seeking population. Other explanations for the varying prevalence are the age, sex and weight differences of the study population, imprecise terminology, the care with which auscultation and/or echocardiography are carried out and interpreted, and some selection biases. Although prevalent throughout the world, the condition is generally benign and can often be regarded as a normal variant. Among the complications of mitral valve prolapse, progressive mitral regurgitation and infective endocarditis are particularly noteworthy. Primary mitral valve prolapse is currently a leading cause of mitral regurgitation and also of infective endocarditis.     

Echocardiographic findings in children with Marfan syndrome

Background

The typical cardiac manifestations of Marfan syndrome are aortic regurgitation with progressive dilatation of the aortic root, which may cause dissection and rupture of the ascending aorta, mitral valve prolapse and mitral valve regurgitation. In this study, we aimed to show echocardiographic findings in 11 patients with Marfan syndrome.

Methods

Diagnosis of Marfan syndrome was based on the Ghent criteria. All patients had a full echocardiographic evaluation. During the evaluation, we investigated the presence of mitral valve prolapse, mitral valve regurgitation, tricuspid valve prolapse, dilatation of the aortic root, and aortic regurgitation.

Results

Eleven patients were diagnosed as Marfan syndrome (seven male, four female, age 4–14 years). All had mitral valve prolapse (nine with mitral valve regurgitation). Among these 11 patients, seven had accompanying tricuspid valve prolapse, six had dilatation of the aortic root and two had aortic regurgitation.

Conclusion

Eleven patients in our clinic were diagnosed as Marfan syndrome since they had distinct characteristics of marfanoid phenotype. Echocardiographic evaluation of these patients showed marked heart valve involvement. In Marfan syndrome, it is known that the aortic valve is affected following mitral valve involvement. In our experience, aortic root dilatation is less common. However, particular attention should be given to following up aortic root status with non-invasive echocardiography to institute measures to prevent complications.     

The Marfan syndrome: cardiovascular physical findings and diagnostic correlates.

Correlations among cardiac symptoms and auscultatory and phonoechocardiographic findings in Marfan syndrome have not been completely defined. A total of 24 patients with Marfan syndrome (16 men and 8 women; mean age 28.2 +/- 8.6 years) were studied. Mitral valve prolapse was noted in 22, of whom 19 had either nonejection systolic click or mitral regurgitation murmur. Mitral regurgitation was noted in 12 patients by Doppler imaging. Aortic root dilatation was noted in 20 patients and aortic regurgitation in six, five of whom had aortic regurgitation murmur (5 of 20 patients had undergone surgery). Proximal aortic dissection was noted in two. Dyspnea (n = 12) was associated with progressive mitral or aortic regurgitation in four, but in the others dyspnea could not be explained by valvular or ventricular abnormalities. Chest pain was related to pneumothorax in five and aortic dissection in two but was not associated with either in 15 patients. Palpitations (n = 12) and lightheadedness (n = 6) were not associated with specific arrhythmias. In conclusion, mitral valve prolapse and aortic root dilatation were the most common cardiovascular abnormalities in Marfan syndrome. Mitral valve prolapse was frequently associated with typical auscultatory findings and symptoms including dyspnea, chest pain, palpitations, and lightheadedness, whereas aortic root dilatation could be clinically silent unless complicated by aortic regurgitation or aortic dissection.     

Mitral valve billowing and prolapse: perspective at 25 years

The syndrome of primary mitral leaflet billow, with or without prolapse, is associated with myxomatous degeneration of the mitral valve apparatus, mainly the posterior leaflet, and the syndrome may be familial. It manifests clinically with an isolated nonejection systolic click (billow), a murmur of mitral regurgitation that is usually late systolic (prolapse), or a combination of murmur and click. Echocardiography identifies and assesses the extent of the billowing of mitral leaflet bodies but there are no specific echocardiographic criteria that can differentiate normal from pathological billowing. Similarly, a prolapsed leaflet is not detected echocardiographically when there is localized and mild failure of leaflet edge apposition but a more severely prolapsed, or flail, leaflet can be demonstrated and confirmed by that technique. Symptoms of the syndrome include anxiety, chest pain and palpitations. The resting electrocardiogram may show ST segment and T wave abnormalities. The majority of patients have a benign course and require reassurance only. Complications include systemic emboli, infective endocarditis, progression to severe mitral regurgitation, arrhythmias and, rarely, sudden death. Patients with prolapse of a leaflet edge are more likely to develop complications than those with only billowing of the leaflet bodies. Surgery, preferably valvuloplasty, is required for severe regurgitation and may also be indicated for potentially lethal tachyarrhythmias unresponsive to medical therapy. Mitral leaflet billow and prolapse may be secondary to, or associated with, many conditions. The prognosis is then principally that of the underlying disease of which ischemic heart disease and hypertrophic cardiomyopathy are the most important.     

Underlying cardiac lesions in adults with infective endocarditis. The changing spectrum

The spectrum of recognized cardiac lesions underlying infective endocarditis has been changing as a result of the decline in incidence of rheumatic heart disease, the recognition of the entity of mitral valve prolapse, and the improvement in cardiac diagnostic techniques. Sixty-three cases of native valve endocarditis diagnosed in Memphis hospitals between 1980 and 1984 were reviewed. All diagnoses of underlying cardiac lesions were confirmed by two-dimensional echocardiography, cardiac catheterization, and/or histopathologic examination of valve tissues. Major categories of underlying lesions were as follows: mitral valve prolapse, 29 percent; no underlying disease, 27 percent; degenerative lesions of the aortic or mitral valve, 21 percent; congenital heart disease, 13 percent; rheumatic heart disease, 6 percent. Thus, mitral valve prolapse and, in the elderly, degenerative lesions have displaced rheumatic and congenital heart diseases as the major conditions underlying endocarditis. Redundancy of the mitral valve leaflets was noted in 17 of 18 patients in whom endocarditis was superimposed upon mitral valve prolapse. The risk of infective endocarditis appears to be substantially increased in the subset of patients with mitral valve prolapse who exhibit valvular redundancy.     

An Unusual Presentation of an Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) in an Adult: Anterior Papillary Muscle Rupture Causing Severe Mitral Regurgitation

We describe a 29-year-old male, previously in good health, with no history of angina pectoris and no risk factors for ischemic heart disease presenting with biventricular failure and severe mitral valve regurgitation. There were no signs or serological test results to suggest infective endocarditis. Transthoracic echocardiography (TTE) revealed severe anterior mitral valve prolapse secondary to papillary muscle rupture, severe mitral valve regurgitation, as well as an anterior myocardial wall hypokinesis. Parasternal short-axis view showed an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), which was confirmed on coronary angiography. This is an unusual presentation of ALCAPA in an adult.     

Left-sided valvular active infective endocarditis. A study of forty-five necropsy patients

Clinical and anatomic features are described in forty-five necropsy patients with left-sided valvular active infective endocarditis. Vegetations in 53 per cent of the patients involved previously anatomically normal valves, and 76 per cent of all patients had previously functionally normal valves. Predisposing factors allowing entrance of virulent or unusual organisms or alterations of host defense mechanisms appear to account for the frequency of infective endocarditis on normal valves. Valvular dysfunction resulting from infective endocarditis occurred in at least 59 per cent and possibly as high as 74 per cent of the forty-five patients causing congestive heart failure in all. Myocardial lesions were present in 87 per cent of the thirty-eight patients in whom multiple histologic sections were examined, but in none did heart failure appear to result from these lesions. Papillary muscle necrosis was present in 58 per cent, but in none did it appear to cause mitral regurgitation. Congestive heart failure could not be attributed in any patient to myocardial lesions. Pericarditis occurred in eight patients (20 per cent), and in each a site of direct extension of the inflammation into the pericardium was apparent. Ring abscesses occurred in twelve of thirty-one patients with aortic valve vegetations and in none of twenty-six with mitral valve vegetations. Ring abscesses indicate severe destruction of valvular cusps and severe valvular dysfunction.     

The multifactorial etiology of mitral valve prolapse, a new entity of postinflammatory mitral valve prolapse

As etiologic factors for mitral valve prolapse, papillary muscle dysfunction due to coronary artery disease, hypertrophic obstructive cardiomyopathy, atrial septal defect and trauma have been reported. Connective tissue diseases such as Marfan's syndrome. Ehlers-Danlos syndrome or Turner's syndrome may also result in mitral valve prolapse. In the majority of patients with mitral valve prolapse, however, the etiology is unknown, in which case the condition is considered primary or idiopathic. We evaluated 33 consecutive surgically-excised mitral valves removed from patients with regurgitant prolapsing mitral valves and congestive heart failure. On microscopic examination, myxomatous degeneration was observed in 14 cases, postinflammatory changes, however, were seen in the other 19 cases and included diffuse vascularization with thick-walled vessels, round-cell infiltration and destruction of valve architecture. These valves showed a varying degree of doming and/or interchordal hooding as well as an increased surface area. Elongated chordae tendineae were seen in 37%, chordal rupture in 16% of the patients. Slightly fused chordae tendineae, minimal commissural fusion and/or fibrous thickening of cusps were also observed, findings which simulate closely rheumatic valvulitis. Patients with postinflammatory mitral valve prolapse were younger at the time of operation and at the onset of symptoms, had smaller surface areas of the anterior mitral leaflet and more marked leaflet thickening than patients with myxomatous mitral valve prolapse. The results of the study show that mitral valve prolapse in patients with severe mitral regurgitation can be attributed to postinflammatory changes; we suggest, therefore, the term "postinflammatory valve prolapse". Postinflammatory mitral valve prolapse may be due to manifest or subclinical rheumatic fever.(ABSTRACT TRUNCATED AT 250 WORDS)