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Query: UMLS:C0014118 (endocarditis)
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For half a century the systolic click and late systolic murmur lay dormant as innocent auscultatory curiosities. The thirteen years since Barlow related these phenomena to mitral leaflet prolapse have witnessed an astonishing information explosion. We have sought to bring together the accumulated data in this review. An Historical Perspective traces the evolution from the now abandoned "pericardial" or "extracardiac" phases, through the leafletchordal phase (redundancy), the myocardial phase (segmental left ventricular contraction abnormalities), to the anular phase (dilatation and faulty systolic contraction). Functional Anatomy is dealt with in terms of pathology, pathophysiology, hemodynamics, angiocardiography, echocardiography, and physical and pharmacological interventions. Clinical Manifestations are concerned with prevalence, natural history, symptoms, physical signs, electrocardiographic abnormalities and roentgen fingings. The four Major Complications- sudden death, infective endocarditis, spontaneous rupture of chordae tendineae, and progressive mitral regurgitation- are examined. Associated Cardiac Diseases, i.e., Marfan's syndrome, ostium secundum atrial septal defect and atherosclerotic coronary artery disease, are discussed, and a section on Treatment deals chiefly with prophylaxis for infective endocarditis and the management of arrhythmias and chest pain. A final section on Evolving Information considers etiologic concepts, the nature of left ventricular contration abnormalities, the cause of chest pain, the relationship to Marfan's syndrome and ostium secundum atrial septal defect, and the effect of aging and sex differences on leaflet chordal redundancy.
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PMID:Mitral valve prolapse. 77 40

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.
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PMID:Mitral valve prolapse. 77 95

A syndrome consisting of an apical systolic click and late systolic murmur appeared over a period of three months in a patient with acromegaly. Levels of growth hormone were highest during this interval. As a result of group D streptococcal endocarditis three months later, the patient sustained cerebral embolism and acquired free mitral regurgitation from ruptured chordae tendineae. During follow-up over 18 months, neither the level of growth hormone nor the degree of mitral regurgitation changed significantly.
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PMID:Acromegaly, the systolic click syndrome, and group D streptococcal endocarditis. 81 74

Atrial fibrillation is rare in children. Previous reports associated it with severe rheumatic heart disease and a poor prognosis. This review is of the unique experience of 35 cases of atrial fibrillation in children in the past 22 years; 23 patients were boys. The age of onset ranged from 1 day to 19 years (average, 8 years). Associated cardiac conditions were severe rheumatic mitral regurgitation (3 cases), cardiomyopathy (5), atrial tumors (2), infective endocarditis (1), paroxysmal atrial tachycardia of infants (4), idiopathic paroxysmal atrial fibrillation (1), Marfan's syndrome with mitral regurgitation (1), endocardial fibroelastosis (1), and structural congenital heart malformations (17). Surgical correction of congenital heart lesions was directly related to the development of atrial fibrillation in 14. Varying arrhythmias of the sick-sinus syndrome were observed in five children. The atrial fibrillation was paroxysmal or transient in 21 patients and persistent in 14. Treatment depended on the underlying condition. Digoxin was used in all cases and cardioversion attempted in ten; no patient was given anticoagulants. Three children had cerebral emboli, with residual defects. Eighteen patients are known to be alive, 13 are dead, and 4 are lost to follow-up. Atrial fibrillation in childhood is an indication for complete investigation of the patient and for the institution of treatment appropriate to the underlying disease.
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PMID:Atrial fibrillation in children. 83 8

Echocardiographic observations are described in 25 opiate addicts with active infective endocarditis involving apparently previously normal valves. Infective endocarditis was isolated to the tricuspid valve in 11 patients, involved both right- (tricuspid valve) and left-sided valves in 7 and was isolated to the left-sided valves in 7 (mitral valve in 6). Twenty patients (80%) had tricuspid valve regurgitation, 12 had mitral regurgitation, 3 had aortic regurgitation and none had pulmonary valve regurgitation. Considering the 75 cardiac valves (excluding the pulmonary) in the 25 patients, echocardiographic abnormalities consistent with active infective endocarditis were detected in 26 (74%) of the 35 clinically incompetent valves but in none of the 40 competent valves. Comparison of the 20 incompetent tricuspid valves with the 12 incompetent mitral valves indicated that (1) the echocardiogram was less sensitive in detecting tricuspid valve lesions, (2) rupture of tricuspid valve chordae tendineae was absent or not detectable, and (3) tricuspid valve vegetations tended to be larger.
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PMID:Echocardiographic observations in opiate addicts with active infective endocarditis. Frequency of involvement of the various values and comparison of echocardiographic features of right- and left-sided cardiac valve endocarditis. 87 7

The benefits and hazards of catheterization and angiography were evaluated in 19 patients with acute aortic and/or mitral endocarditis and heart failure. In 14 patients (74%), the bedside diagnosis of valvular insufficiency and heart failure was proved correct. In three patients with both aortic and mitral valve disease, angiography (without hemodynamic measurements) was necessary to clarify the diagnosis. Angiography detected four aortic aneurysmal erosions that were unsuspected clinically, but missed three others. After angiography, heart failure worsened in two patients with severe progressive aortic insufficiency and one died. Thus, catheterization-angiography was of greatest value if more than one left-sided valve lesion was present, if extravalvular diseases mimicked heart failure, or if extravalvular infection was present. Patients with isolated, clear-cut mitral insufficiency usually do not need these diagnostic procedures, and they are probably contraindicated in patients with severe aortic regurgitation with rapidly progressing heart failure.
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PMID:Role of cardiac catheterization in infective endocarditis. 91 34

A 24-year-old woman with Marfan's syndrome and mitral regurgitation had clinical features suggestive of infective endocarditis. The causative organism was Streptococcus viridans. Initial therapy with penicillin G, in a dose that should have been bactericidal and hence curative according to the results of the initial quantitative antimicrobial studies, became inadequate. The strain of S. viridans displayed considerable variation in both growth properties and antimicrobial sensitivity during the course of therapy. In addition, a different strain of S. viridans was cultured 1 month after treatment had begun. It is therefore important to repeat cultures and antimicrobial sensitivity testing during treatment of infective endocarditis.
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PMID:The heterogeneity of Streptococcus viridans: therapeutic considerations in infective endocarditis. 112 90

Ten patients with the billowing mitral leaflet syndrome complicated by infective endocarditis are reported. Two patients had a non-ejection systolic click and 8 had both a non-ejection systolic click and a late systolic murmur. These auscultatory features were difficult to detect in 4 instances in that they were intermittent, soft, or brought out only with postural change. Seven patients were unaware of their cardiac lesions. A low grade pyrexia was present in all 10 patients. Four patients presented with clinical features caused by reversible neurological lesions. Blood cultures were positive in all patients, with Staphylococcus albus the infecting organism in 6. Antibiotic therapy was successful with significant mitral regurgitation supervening in only one instance. The importance of the billowing leaflet as a potential site of infective endocarditis is emphasized. It seems that antibiotic prophylaxis is indicated at times of increased risk of infection in subjects with a non-ejection systolic click or a late systolic murmur.
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PMID:Infective endocarditis in the billowing mitral leaflet syndrome. 113 36

Nonejection clicks and associated mitral systolic murmurs are common in routine cardiologic practice and can result from multiple etiologic factors affecting the complex mitral valve mechanism. Such factors include a specific syndrome the essential feature of which is that the mitral leaflets or part thereof, primarily the posterior one, are voluminous. The syndrome has stimulated widespread interest and study during the last decade and various descriptive terms, including the "billowing mitral leaflet syndrome" (BMLS), have been applied to it. A familial occurrence of the BMLS may be detected and symptoms include chest pain, palpitations, syncope, and anxiety. Arrhythmias, conduction defects, and ECG abnormalities which mimic occlusive coronary artery disease are important features which remain ill understood. It is suggested that there is a possible relationship between the so-called "athlete's heart" and the BMLS. We also postulate that the entity of acute myocardial infarction without demonstrable occlusive coronary artery disease is, in at least some instances, a complication of the BMLS-possibly on the basis of coronary spasm. More severe mitral regurgitation, infective endocarditis, or, rarely, sudden death may supervene in the BMLS but we conclude, from published data and our own experience, that the prognosis is generally good.
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PMID:The problem of nonejection systolic clicks and associated mitral systolic murmurs: emphasis on the billowing mitral leaflet syndrome. 119 42

Between April 1969 and November 1973 103 patients underwent isolated mitral replacement with three-cusp stented tissue valves. Autologous fascia lata was used in 50 patients, homologous fascia lata in 21, and heterologous pericardium in 32. The early mortality rate (14.6%) was influenced by age, the extent of preoperative cardiac disability, and low cardiac output. The survivors were followed up for periods varying from 8 to 60 months (average 37 months). In general, a factor in late death (13.6%) was high preoperative pulmonary artery pressure. In the autologous fascial series valve failure and infective endocarditis were significantly related to late mortality. The results with homologous fascia and pericardium were better than with autologous fascia valves. The incidence of postoperative mitral regurgitation was singificantly lower in the homologous fascial and pericardial series and none of these grafts had to be removed. The incidence of thromboembolism was low without anticoagulants. Actuarial analysis showed a survival rate at five years of 82.2 per cent. We no longer use autologous fascial valves. Though better results have been obtained with both homologous fascia and pericardium we prefer the physical characteristics of heterologous pericardium and it is easy to obtain.
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PMID:Tissue valves in the mitral position. Five years' experience. 119 27


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