Infective endocarditis--a 25 year overview of diagnosis and therapy.

Diagnosis and management of infective endocarditis have significantly changed in the past 25 years. Improved bacteriologic techniques have allowed detection of cases of infective endocarditis caused by unusual organisms. Bactericidal therapy has become available for patients with gram-negative endocarditis and antimicrobial therapy has improved. Echocardiography has become an important diagnostic and management aid, and cardiac valve replacement has dramatically improved the outlook for many patients.

CNS embolism due to atrial myxoma: clinical features and diagnosis.

The records of 40 patients with pathologically verified atrial myxoma identified during the period 1957 to 1977 were studied. The patients ranged in age from 17 to 77 years; women predominated by a ratio of 3:1. Thirty-five patients had left atrial myxoma, three patients had right atrial myxoma, one patient had bilateral myxomas, and one patient had a right atrial myxoma and subsequent removal of a left atrial myxoma. Ten of the 37 patients (27%) with left atrial myxoma had at least one neurologic event suggesting ischemia secondary to embolization. Transient or permanent ischemic events were noted in the cerebral hemispheres, brainstem, cerebellum, retina, and spinal cord. Although some patients were seen at the initial evaluation with primarily neurologic events, cardiac and constitutional symptoms dominated the clinical picture. Echocardiography and sector scanning provide a practical method for early clinical diagnosis.

Prognosis in rupture of the ventricular septum after acute myocardial infarction and role of early surgical intervention.

Since 1944, 91 patients (50 men and 41 women, mean age 68 years [range 39 to 86]) with ventricular septal rupture after acute myocardial infarction were seen at the Mayo Clinic. Patients were divided into 4 groups according to therapy and timing of surgical intervention. Fourteen patients seen before 1965, when surgery was not performed for such a complication or not readily available, were excluded from the analysis. Group 1 (n = 22) had surgery within 48 hours of septal rupture, group 2 (n = 6) underwent operation between 2 and 14 days, group 3 (n = 24) had surgery after 14 days, and group 4 (n = 25) only received medical treatment. Short-term (30 days) survivors (45%, 35 of 77 patients) were compared with nonsurvivors. Using logistic regression, by univariate analysis, 3 variables were significantly associated with outcome: age (p less than 0.01), cardiogenic shock (p less than 0.00001), and long delay between ventricular septal rupture and surgical intervention (p less than 0.004). By multivariate analysis, however, only cardiogenic shock (p less than 0.00001) and age (p less than 0.007) correlated with an adverse outcome. In patients with cardiogenic shock after septal rupture, the prognosis was uniformly fatal unless patients undergo early surgery. None of the 23 patients in groups 2, 3 or 4 survived, whereas 5 of 13 patients (38%) who had surgery within 48 hours of septal rupture survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypertrophic obstructive cardiomyopathy and lentiginosis: a little known neural ectodermal syndrome.

Eleven patients, 10 male, with classic hypertrophic obstructive cardiomyopathy and lentiginosis are described. Physical examination showed differences from the few previously reported cases in that (1) this condition was not confined to children; (2) mental retardation, sensorineural deafness and gonadal and somatic infantilism were either rare or absent; and (3) detailed family studies provided no evidence that this condition was inherited. Nine patients underwent cardiac catheterization and left ventricular angiography; all had left ventricular outflow obstruction and three had concomitant right ventricular outflow obstruction with a pressure gradient in excess of 100 mm Hg. Ten of the 11 patients were severely symptomatic, and 7, each with a left ventricular pressure gradient of more than 70 mm Hg, underwent successful septal myotomy/myectomy that resulted in marked symptomatic improvement that was maintained after long-term follow-up.

Hypertrophic obstructive cardiomyopathy: ten- to 21-year follow-up after partial septal myectomy.

This study reviews the outcome in 36 consecutive patients who survived partial septal myectomy for hypertrophic obstructive cardiomyopathy operated on between 1960 and 1972. All patients were followed up until death or until June 1981 (mean 13.4 years). Of the 26 survivors, 17 had been more than mildly symptomatic preoperatively, but only 1 remained so postoperatively. The operation was effective in relieving the obstruction (peak systolic pressure gradient reduced from 79 to 8 mm Hg [p less than 0.001]), and mitral regurgitation was relieved. No survivor's symptoms worsened, but 10 died late--4 suddenly, 5 from congestive heart failure, and 1 from a malignancy. The 10-year survival rate was 77%. No correlation with outcome was found with respect to age, surgical approach, preoperative functional class, pressure gradient, left ventricular end-diastolic pressure, or presence of atrial fibrillation, but atrial fibrillation occurring late postoperatively (12 patients) was associated with an increased frequency of late death (7 of 10 late deaths) or continuing New York Heart Association functional class III status. Early or late postoperative complete heart block occurred in 1 patient each. Thus, these results suggest a favorable effect of operation and support continued surgical intervention for appropriate patients.

Echocardiogram in common (single) ventricle: angiographic-anatomic correlation.

Common ventricle is a rare congenital anomaly in which the ventricular chamber receives blood from two separate atrioventricular (A-V) valves or a common A-V valve. Diagnosis of common ventricle was established in 55 patients aged 3 months to 33 years (mean 10 years) at cardiac catheterization in all 55 and at operation or autopsy in 24. In common ventricle with two angiographically demonstrable A-V valves (47 patients), the echocardiographic features included: (1) simultaneous recording (in the same sonic beam with the transducer held stationary) of the echoes of an anterior and a posterior A-V valve without an intervening septal echo (45 of 47 patients); (2) absence, on a base to apex scan, of the ventricular septal echo in the usual position separating the A-V valves (47 of 47 patients); (3) recording, in patients with common ventricle and outflow chamber, of this small outflow chamber anterior to the A-V valves (20 of 23 patients); and (4) recording of echographic continuity of the posterior A-V valve and great artery (27 of 31 patients). In common ventricle with a common A-V valve (8 patients), the echocardiographic features included: (1) a single demonstrable A-V valve, located posteriorly in the ventricle, which showed a large amplitude of excursion during diastole; and (2) absence of a second A-V valve echo or ventricular septal echo. Eight patients were studied postoperatively after surgical correction by ventricular septation. Echographic features included visualization of a prosthetic septum that produced a dense echo and divided the common ventricle into "right" and "left" ventricular chambers. This septum had a large excursion anteriorly during systole. Because common ventricle is now amenable to surgical correction, echocardiography should play an important role in assessment of ventricular anatomy in this complex congenital cardiac defect.

Idiopathic hypertrophic subaortic stenosis in the young.

Thirty-six young patients with idiopathic hypertrophic subaortic stenosis were studied. Twenty-seven patients were male and 9 female, and their mean age was 11.3 years (range 5 months to 20 years). Twenty-three patients (64 percent) had symptoms, the most common being dyspnea, angina and syncope. Diagnostic difficulties were encountered frequently in younger patients, especially those with right heart involvement, and in asymptomatic patients with murmurs suggestive of other cardiac defects. Patients were classified retrospectively into three groups on the basis of management. The first group consisted of 16 patients who were operated on; 4 of these patients died, 1 operatively and 3 suddenly late postoperatively (at 1.6, 2 and 10 years). The 12 long-term survivors (average follow-up period 6.2 years) have had good relief of symptoms. The second group comprised seven patients treated with propranolol; none of these died. The 13 patients in the third group received no therapy; 7 of these patients died, 6 suddenly and 1 from congestive cardiac failure. Idiopathic hypertrophic subaortic stenosis is a serious disorder that may present at any age and that may be difficult to diagnose. All patients with this disorder should be treated with propranolol; surgical intervention, although it does not totally abolish the risk of sudden death, appears to offer symptomatic improvement in most cases over a long-term follow-up period.

The natural history of idiopathic dilated cardiomyopathy.

Between 1960 and 1973, a total of 104 patients at the Mayo Clinic had a diagnosis of idiopathic dilated cardiomyopathy on the basis of clinical and angiographic criteria; these patients were followed up for 6 to 20 years. Twenty-one percent of the patients had a history of excessive consumption of alcohol, 20 percent had had a severe influenza-like syndrome within 60 days before the appearance of cardiac manifestations and 8 percent had had rheumatic fever without involvement of cardiac valves several years before; thus, possible etiologic risk factors of infectious-immunologic type may be important. Eighty patients (77 percent) had an accelerated course to death, with two thirds of the deaths occurring within the first 2 years. Twenty-four patients (23 percent) survived, and 18 of them had clinical improvement and a normal or reduced heart size. Univariate analysis at the time of diagnosis revealed three factors that were highly predictive (p less than 0.01) of the clinical course: age, cardiothoracic ratio on chest roentgenography and cardiac index. Systemic emboli occurred in 18 percent of the patients who did not receive anticoagulant therapy and in none of those who did; thus, anticoagulant agents should probably be prescribed unless their use is contraindicated.

Diastolic opening of aortic valve: an echographic observation.

Echocardiography provided excellent insight into the altered hemodynamics in a patient with acute, severe aortic regurgitation due to bacterial endocarditis. The most notable echographic findings were premature closure of the mitral valve and diastolic opening of the aortic valve. At surgery, the patient proved to have a large perforation in the base of the noncoronary cusp.

Unusual echocardiographic findings in a patient with left atrial myxoma.

A 53-year-old patient is presented who had left atrial myxoma with unusual echocardiographic findings. The patient had been regarded for 10 years as having mitral stenosis. This error in diagnosis was due in part to misinterpretation of his echocardiograms. This report is presented to emphasize not only the unusual echocardiographic findings but also the subtle findings on the phonocardiogram and apexcardiogram which should have led to the correct diagnosis.

Ebstein's anomaly: the clinical features and natural history of Ebstein's anomaly of the tricuspid valve.

The clinical features and natural history of Ebstein's anomaly are analyzed on the basis of experience with 67 consecutive patients who have had a mean follow-up of 12 years. Of the total group, 26 (39%) of the patients remained in functional Class I or II and 41 (61%) progressed at some time into Class III or IV. Death occurred in 14 (21%) of the patients, and these were characterized by one or more of the following features: (1) they were in functional Class III or IV, (2) they had moderate to severe cardiomegaly with a cardiothoracic ratio greater than 0.65, (3) they had cyanosis or an arterial oxygen saturation of less than 90%, and (4) they were infants when the diagnosis was made (10 patients, of whom 5 died). In patients in Class III or IV, a comparison was made between patients treated medically (10) and those treated surgically--either with tricuspid valve replacement (15) or with a modified tricuspid annuloplasty (16). It appears that modified tricuspid annuloplasty is the procedure of choice, because despite an operative mortality of 25% (4 patients), 12 of the 16 patients so treated improved clinically or had a reduction in heart size.

Treatment of penicillin-sensitive streptococcal infective endocarditis.

Patients with infective endocarditis caused by penicillin-sensitive streptococci (minimal inhibitory concentration less than or equal to 0.1 microgram/ml of penicillin) may be treated successfully with one of the following three regimens: (1) aqueous penicillin G administered intravenously for 4 weeks, (2) aqueous penicillin G administered intravenously for 4 weeks in combination with streptomycin for the first 2 weeks of therapy, or (3) parenterally administered penicillin plus streptomycin for 2 weeks. No substantial difference in the relapse rate exists among the three regimens, and a cure rate of at least 98% may be anticipated with each of the three forms of therapy. The major advantage of the 2-week regimen is that it is more cost-effective than 4 weeks of hospitalization and therapy. The major disadvantage of the 2-week regimen and the 4-week regimen that uses streptomycin is the relatively low risk of streptomycin-associated vestibular toxicity. Patients unable to tolerate penicillin may be treated with vancomycin or a cephalosporin administered parenterally for 4 weeks.

An unusual case of idiopathic hypertrophic subaortic stenosis.

Echocardiography revealed idiopathic hypertrophic subaortic stenosis in a patient previously operated on for calcific aortic valve disease. The echocardiography in addition showed symmetric left ventricular hypertrophy and unusual localization of the septal hypertrophy to the apical half of the septum.

Left atrial myxoma simulating peripheral vasculitis.

Left atrial myxoma remains a diagnostic challenge clinically. A brief review of previously reported cases and their individualistic clinical and laboratory features are described. The present case report documents an unusual clinical presentation, initially directing attention to the central nervous system as well as to the peripheral arterial system. Histologic evidence of peripheral arterial myxomatous emboli, associated with vasculitis but without other confirmatory immunologic evidence of collagen vascular disease, had predated for 14 months the subsequent echocardiographic diagnosis of left artrial myxoma. These findings further emphasize the importance of recognizing the enigmatic and variable clinical presentation of left atrial myxoma.

Echophonocardiographic findings in patients with prosthetic heart valve malfunction.

This report represents a retrospective analysis of preoperative echophonocardiographic studies in 118 patients who had surgery for the repair or replacement of malfunctioning prosthetic valves. A variety of prostheses implanted in the mitral, aortic, and tricuspid positions were assessed. The most frequent prosthetic malfunctions were regurgitation, thrombosis, ball variance, and infective endocarditis. There were echophonocardiographic abnormalities in 71% of the cases. The retrospective diagnosis of prosthetic dysfunction varied according to the type of prosthesis, type of malfunction, and site of implantation of the valve. A high percentage of the prostheses did have echophonocardiographic abnormalities, and consideration of these, in conjunction with clinical data, may help the clinician in the evaluation of prosthetic heart valves.

Atrial myxomas: a review of clinical experience in 40 patients.

We have reviewed the Mayo Clinic experience in the diagnosis and management of 40 cases of atrial myxoma seen over a 20-year period, 1957 to 1977. The diagnosis of atrial myxoma was seldom made clinically on the basis of history, physical examination, chest roentgenogram, or electrocardiogrammthere was no reduction in the time interval from onset of symptoms to operation between patients seen in the period 1957-1967 and those seen in 1968-1977. Although myxomas are rare, they should be considered in the differential diagnosis of patients with suspected mitral valve disease and congestive heart failure or constitutional disturbances who are in sinus rhythm. The greater number of patients diagnosed preoperatively in the second 10-year period, 1968-1977, reflects the introduction of echocardiography as a noninvasive diagnostic procedure. Echocardiography should be performed on all young patients with embolic disease and on those in whom the diagnosis of myxoma needs to be ruled out before exploratory or definitive surgery is undertaken so that unnecessary and potentially serious complications can be avoided. Surgical excision of the myxoma can be performed with low mortality (1 death occurred in 37 cases or 2.7%), and it provides excellent and sustained symptomatic relief. The recurrence rate is low (5.4%), but long-term follow-up and serial echocardiography are advisable.

Prosthetic valve endocarditis.

Prosthetic valve endocarditis is an infrequent but serious complication of cardiac valve replacement. The overall frequency of prosthetic valve endocarditis is approximately 2%. The frequency of early-onset and late-onset infections is 0.78% and 1.1%, respectively. Staphylococci are the most common isolate from patients with early-onset infection, accounting for 47.5% of the total number of isolates. Staphylococcus epidermidis causes 27% of these staphylococcal infections. Among patients with late-onset infection, streptococci are the predominant microorganism, constituting 42% of the total number of isolates from patients in this group. The overall mortality among patients with prosthetic valve endocarditis is high--59%; the mortality among patients with early- or late-onset infections is 77% and 46%, respectively. Most patients with staphylococcal prosthetic valve endocarditis should undergo cardiac valve replacement in addition to antimicrobial therapy. Closely monitored anticoagulant therapy should be cautiously continued in patients with prosthetic valve endocarditis.

Management of complications of infective endocarditis.

Complications of infective endocarditis may be considered as those that involve the heart and adjacent structures or those that are extracardiac. Congestive heart failure is the most common serious complication of infective endocarditis and is the leading cause of death among patients with this infection. In patients with severe heart failure unresponsive to medical therapy after 24 to 48 hours, prompt cardiac valve replacement should be considered, irrespective of the duration of preoperative antimicrobial therapy. We believe that all patients with bacterial infective endocarditis who are stable hemodynamically and who have not had multiple large emboli should receive at least one course of antimicrobial therapy in an attempt to sterilize the infected valve before cardiac valve replacement is considered. Most patients with multiple major embolic events should undergo cardiac valve replacement or debridement of the infected valve. The technical limitations and the experience with two-dimensional echocardiography in patients with infective endocarditis who have valve vegetations demonstrated by echocardiography are not yet sufficient to justify cardiac valve replacement solely on the basis of echocardiographic findings. The highest frequency of major embolic events occurs in association with infections that produce large mobile valve vegetations, such as those caused by Haemophilus parainfluenzae and other slow-growing fastidious gram-negative bacilli, fungi (especially Aspergillus), and nutritionally variant viridans streptococci.

Further echocardiographic observations in pericardial effusion.

Although the presence of fluid behind the left atrium, on echocardiography, has been used to differentiate pleural from pericardial effusions, five cases are reported showing that pericardial fluid can and does accumulate behind the left atrium. Abnormal valvular motion, as a result of free swinging of the heart in the pericardial sac, was also demonstrated in these patients.

Chronic aortic regurgitation: indications for operation--1988.

Timing of operation in a patient with severe aortic regurgitation is a difficult and controversial decision, especially when the patient is asymptomatic or minimally symptomatic. A rational decision can be made when the pathophysiologic features of aortic regurgitation and the natural history of medically treated patients are understood and the benefits and risks associated with aortic valve replacement are known. Proper interpretation of the literature involving echocardiography and nuclear cardiology is essential, as is consideration of the constantly changing surgical techniques and results. Aortic valve replacement should be recommended for those patients with chronic aortic regurgitation who are severely symptomatic (New York Heart Association Functional Class III or IV), in order to ameliorate symptoms and increase longevity. In asymptomatic or minimally symptomatic patients, close continued serial follow-up is necessary in order to detect the onset of resting left ventricular dysfunction and to recommend the optimal timing for surgical intervention.