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Congestive heart failure (CHF) increases with age, but most CHF in the elderly is due to diastolic dysfunction with preserved systolic function. The etiology, pathophysiology, diagnosis, natural history, and treatment of hypertrophic and restrictive cardiomyopathies in the elderly are discussed as a paradigm for CHF with normal systolic function. Hypertrophic obstructive and hypertensive hypertrophic cardiomyopathies are compared and contrasted. As an example of a restrictive cardiomyopathy, the various types of amyloidosis and their clinical import in older patients are covered.

We have observed a group of patients with mitral valve disease and severe symptoms but also with low transmitral gradients and normal cardiac outputs who defy the traditional hemodynamic explanation of mitral stenosis. We performed a 10-year retrospective chart review of all mitral valve replacements at our institution to further characterize this population. The study group consisted of 16 of 132 patients (12%) with symptomatically severe (New York Heart Association [NYHA] class 3.3 +/- 0.5) mitral stenosis but a low (< 10 mm Hg) transmitral gradient, a normal cardiac output (4.8 +/- 1.2 L/min), and a preserved valve area (1.6 +/- 0.4 cm2). Sixteen patients were randomly chosen from the remaining group to serve as a comparison population. Study patients were noted to have less atrial fibrillation, lower wedge and mean pulmonary artery pressures, and a higher incidence of subvalvular disease identified at the time of surgery than did the comparison population. Left ventricular end-diastolic pressure and cardiac output did not differ. Study patients did well with surgery and reported an excellent functional benefit. We believe that this subgroup of patients with mitral valve disease is important, may be missed by using conventional criteria of valve area to determine timing of surgical intervention, and may have their symptoms primarily because of subvalvular disease. Further and perhaps most important, this group illustrates the ongoing need for careful clinical assessment skills and judgement in the face of ever-increasing technology.

Echocardiographic abnormalities of the mitral valve and aortic root were compared with auscultatory findings and with assessment of aortic root size by chest roentgenography in 61 patients with the Marfan syndrome. Echocardiography was more sensitive than physical examination in detecting valvular and aortic root abnormalities. Although physical examination revealed findings of mitral valve disease and/or of aortic regurgitation in 52 percent of patients (mitral valve disease in 44 percent and aortic regurgitation in 23 percent), echocardiography detected abnormalities of the mitral valve and/or aortic root in 82 percent of patients (mitral valve prolapse in 57 percent and aortic root enlargement in 69 percent). Prevalence of mitral valve prolapse was approximately equal in male and female patients, whereas aortic root enlargement was more frequent in males (83 percent) than in females (50 percent). Echocardiographically detected aortic root enlargement was frequently not apparent on chest x-ray films. Indeed, five patients with markedly increased aortic root diameters (ranging from 6.0 to 7.9 cm) had no evident enlargement of the aortic root on routine chest x-ray films. In all four of those patients who had angiographic and/or pathologic correlations, confirmation of marked aortic root dilatation was obtained. There are limitations to echocardiographic evaluation of the presence and severity of underlying cardiovascular disease in patients with the Marfan syndrome. Mitral valve disease may not be detected, especially in patients with left ventricular dilatation. In addition, due to anteroposterior compression of the left atrium by the enlarged aorta, left atrial size may be underestimated in patients with aortic root enlargement.

This paper provides guidelines for the optimal, rather than minimal, performance of ultrasonic examination of the heart for current clinical applications using conventional echocardiographic equipment. Since the original report of this Inter-Society Commission on Heart Disease Resources Committee in 1975, M-mode echocardiography has continued to be a valuable clinical tool, and two-dimensional echocardiography has been developed. Guidelines are presented for optimal physician and cardiac sonographer training, case loads, space and support systems, equipment design features and performance testing, and administrative considerations. The developing area of Doppler ultrasound and the competitive technologies are noted.

Thirty-three patients who had aortic valve replacement (AVR) for isolated aortic stenosis (AS) were retrospectively evaluated to determine the influence of preoperative ventricular function on the results of operation. Patients were arbitrarily divided by ejection fraction (EF) into Group I having EF 0.46 or above and Group II having EF 0.45 or below. Group II had significantly lower mean EF and cardiac index and higher left ventricular end-diastolic volume index and end-diastolic pressure. There were two early and no late deaths in 21 Group I patients. There were no early and five late deaths in 12 Group II patients. All Group I survivors and six of 12 Group II patients were New York Heart Association (NYHA) functional Class I or II at follow-up evaluation. No preoperative hemodynamic index was predictive of which Group II patients would do well. Mortality was substantially higher in patients with severe preoperative disability. Early or late death occurred in five of 12 patients who were NYHA functional Class IV before operation but in only two of 21 patients who were functional Class II or III preoperatively. We conclude that patients with isolated AS and poor left ventricular function have a low hospital mortality but an increased risk of late death or poor functional result following AVR. Excellent functional results can be obtained after AVR in most patients with AS and good left ventricular function and approximately half of the patients with AS and poor left ventricular function.

Echocardiography is a valuable technique for the diagnosis and serial follow-up of patients with impaired cardiac function. It is subject to certain limitations due to the assumptions inherent in deriving ventricular volume from a one-dimensional measurement and must be interpreted with caution in cases of suspected regional abnormalities of contraction. Given these caveats, echocardiography is valuable in the quantitative assessment of cardiac size and the level of compensation in patients with primary myocardial disease, valvular heart disease, and left ventricular hypertrophy. It can detect abnormal contraction in some patients with ischemic heart disease and provides an accurate method to serially follow changes produced as a result of drug or surgical therapy. Finally, two-dimensional techniques promise to provide a new perspective on the evaluation of patients with regional wall motion abnormalities.

The noninvasive diagnosis of left ventricular aneurysm has markedly improved with gated blood pool scintigraphy. However, in patients with giant anterior ventricular aneurysms, the gated blood pool scintigram performed in two standard views (anterior and 40 degree left anterior oblique) may incorrectly suggest ischemic cardiomyopathy. We retrospectively identified five patients who underwent resection of a ventricular aneurysm over a 2 1/2 year period and who had preoperative scintigraphic studies that appeared to show severe diffuse left ventricular dysfunction. contrast ventriculography demonstrated preserved wall motion in septal, inferior and lateral segments not seen by gated blood pool scintigraphy and showed extraordinarily large anterior aneurysms. M-mode or two-dimensional echocardiograms showed intact posterior wall function in all patients, suggesting severe regional myocardial disease rather than global dysfunction. Two-dimensional echocardiography showed additional segments with preserved function as well as discrete aneurysms in all patients. We conclude that gated blood pool scintigraphy, when performed in two standard views, may fail to correctly diagnose some patients with very large anterior wall aneurysms. M-mode echocardiography, two-dimensional echocardiography and additional scintigraphic views that visualize the posterior portions of the left ventricle improve noninvasive diagnosis of patients with resectable giant left ventricular aneurysms.

Hypertrophic cardiomyopathy (HCM) or idiopathic hypertrophic subaortic stenosis (IHSS) has been defined as an autosomal dominant myocardial disease characterized by specific physical findings, echocardiographic features, asymmetric septal hypertrophy and disordered myocardial architecture. Echocardiographic and scintiphotographic studies failed to reveal evidence of asymmetric septal hypertrophy in four patients with systolic anterior movement of the mitral valve and the typical ausculatory and peripheral pulse abnormalities characteristic of HCM. Postmortem examimination in one patient demonstrated mild concentric left ventricular hypertrophy and a normal arrangement of myocardial muscle fibers. These observations in four patients demonstrate that both systolic anterior movement of the mitral valve and the physical findings characteristic of HCM are not specific for the autosomal dominant myocardial disease characterized by asymmetric septal hypertrophy and abnormal septal histology.

Four patients are reported who underwent repair of partial anomalous pulmonary venous drainage with intact atrial septum. One patient also had azygos continuation of the inferior vena cava and two patients had associated mitral stenosis. Diagnostic considerations and guidelines for operative repair are presented.

The families of 12 probands with classic mitral valve prolapse were studied for evidence of mitral valve prolapse. Seventy parents, sibs, and progeny were included in the analysis. Forty-seven percent (16 of 34) of progeny were affected compared with 30 percent (3 of 10) of parents. Thirty-eight percent (10 of 26) of sibs were affected. A three-compartmental penetrance model was devised to account for the variation in expression with age. This includes a latent stage (time before onset of signs), an affected stage, and a stage in which the subjects are withdrawn (because of treatment, regression, or death). The implications of this model are discussed.

Echocardiographic evidence of left ventricular hypertrophy with well-preserved systolic function was established in 4 chronic hemodialysis patients with a history of refractory pulmonary congestion. In each patient a trial of treatment with verapamil (40 mg t.i.d.) resulted in symptomatic improvement, an increase in nadir intradialysis blood pressure, and a marked reduction in intradialytic symptomatic hypertensive episodes and the need for intravenous saline administration. These data suggest that (a) a subset of chronic hemodialysis patients with refractory congestive heart failure and dialysis-related hypotension have hypertrophic cardiomyopathy with well-preserved systolic function, and (b) such patients may derive greater benefit from verapamil treatment than from the more conventional approach to drug therapy with digoxin and nitrates.

Previously reported cardiac manifestations of carcinoid disease include right-sided valvular dysfunction and vasomotor disturbances. This report describes a 62-year-old white man who had electrocardiographically documented widespread elevation of the S-T segment, arrhythmias, and cardiac arrest after exercise. Noninvasive and laboratory studies confirmed the diagnosis of malignant carcinoid disease with tricuspid valve involvement. Coronary angiography revealed mild atherosclerosis. Thus, coronary artery spasm is a previously unrecognized manifestation of carcinoid heart disease and introduces what may be an important relationship of endogenous, excessive vasoactive amines to human coronary vasospasm.

The echocardiographic measurements of cardiac chamber dimension, ejection phase indices of left ventricular function and the systolic time intervals of 23 adult patients with sickle cell anemia were compared to those of normal control subjects. Patients with sickle cell anemia had a significantly greater mean left ventricular systolic dimension index, left ventricular diastolic dimension index, left ventricular mass, stroke volume index, interventricular septal width, aortic root index and left atrial index. No significant differences were noted between the mean velocity of circumferential fiber shortening, ejection fraction or systolic time intervals. The anemic population was divided into two groups; one consisting of patients less than 30 years old and the other of patients over 30 years old. There was no significant differences between the ventricular dimensions, velocity of circumferential fiber shortening, ejection fraction and systolic time intervals of the two groups. These data indicate that the chronic volume overload of sickle cell anemia is well tolerated without development of left ventricular dysfunction.

Echocardiography has proven a useful and sensitive means to determine noninvasively the cardiac dimensions. This report describes the echocardiographic detection of progressive dilatation of the aortic root over an 18-month period, which led to death in a 33-year-old man with Marfan's syndrome; however, at no time was the aortic dilatation evident on chest x-ray films. This patient illustrates the sensitivity of the echocardiogram in detecting and assessing the severity of disease of the aortic root not readily assessed by other noninvasive techniques and the value of serial measurements of aortic dimensions in patients with proven or suspected dilatation of the aortic root.

Ten patients with roentgenographically demonstrable mitral annulus calcification (MAC) were found to have distinctive echocardiographic patterns. MAC was confirmed at the time of cardiac catheterization in six of these patients. Standard M-mode echocardiograms revealed a dense band of echoes in the region of the mitral annulus in contrast to the thin and delicate echoes generally recorded from the normal mitral annulus. Intraoperative and pathologic confirmation of thickening and calcification limited to the mitral annulus was made in two patients who underwent mitral valve replacement for severe mitral regurgitation due to myxamatous "floppy" valve. Patients with marked MAC may have coexisting aortic valve or papillary muscle calcification which can be recognized by echocardiography. Over-attenuation of left ventricular wall echoes in patients with marked MAC, and reduction in E-F slope of the anterior mitral valve leaflet in others can simulate pericardial effusion and mitral stenosis, respectively. Thus, recognition of MAC can avoid confusion with similar echocardiographic patterns due to other common cardiac abnormalities.

Although mitral valve prolapse is often associated with a systolic click or murmur, it is not widely appreciated that a sound or murmur may also occur in diastole. Nine patients with a systolic click or murmur and echocardiographic evidence of mitral prolapse had, in addition, a diastolic sound or an early diastolic murmur best heard at the apex or left sternal border. The sound, which was of high frequency and easily audible, followed A2 by 70-110 msec (mean 94 +/- 5 msec), and coincided with the point where the prolapsed posterior leaflet returned from the left atrium and recoapted with the anterior mitral leaflet. The diastolic sound occurred 40-60 msec (mean 53 +/- 4 msec) before the E point of the echocardiogram and O point of the apexcardiogram, and even longer before the rapid-filling wave. The diastolic murmur, also of high frequency, was brief and decrescendo, and simulated aortic regurgitation in two patients. Thus, mitral prolapse may be associated with a sound or murmur in diastole. When a diastolic sound or murmur is best heard apically, even if accompanied by a systolic murmur, mitral valve prolapse should be considered.

Using echocardiography, we identified 21 patients with a syndrome that included severe concentric cardiac hypertrophy, a small left ventricular cavity, and supernormal indexes of systolic function without concurrent medical illness or ischemic heart disease. Thirteen of the patients presented with dyspnea or chest pain. All patients studied had a history of hypertension and were compared with normotensive controls matched for age and sex. The patients were elderly (mean age, 73.3 years), predominantly female (16 patients), and mostly black (15 patients). Their cardiac function was characterized by excessive left ventricular emptying (ejection fraction on two-dimensional echocardiography [patients vs. controls], 79 +/- 4 vs. 59 +/- 5 per cent, P less than 0.001) and abnormal diastolic function as manifested by a prolonged early diastolic filling period (279 +/- 25 vs. 160 +/- 45 msec, P less than 0.001) and reduced peak diastolic dimension increase (11 +/- 4 vs. 16 +/- 5 cm per second, P less than 0.05). In spite of the clinical presentation of heart failure, all of 9 patients receiving either beta-receptor antagonists or calcium-channel blocking agents obtained symptomatic relief, whereas 6 of 12 patients receiving vasodilator medications had severe hypotensive reactions, including one death. We conclude that this unique subset of hypertensive patients has a clinical syndrome that warrants recognition and tailored management.

Mitral valve area (MVA) determined at cardiac catheterization was compared with M mode echocardiographic measurements in 44 patients with mitral stenosis and no substantial mitral regurgitation. Despite statistically significant correlations, measurements of anterior leaflet motion, including rate of diastolic closure (EF slope) were not useful in predicting severity of stenosis. In contrast, maximal diastolic separation of anterior and posterior leaflets (SEP) was more closely correlated with MVA and appears to have some predictive value. Narrow separation was associated with severe mitral stenosis. Wide separation was associated with relatively mild stenosis. Intermediate values in 16 of 44 patients (36%) were not of predictive value. Recognizing this limitation, measurement of maximal diastolic mitral leaflet separation from M mode echocardiograms is proposed as a simple and useful method for assessing severity of mitral stenosis.

We report a patient who developed aortic regurgitation and first degree atrioventricular block caused by infective endocarditis complicating aortic valve stenosis. There was premature closure of the mitral valve and, in the absence of an Austin Flint murmur, a simultaneous high frequency sound was audible which we regard as a presystolic first heart sound. That such a sound may be associated with valve closure, even though this precedes electrical and mechanical ventricular systole, provides further support for the valvular origin of the first heart sound.

The echocardiographic diagnosis of mitral stenosis is based on the finding of a decreased early diastolic slope of the anterior mitral leaflet. This finding is also seen in other conditions in which the rate of left ventricular filling is reduced by decreased compliance of the ventricular myocardium rather than by mitral valve obstruction. Patients with "true" mitral stenosis have been differentiated from those with decreased ventricular compliance resulting in "false" mitral stenosis by the direction of movement of the posterior mitral valve leaflet. This report describes a patient with mitral stenosis proved at cardiac catheterization whose echocardiogram showed posterior motion of thickened posterior mitral leaflet during diastole, a finding previously considered to exclude organic mitral stenosis. This false negative echocardiographic finding in proved mitral stenosis has not previously been reported.

The echocardiographic examination is described and the current status of ultrasound in cardiac diagnosis is summarized. Planning guidelines are provided for hospital based echocardiography laboratories including resource criteria for professional personnel and training, equipment, space, and support systems. Minimal case loads for maintaining quality performance of the examiner are recommended and various administrative patterns for organizing an echocardiography service are discussed. Approaches for establishing professional fees and emerging applications of diagnostic ultrasound in cardiovascular medicine are briefly reviewed. There is a description of the procedure for conducting an adequate echocardiographic examination in children and adults.

Echocardiography is a sensitive technique for the detection of pericardial effusion, but the abnormal echocardiographic patterns seen with effusions are not, however, entirely specific for that diagnosis. This study describes four patients in whom anatomic structures, a coronary artery to coronary sinus fistula (one case) and tumors metastatic to pericardium (three cases), produced posterior and, in two cases, anterior spaces compatible with pericardial fluid. Echocardiographic patterns mimicking pericardial effusion have previously been reported in patients with anatomic abnormalities such as mitral anular calcification, pleural effusions, left atrial enlargement, anterior mediastinal or pericardial tumors, foramen of Morgagni hernia and pseudoaneurysm of the left ventricle. It appears that structures of fluid or tissue density, interposed between the heart and the airfilled lung, can produce echocardiographic patterns simulating pericardial effusion.