Hypertrophic and restrictive cardiomyopathies in the elderly.

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.

Severely symptomatic mitral stenosis with a low gradient: a case for low-technology medicine.

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.

Calcium channel blockade in dialysis patients with left ventricular hypertrophy and well-preserved systolic function.

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.

Hypertensive hypertrophic cardiomyopathy of the elderly.

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.

Coronary artery spasm and cardiac arrest in carcinoid heart disease.

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.

Partial anomalous pulmonary venous return with intact atrial septum: report of four cases.

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.

Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model.

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 assessment of cardiovascular abnormalities in the Marfan syndrome. Comparison with clinical findings and with roentgenographic estimation of aortic root size.

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.

Presystolic mitral closure sound in aortic regurgitation with left ventricular hypertrophy and first degree heart block.

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.

Optimal resources for ultrasonic examination of the heart. Echocardiography study group.

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.

Clinical recognition of giant left ventricular aneurysm.

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.

Comparative analysis of noninvasive cardiac parameters in the detection and evaluation of adriamycin cardiotoxicity.

Serial electrocardiograms, phonocardiograms and echocardiograms were recorded in a prospective study of 45 closely-followed patients receiving chemotherapy with Adriamycin (doxorubicin hydrochloride, Adria Labs.). QRS voltage, systolic time intervals (STI), echocardiographic ejection fraction (EF) and rate of ventricular circumferential fiber shortening (Vcf) were compared as indicators of Adriamycin cardiotoxicity. Seven patients (16%) developed a decline in left ventricular function. Four of these seven patients (57%) developed symptoms and signs of congestive heart failure (CHF). The pre-ejection period/left ventricular ejection time (PEP/LVET) was earliest to change and was the least specific of the noninvasive parameters. The ejection fraction was the most specific parameter in predicting clinical cardiotoxicity. In every case of congestive heart failure, significant changes in ejection fraction, Vcf and PEP/LVET preceded the onset of symptoms, suggesting that measurement of the ejection fraction and systolic time intervals will allow early prediction and avoidance of heart failure. A fall in the ejection fraction of greater than or equal to 10% may represent sufficient grounds for discontinuing Adriamycin.

Diastolic sounds and murmurs associated with mitral valve prolapse.

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.