Nicholas J. Fortuin

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.

Effect of Low-Level Carbon Monoxide Exposure on Onset and Duration of Angina Pectoris: A Study in Ten Patients with Ischemic Heart Disease

Ten volunteers with ischemic heart disease (stable angina) were exposed in a double-blind experiment to 0, 50, or 100 ppM CO for 4 hr prior to treadmill exercise. COHb averaged 1.3, 2.9, and 4.5% just after exposure. CO decreased exercise time to anginal pain and prolonged duration of pain after stopping exercise. 5 of 10 had deeper ST-segment depression.

Exercise-induced S-T segment elevation: Clinical, electrocardiographic and arteriographic studies in twelve patients

S-T segment elevation was observed in the exercise electrocardiograms of twelve patients, eleven of whom had clinical evidence of coronary artery disease. In eight patients the S-T segment changes were observed in anterior precordial leads, and seven of these had evidence of total or near total occlusion of the left anterior descending coronary artery by coronary arteriography. Four patients had elevation in inferior limb leads II and III, and in each of these total or near total occlusion of the right coronary artery was demonstrated. The evidence presented suggests that S-T segment elevation in the exercise electrocardiogram is a manifestation of severe myocardial ischemia, and the electrocardiographic changes will accurately predict the site of the responsible anatomic lesions.

Evaluation of left ventricular function in patients with sickle cell anemia

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.

Hypercontractile cardiac states simulating hypertrophic cardiomyopathy.

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 auscultatory and peripheral pulse abnormalities characteristic of HCM. Postmortem examination 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.

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.

Current Management of Atrial Myxoma with Emphasis on a New Diagnostic Technique

Sixteen patients aged 22–64 years have undergone removal of atrial myxoma at the Johns Hopkins Hospital. The first two patients had myxomas removed by closed surgical approach, and both died. Fourteen consecutive patients have undergone operation with the aid of cardiopulmonary bypass, and all survived. There has been one late death unrelated to myxoma. Current surgical approach is excision of the tumor and its stalk or base and resection of the portion of intra-atrial septum to ensure complete removal. Recently a new diagnostic technique of two-dimensional phased array real time echocardiography has been used in four patients, including a pregnant woman, to confirm the clinical diagnosis of atrial myxoma. This technique provides a two-dimensional view of both atria and ventricles in cross section or sagittal view. This noninvasive method is painless, is independent of cardiac function and carries no risk or radiation hazard. The two-dimensional echocardiogram is superior to the standard one-dimensional or M-mode echocardiogram for diagnosis of myxoma in that it can evaluate simultaneously both right and left atria, can detect smaller space-occupying lesions because of better resolution and can more readily determine the size of the lesion and its stalk, can evaluate the mobility of the tumor and can determine more accurately the extent of tumor obstruction. Because of these qualities, we have relied on two-dimensional echocardiography as the definitive diagnostic procedure for detection of atrial myxoma. Currently operation for atrial myxoma is performed without cardiac catheterization or angiocardiography.

The evaluation of left ventricular function by echocardiography

Ventricular minor-axis dimensions were measured by echocardiography at end-systole (S8) and end-diastole (SD) in five groups of patients: (I) normal; (II) mitral stenosis; (III) compensated volume overload; (IV) idiopathic hypertrophic subaortic stenosis; and (V) congestive heart failure. Cardiac pump function was evaluated by determination of left ventricular volumes and ejection fraction (EF) from the echographic dimensions using formulae previously reported. The mean velocity of circumferential fiber shortening (VCF), a parameter of cardiac muscle performance previously obtained only by invasive methods, was determined from the echographic dimensions by the formula:[See Equation in PDF File]. The duration of minor-axis shortening (dt) was measured directly from the echocardiogram.Dimension and volume measurements in these groups of patients were similar to those reported in similar patients determined by angiographic methods. Measurement of the relative changes in echographic dimensions with systole (%&Dgr;S), EF, and VCF allowed separation of patients with clinical heart failure (group V) from normal subjects (group I). Patients in group II had reduced values for these parameters compared to group I. Those in III did not differ significantly, and those in IV had increased values. In general, VCF, EF, and %&Dgr;S showed similar trends, but individual patients sometimes differed. The findings reported here demonstrate the feasibility of evaluating ventricular function by a noninvasive method in a large group of ambulatory patients.

Echocardiographic mimicry of pericardial effusion

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.