Bernard F. Schreiner

Reversible cardiomyopathy associated with hemochromatosis.

THE patient with familial idiopathic hemochromatosis and severe biventricular failure, described below was returned to normal life by conventional therapy and repeated phlebotomies. Case Report A 3...

Cardiac output in acute myocardial infarction: Serial determination by precordial radioisotope dilution curves☆

Abstract Serial determinations of cardiac output were performed in 16 patients with acute myocardial infarction, utilizing precordial detection of indicator-dilution curves following an intravenous injection of radioiodinated human serum albumin. The evolution of hemodynamic events in these patients is documented, and the clinical data are correlated with concurrent circulatory findings. Results indicate that: (1) Persistently low cardiac index and stroke index were found only in fatal cases. (2) Survivors either maintained a normal cardiac index and stroke index or, if initially observed as abnormal, values for these two parameters generally returned to normal during recovery. (3) Low stroke index was the commonest basic hemodynamic abnormality. Normal values for stroke index were not found in the fatal cases. (4) Ventricular gallop rhythm and tachycardia were found to be reliable clinical guides to the presence of low stroke index. (5) Pulse pressure values were not a useful clinical measure of stroke index.

Familial Mitral Valve Prolapse and Myotonic Dystrophy

When a patient with mitral valve prolapse was found to have myotonic dystrophy, his family was studied to ascertain the occurrence of these abnormalities. Of 25 relatives screened, 8 had evidence of both myotonic dystrophy and mitral valve prolapse and 2 had evidence of myotonic dystrophy alone. Thirteen patients had evidence of neither condition and 1 had mitral valve prolapse alone. The association of these two conditions within a family has not previously been observed and valvular heart disease has not been recognized in myotonic dystrophy. This association should be looked for in other patients.

Pulmonary Blood Volume in Congestive Heart Failure

By utilizing indicator-dilution techniques, determinations of the pulmonary blood volume (PBV) and central blood volume (CBV) were made in 25 patients during simultaneous right and transseptal left heart catheterization. Of these, 12 patients had normal hemodynamics, six had heart failure associated with mitral stenosis, and seven had heart failure secondary to nonvalvular myocardial disease.In the normal patients the PBV averaged 271 ml/m2 (SE, ±10.0) and the CBV 596 ml/m2 (SE, ±25.7). The mean value for PBV and CBV in the patients with heart failure associated with mitral stenosis was 238 ml/m2 (SE, ±26.3) and 600 ml/m2 (SE, ±40.9), respectively. Among the patients with nonvalvular heart failure the PBV averaged 292 ml/m2 (SE, ±21.4) while the CBV averaged 679 ml/m2 (SE, ±45.8). In 12 of the 13 patients with heart failure, the individual values for PBV and CBV did not differ significantly from normal. However, in each of the 13 patients pulmonary artery, left atrial, and mean pulmonary distending pressures were significantly elevated, whereas the relative pulmonary vascular compliance was greatly reduced.These findings indicate that the pressurevolume relationships within the pulmonary vascular bed are greatly altered in the presence of chronic pulmonary venous hypertension. Normal values for PBV were associated with significant pressure elevations in the presence of pulmonary congestion and edema.It is concluded that in the pathogenesis of heart failure elevations in pulmonary vascular pressures play a dominant role while changes in PBV are of but secondary importance. These findings do not support many of the traditional concepts regarding the role of pulmonary blood volume changes in the pathophysiology of heart failure.

Acute effects of increase in pulmonary vascular distending pressures on pulmonary blood volume and pulmonary extravascular fluid volume in man.

The acute hemodynamic effects of supine leg exercise or atrial pacing were studied in 114 patients undergoing right and either transseptal (87 cases) or retrograde (27 cases) left heart catheterization. Seventy-one patients – 15 with coronary artery disease, 22 with aortic valve disease, and 34 with mitral valve disease – performed exercise on a bicycle ergometer. Forty-three patients, of whom 22 had coronary artery disease, nine aortic valve disease and 12 mitral valve disease, were studied during rapid atrial pacing. Cardiac index (CI), pulmonary artery mean (PAm), and left atrial mean (LAm) pressure, pulmonary blood volume (PBV) and pulmonary extravascular volume (PEV) were measured during the control state and during acute intervention. Both exercise and pacing resulted in significant elevations in PAm (range 37–65%) and LAm (range 36–43%) mean pressures in all patients. Cardiac index rose between 34 and 58% in the exercise groups, but did not change in those who were paced. During intervention both PBV and PEV increased significantly in all but the nine patients with aortic valve disease who were paced. Although volume increases occurred, they did not achieve the 5% significance level. For all patients the mean increment in PBV ranged between 37 and 123 ml/ml over control, while PEV rose between 15 and 35 ml/m2. In each group the increases in PEV and PBV were proportionate, so that the ratio of PEV/PBV did not change significantly between the control and intervention states. Thus PEV and PBV increases occurred with elevations in pulmonary vascular pressures whether or not blood flow increased. Our data in patients with normal pulmonary vascular beds (i.e., coronary artery disease and aortic valve disease) strongly support the hypothesis that recruitment of vascular channels accounts for the acute changes in PEV and PBV and that the changes in PEV over a brief period of time do not necessarily reflect a “true” increase in extravascular lung water. Although pressures are higher in the lungs of patients with mitral valve disease, the data also suggest that recruitment is likely to be the mechanism for the observed proportionate increase in pulmonary extravascular volume and pulmonary blood volume.

Surgical Management of an Aneurysm of the Left Main Coronary Artery

A 28-year-old woman underwent cardiac catheterization three months after sustaining an acute anterolateral myocardial infarct. An aneurysm of the left main coronary artery was found at angiography and also was visualized by two-dimensional echocardiography. The patient had no evidence of atherosclerotic vascular disease. At operation, vein grafts were placed to bypass the aneurysm and the orifice of the left coronary artery was oversewn to exclude the aneurysm from the coronary circulation.

Radiation technique for production of localized myocardial necrosis in the intact dog.

Summary The radiation effects of 2,500 to 20,000 roentgens on the canine myocardium have been studied. A collimated x-ray beam, 2.54 cm in diameter, was delivered to the heart of 12 dogs at a rate of 210 roentgens per minute. A localized area of myocardial necrosis was evident by the 7th to 10th post-irradiation day in the hearts which received a dose of 20,000 roentgens. Evolution of the myocardial lesion was documented electrocardiographically, and by pathologic examination. Microscopically, the cardiac necrosis was strikingly similar to vascular infarction in the human myocardium. The use of this radiation technique for experimental production of localized myocardial necrosis is discussed.

Heart Block and Idiopathic Pulmonary Hemosiderosis

A young woman with idiopathic pulmonary hemosiderosis (IPH) and advanced heart block below the atrioventricular node requiring pacemaker therapy is discussed. Thirteen years after the onset of IPH she is free of pulmonary symptoms despite having never received steroid or antimetabolite therapy.