Stanley J. Goldberg

Persistent pulmonary vascular obstruction in newborn infants

Severe pulmonary vascular obstruction in the absence of obvious pulmonary parenchymal pathology was found in 5 full-term neonates and was characterized clinically by cyanosis and tachypnea. Cardiac catheterization and cineangiography at age 32 to 55 hours revealed pulmonary hypertension with right-to-left shunt through the ductus arteriosus in each instance. One infant died and autopsy revealed only a patent ductus arteriosus and marked medial hypertrophy of the muscular pulmonary arteries. Clinical resolution of pulmonary vascular obstruction occurred within 4 weeks in the survivors; this was confirmed in one infant by recatheterization. The possible causes of the persistence of pulmonary vascular obstruction are discussed.

A comparison of the maximal endurance of normal children and patients with congenital cardiac disease

A technique to measure maximal endurance in children is described, and the results of its application to normal children and to those with congenital cardiac lesions are recorded. The children with heart disease were unable to achieve pulse rates as high as could the normal ones, and had considerably less endurance. The maximal endurance score of a child with cardiac disease tended to reflect his functional status as indicated by cardiac catheterization studies.

The effects of meperidine, promethazine, and chlorpromazine on pulmonary and systemic circulation

Abstract The effects of meperidine, promethazine, and chlorpromazine (Demerol, Phenergan, and Thorazine) were studied in the intact, unanesthetized dog. MPC caused significant pulmonary vasoconstriction and systemic vasodilation in this preparation. If MPCs pharmacologic properties in humans are equivalent to those found in the present study, its suitability as a precardiac catheterization agent in some forms of cardiac disease may be open to question.

Maximal exercise capability of children as a function of specific cardiac defects

Abstract The results of a maximal exercise test are summarized for 288 patients with isolated cardiac lesions and 234 normal children. All groups of patients had significantly different scores from those of normal children, but each lesion had a characteristic pattern. Data are given for patients with aortic stenosis, tetralogy of Fallot, ostium primum and secundum atrial defects, patent ductus arteriosus, ventricular septal defects, pulmonary stenosis, coarctation of the aorta, and insufficiency of the mitral or aortic valve. The results are presented graphically in a manner that permits comparison of a given child to others with similar defects and to normal children. It is suggested that the maximal endurance test may be helpful in selecting catheterization candidates for special investigations and in following the clinical courses of certain patients.

Tricuspid Atresia with Increased Pulmonary Blood Flow An Analysis of 13 Cases

The pathologic and clinical findings in 13 cases of tricuspid atresia and increased pulmonary blood flow encountered in UCLA Medical Center were reviewed. In seven, the great vessels were normally related (group I), and in six, the great vessels were transposed (group II). In all group II cases, aortic arch anomalies were encountered, a finding not duly emphasized in previous reports. Comparison of the two groups revealed significant clinical and laboratory differences. Group II patients typically presented with severe congestive heart failure and expired within the first 2 months of life. Group I patients usually presented with mild congestive heart failure which responded to therapy. Five of the latter demonstrated progressive change from increased to decreased pulmonary blood flow during their clinical course and eventually required palliative shunt procedures, four prior to the age of 2 years and one at 5 years of age.While the electrocardiogram demonstrated right atrial enlargement and left ventricular hypertrophy in both groups, the mean frontal plane axis was between −15° and −100° in group I and −75° and +75° in group II. QRS vector loops tended to be superiorly oriented in group I and inferiorly oriented in group II.Because the natural history and prognosis in these two groups differ significantly, diagnostic efforts should include determination of the relationship of the great vessels. When transposition of the great vessels is demonstrated, aortography should be performed because of the high incidence of aortic arch anomalies.

Fatality Immediately Following Rapid Infusion of Macroaggregates of 99mTc Albumin (MAA) for Lung Scan

LUNG SCANNING has recently gained increasing acceptance as a diagnostic aid in pulmonary disease since it supplies information which is in some ways similar to that obtained by pulmonary angiography (1–4) and is performed with less technical difficulty and risk (5, 6). To date only one adverse reaction has been reported (7, 8). The purpose of this report is to record an immediate and fatal reaction following infusion of 99mTc-MAA (technetium-labeled MAA). The possible relationship between our patients advanced pulmonary vascular disease and the fatal reaction will be discussed. Case Report In this 7-year-old Caucasian male, a thoracolumbar meningomyelocele had been diagnosed at birth and a ventriculojugular shunt procedure performed to alleviate noncommunicatirig hydrocephalus. The shunt functioned well until the child was six and a half years of age when it required revision. At that time a chest film demonstrated normal cardiac size and contour. Fifteen months later, persistent dependent edema develope...

Cardiac Output as a Function of Ventricular Rate in a Patient with Complete Heart Block

Hemodynamic effects of varying heart rate and activity have been studied in a young woman in good health except for complete heart block. As cardiac rate was increased from 50 to 110 beats per minute by means of an external cardiac pacemaker, cardiac output and stroke volume were found to reach maximum values at a rate of 75. Stroke volume increased 43 per cent with exercise when heart rate was fixed at 68 beats per minute. The ability of this patient to increase stroke volume with exercise, in contrast to average normal subjects, may be related to her past experience with chronically increased volume loads on the heart secondary to heart block.

Angiographic demonstration of the ventricular septum. A new technic.

In the cardiac disease, idiopathic hypertrophic subaortic stenosis (IHSS) (1), there is hypertrophy of the myocardium which is often asymmetrically distributed. Although published descriptions appeared earlier, it was not until a clinical report by Brock (3) in 1957 and a pathologic report by Teare (10) in 1958 that the disease became well known. Since that time many other papers have appeared. The disease is much more common than was first realized. Most of the authors describing their cases emphasize that the often massive myocardial hypertrophy may primarily involve the ventricular septum (2, 4, 8, 10, 11), although usually the free wall of the left ventricle and sometimes the right ventricle are involved as well. Frequently the first manifestation of the disease is a heart murmur. The most common symptoms are dyspnea and angina. The patient is often asymptomatic, however. The electrocardiogram is almost always abnormal (2). At catheterization the demonstration of a pressure gradient within the left ve...

Pulmonary and systemic hemodynamic effects of cardiac glycosides

any physiological actions of digitalis glvcosides have been elucidated only recently although their effects in reversing congestive heart failure and slowing the heart rate have long been known.1-3 These drugs increase myocardial contractility and produce generalized vasoconstriction with consequent increase in systemic resistance. In some species, they also evoke constriction of the hepatic veins, with resultant splanchnic pooling. These actions are similar in normal subjects and those with congestive cardiac failure, but cardiac output varies with the way in which venous return is altered. In contrast to the extensive information on cardiac and systemic actions, little is known of the way in which digitalis glycosides affect the lung circulation.4-6 Because of the potential importance of digitalis-induced vasoconstriction on the pulmonary vasculature, this report concerns the effects of acetyl strophanthidin, strophanthidin (ouabain), and digoxin on the pulmonary as well as systemic circulation of the intact unanesthetized dog. In additional experiments, to eliminate the possible role of altered right or left heart dynamics, a preparation was devised in which a mechanical pump replaced the right heart and maintained pulmonary blood flow constant. Concurrently, left atria1 pressure was maintained at con-trol level after glycoside injection.

Subpulmonic Obstruction in Complete (d) Transposition Produced by Redundant Tricuspid Tissue

Two cases are reported in which complete transposition of the great vessels was associated with an unusual form of subpulmonic obstruction. In each case, redundant tricuspid valvular tissue protruded through a ventricular septal defect into the left ventricular outflow tract, producing severe obstruction to the outflow of blood from the left ventricle.The clinical course suggested the presence of left ventricular outflow obstruction. Both patients demonstrated progressive clinical deterioration characterized by increasing cyanosis, respiratory distress, and decrease in intensity of the cardiac murmur. Serial laboratory determinations revealed increasing hemoglobin and decreasing systemic oxygen saturation. The clinical deterioration was relieved only temporarily by atrial balloon septostomy.The angiographic findings appear to be specific for this type of subpulmonic obstruction. In each case, a large asymmetric filling defect was demonstrated at the anterior border of the left ventricular outflow tract below the pulmonic valve.When the clinical and laboratory findings suggest pulmonary or subpulmonary obstruction, careful evaluation of the outflow tract is indicated. If diagnosed clinically, redundant tricuspid tissue could perhaps be resected during a definitive operation, thus relieving the outflow obstruction.