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Biomedical subjects

W F Friedman

Publications and source records attributed to W F Friedman.

At least 55 records · Page 3Linked to original sources

Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome.

The most common cardiac defect in Turner syndrome has been described previously as coarctation of the aorta. We have evaluated 35 consecutive patients with Turner syndrome by clinical examination and by M-mode and two-dimensional echocardiography. Twelve patients (34%) had isolated, nonstenotic bicuspid aortic valve. A high correlation (82%) existed between the presence of a systolic ejection click and echocardiographic evidence of a bicuspid aortic valve. These data indicate that bicuspid aortic valve may be the most common cardiac anomaly in Turner syndrome.

Adolescent↗

The cardiovascular effects of metrizamide in infants.

A prospective study was performed in 30 children under 3 years of age to compare the cardiovascular effects of a nonionic contrast material of low osmolality, metrizamide, with those of a conventional ionic contrast material, meglumine sodium diatrizoate. Left ventricular end-diastolic pressure, left ventricular peak systolic pressure, heart rate, echocardiographically obtained end-diastolic and end-systolic dimension, and blood chemistries were obtained before and after angiography. Neither contrast material changed serum sodium, potassium, or creatinine levels. However, serum osmolality rose significantly following injection of diatrizoate, but not metrizamide. Both end-diastolic and end-systolic dimensions increased after diatrizoate injection. However, end-diastolic dimension was unchanged and end-systolic dimension fell after metrizamide infusion. Both contrast materials raised left ventricular peak systolic pressure and left ventricular end-diastolic pressure after injection; the magnitude of these pressure changes was the same for both agents. Heart rate increased with diatrizoate to a significantly greater degree than with metrizamide. There was no difference in the quality of radiographic opacification. It is concluded that although the effects on intracardiac pressures are similar for both contrast materials, metrizamide may be advantageous in the critically ill infant because it causes a smaller increase in osmolality, fewer changes in cardiac dimensions, and a reduced heart rate challenge.

Angiocardiography↗

Ventricular and pulmonary artery volumes in patients with absent pulmonary valve. Factors affecting the natural course.

Right and left ventricular (RV and LV) volumes were determined in 19 patients with absent pulmonary valve syndrome using Simpson's rule and area-length methods. The volume of the proximal right pulmonary artery (RPAV) was calculated at maximal and minimal size using the area-length method. Patient groups included four newborns who responded to medical management (group 1A), seven critically ill newborns who died (group 1B), four infants ages 1-10 months (group 2) and four children ages 2-8 years (group 3). The RV end-diastolic volume in groups 1B and 2 was significantly greater than that in groups 1A and 3. The RV ejection fraction in groups 1B and 2 was significantly less than that in normal patients and groups 1A and 3. Maximal RPAV correlated well with RV stroke volume and end-diastolic volume. The maximal RPAV in group 1B was significantly greater than that in groups 1A and 3. Pulmonary arterial compliance was greater than normal in all groups, and the compliance in group 1B was more than two times that in the other groups. We conclude that the increased right pulmonary artery compliance and pulmonary regurgitation in patients with absent pulmonary valve contribute to bronchial obstruction and right-heart failure and are the causes of the high morbidity and mortality in these patients. The management should be directed to the alleviation of bronchial obstruction and right-heart failure.

Cardiac Volume↗

The vasoactivity of the fetal lamb ductus arteriosus studied in utero.

The ductus arteriosus of the undisturbed fetal lamb was studied chronically by techniques that allowed direct serial measurements of the calibre of the fetal channel. When the direct actions were studied of vasoactive agents on the ductus arteriosus, prostaglandins did not dilate the vessel beyond its resting dimensions. The cyclooxygenase inhibitor, indomethacin, was a potent vasoconstrictor with a plateau of the dose-effect relationship occurring at 0.2 mg/kg. Fetuses of 95--98 days gestational age were equally sensitive to indomethacin when compared to animals near term. Although the prostaglandins, PGE1, PGE2 PGF2 alpha, PGH2, PGI2 and PGG2, had no direct effect on the ductus arteriosus, PGE1, PGE2, PGH2 and PGI2 reversed the vasoconstrictor action of indomethacin. Autonomic nervous system mediators and blockers, i.e., acetylcholine, atropine, norepinephrine, propranolol, phentolamine and methoxamine, had neither direct effects on the ductus arteriosus nor any influence of indomethacin-induced vasoconstriction. The same was true of angiotensin 1, angiotensin 2, blockade of conversion of angiotensin 1 to 2, serotonin, methysergide, aminophylline, adenosine, and dibutyryl cyclic AMP. Imidazole, a blocker of thromboxane synthase, had no direct effect on the ductus arteriosus, but reduced significantly the magnitude of the indomethacin constrictor action. The major finding of this investigation was the exquisite sensitivity of the ductus arteriosus to manipulations of the prostaglandin environment. The results suggest that both locally generated prostaglandins, as well as prostanoids in the circulation, may be involved in ductal patency and closure. A high degree of control is likely of the circulation of the ductus arteriosus and constriction of the fetal channel is probably the result of an active process.

Adenosine↗

From cyanotic infant to acyanotic adult - the odyssey of blue babies.

An edited summary of an Interdepartmental Conference arranged by the Department of Medicine of the UCLA School of Medicine, Los Angeles. Director of Conferences: William M. Pardridge, MD, Associate Professor of Medicine.In the past two decades we have witnessed the maturing of diagnostic and surgical skills in the management of congenital heart disease. Although longevity and quality of life have improved, cures are few; varying degrees of postoperative medical supervision are therefore needed. This new patient population of adults requiring long-term medical care continues to increase. Proper management of such patients can be taxing, requiring knowledge not only of the preoperative disease but also of the nature and effects of surgical intervention and of the presence, type and extent of late postoperative residua and sequelae. The tetralogy of Fallot is taken as a model because it is well known in both pediatric and adult medicine, because intracardiac repair includes a wide range of techniques and because postoperative residua and sequelae comprise a broad spectrum of patient care concerns.

Adult↗

Echocardiography in congenital heart disease.

The combination of M-mode and two-dimensional echocardiography provides precise anatomic and helpful functional information for most types of congenital heart defects. Great strides have been made in the past ten years in ultrasound technology and its applications. The examination of the congenitally deformed heart by echo methods is now an intrinsic component of patient evaluation; a remarkable array of important clinical decisions concerning such patients depends, at least in part, on the results of echocardiographic study.

Aortic Valve Stenosis↗

Preschool assessment of infants with a patent ductus arteriosus: comparison of ligation and indomethacin therapy.

We report the preschool growth and development of preterm infants with problematic patent ductus artferiosus requiring either surgical ligation or indomethacinn treatment in the neonatal period. Of 60 who completed longitudinal growth assessments, no differences in growth to 3 years could be demonstrated except for four cases of regressed retinopathy of prematurity among the ligated infants. Developmental tests (Bayley Scales of Infant Development and the Peabody Picture Vocabulary Test) showed no group differences at preschool ages. Although psychomotor performance on the Bayley Scales in the first 1 1/2 years was significantly lower among the surgically treated infants, later analysis showed that a similar number in both treatment groups scored within the normal range. No selective short- or long-term adverse effects of indomethacin treatment could be demonstrated.

Body Height↗

Evaluation of right ventricular and right atrial size in children with atrial septal defect using two-dimensional apex echocardiography.

The ability of two-dimensional apex echocardiography to evaluate right ventricular and right atrial size was evaluated in 43 normal children and compared with 20 patients with a secundum atrial septal defect. From the apical four chamber view, the long axis length and short axis diameter of the right ventricle and right atrium were measured. The end-diastolic area of the right ventricle and end-systolic area of the right atrium were determined by planimetry. These data were indexed for body surface area. When compared with normal subjects, the area index of the right atrium and right ventricle were enlarged significantly in patients with a secundum atrial septal defect (p less than 0.001 and p less than 0.001, respectively). Mean values for the short axis dimension of the right ventricle and for the short axis and long axis diameter of the right atrium were greater in atrial septal defect patients than in normal subjects (p less than 0.001 for all). However, when both groups were compared, considerable overlap existed between the dimension measurements. Right ventricular long axis length failed to separate the two groups. Thus, two-dimensional apex echocardiography provides a noninvasive means of assessing right ventricular and right atrial size in children with an atrial septal defect.

Adolescent↗

Complex cyanotic congenital heart disease correctable with an intra-atrial baffle.

The clinical, angiocardiographic, and surgical findings in a cyanotic child with dextrocardia, L-loop, laterally inverted but otherwise normally related great arteries, and situs ambiguus are described and compared with other similar cases. Electrocardiographic, echocardiographic, and angiographic data are reviewed, and the segmental approach to diagnosis is stressed. Both systemic and pulmonary veins returned to the right-sided atrium. Blood then passed either through the mitral valve to the morphologically left ventricle and to the aorta or through an atrial septal defect to a small left-sided atrium, right ventricle, and pulmonary artery. Total correction was undertaken with an intra-atrial baffle to direct pulmonary venous return to the morphologically right ventricle and systemic venous return to the right ventricle. The subpulmonic stenosis was alleviated with an outflow patch.

Blood Vessel Prosthesis↗

Clinical problems of postoperative pulmonary vascular disease.

The younger the patient with a large left to right shunt at the time of operation, the greater the likelihood that pulmonary vascular resistance will fall to normal thereafter. In older patients, the degree to which the pulmonary vascular resistance is elevated before operation is a critical factor determining operability and prognosis. Patients at particularly high risk for the development of significant pulmonary vascular obstruction early in life are those with certain forms of cyanotic congenital heart disease, such as complete transposition of the great arteries with ventricular septal defect and patent ductus arteriosus, and truncus arteriosus. Other conditions in which pulmonary vascular obstruction appears to progress rapidly include large ventricular septal defect, complete atrioventricular canal defect and left to right shunt lesions in an environment of high altitude or associated with unilateral pulmonary arterial absence of the Down's syndrome. In this report the framework is reviewed for recognizing that important pre- and postnatal modifiers of the pulmonary vascular bed may be lesion-dependent. Thus, the growth and development of the pulmonary vascular bed during fetal and early postnatal life, as well as the morphologic alterations described in detail by Heath and Edwards, are likely to determine the ultimate intensity and magnitude of pulmonary vascular obstruction. Commentary is also provided concerning the management of patients with high pulmonary vascular resistance during pregnancy and delivery, their response to exercise, and the possibility of medical treatment designed to reduce pulmonary vascular resistance, and perhaps prolong life and enhance its quality.

Adult↗

Two-dimensional echocardiographic assessment of right ventricular volume in children with congenital heart disease.

The ability of 2-dimensional echocardiography to measure right ventricular (RV) volume and ejection fraction was assessed in 22 children with congenital heart disease. From the apical 4 chambers 2-dimensional echocardiographic image, the long-axis length of the right ventricle was measured and the area planimetered. On the anteroposterior and lateral cineangiocardiographic planes, the right ventricle was separated into 2 parts: RV sinus and outflow tract. The longest length, inflow tract length, and area of the sinus were measured from biplane cineangiographic views. The echographic long-axis length correlated well with the longest length of the RV sinus measured from both anteroposterior and lateral cineangiographic views at both end-systole and end-diastole. Moreover, the echographic area correlated well with the sinus area obtained from both cineangiographic views. From these regression analyses, the echographic long axis length and area were corrected to the angiographic longest length and area of the sinus. The new corrected echographic longest length and area were applied to 3 formulas (2 biplane and 1 uniplane) to calculate the sinus volume of the right ventricle. Total RV volume was then derived from the sinus volume. RV volumes and ejection fraction determined by 2-dimensional echocardiography were compared with those obtained from biplane cineangiography using Simpson's rule method. All formulas tested predicted RV volumes and ejection fraction with equal accuracy. Thus, 2-dimensional echocardiography can assess RV volume and ejection fraction in children with congenital heart disease.

Angiocardiography↗

Age-related effects of digoxin on myocardial contractility and Na-K pump in sheep.

The age-dependent effects of an acute nontoxic, positively inotropic dose of digoxin on myocardial monovalent cation active transport were determined in fetal, newborn, and adult sheep. Thirty-five lightly sedated, closed-chest animals were instrumented to record electrocardiogram, left ventricular (LV) pressure, and rate of change of LV pressure (LV dP/dt). Ouabain-inhibitable uptake of Rb+ (86Rb+) was measured in both right ventricular (RV) and LV slices from control animals and in animals infused with [3H]digoxin (0.04 mg/kg) sufficient to cause an increase in LV dP/dt without toxicity. Sixty minutes after digoxin, LV dP/dt increased 123% over base-line values in fetuses, 131% in newborns, and 165% in adult animals. RV and LV myocardial digoxin concentrations were similar in all groups. Rb+ active transport was significantly reduced in both RV and LV tissue from all animals 60 min after digoxin. Control animals showed no significant changes in contractility or Rb+ active transport among the control group of fetal, newborn, or adult sheep. Acute infusions of digoxin increased LV contractility in each age group and was accompanied by digoxin-induced inhibition of myocardial Rb+ active transport. No age-related differences in the extent of Rb+ active transport among control or among digoxin-treated animals were observed under these experimental conditions. These studies suggest that the differential response to the therapeutic and toxic effects of digoxin in sheep of various ages does not reside in an age-dependent response of the myocardial sodium pump to digoxin.

Animals↗

Two-dimensional echocardiographic assessment of left ventricular volumes and ejection fraction in children.

The ability of two-dimensional echocardiography to measure left ventricular volumes and ejection fraction was evaluated in 25 children with congenital heart disease. Dimensions and planimetered areas were obtained in the short-axis view at the mitral valve and high and low papillary muscle levels and in the apical two- and four-chamber views. Eight algorithms using five geometric models were assessed. Left ventricular end-diastolic volume, end-systolic volume and ejection fraction were compared with data from biplane cineangiocardiograms. The correlation varied with the algorithm used. Algorithms using short-axis views appeared superior to those using only apical long-axis views. Four algorithms estimated left ventricular volumes with equal accuracy (Simpson's rule, assuming the ventricle to be a truncated cone; Simpson's rule, assuming the ventricle to be a truncated ellipse; hemisphere cylinder; and ellipsoid biplane). The single algorithm that best estimated left ventricular ejection fraction was the ellipsoid biplane formula using the short-axis view at the papillary muscle level (r = 0.91, slope = 0.94, SEE = 6.7%). Thus, two-dimensional echocardiography can accurately assess left ventricular volumes and ejection fraction in children with congenital heart disease.

Adolescent↗

Two-dimensional echocardiographic evaluation of discrete subaortic stenosis from the apical long axis view.

M-mode standard two-dimensional (2DE) left parasternal long axis echocardiographic examination of the left ventricular outflow tract (LVOT) was evaluated exclusively with respect to its utility in identifying discrete subaortic stenosis (SUB-AS). Important details of the anatomy of the subaortic area may also be obtained from 2DE apical long axis imaging. According, 18 patients with discrete SUB-AS were prospectively evaluated by M-mode and 2DE. The M-mode findings included narrowing of the LVOT and early systolic closure of the aortic valve. However, these findings were variable and highly dependent upon scan speed, fluid flow dynamics, and beam angulation. 2DE findings varied using the standard long axis view at the left parasternal border, depending upon the type of obstruction present. A discrete membrane produced linear echoes adjacent and parallel to the interventricular septum beneath the aortic valve. Fibromuscular obstruction produced a localized dense ridge of echoes in the LVOT. These findings were not apparent in five patients studied. In these patients, the 2DE apical long axis view was employed to image the subaortic area. From this tomographic corss-section a fibrous membrane was imaged as a linear echo parallel to the aortic valve. The membrane extended across the LVOT from the ventricular septum to the anterior leaflet of the mitral valve. The 2DE apical long axis view therefore provides an additional approach in the evaluation of patients with discrete SUB-AS.

Adolescent↗

The cardiovascular effects of dopamine in the severely asphyxiated neonate.

The cardiovascular effects of dopamine were evaluated in 14 severely asphyxiated neonates. After a period of stabilization, either dopamine 2.5 micrograms/kg/minute or placebo was infused in a randomized double-blind protocol. In seven dopamine-treated infants, echocardiographically determined shortening fraction and mean velocity of circumferential fiber shortening increased when compared to preinfusion values (P less than 0.05). There was no significant change in these echo indices of cardiac function in the placebo-treated group. Systolic blood pressure rose in the dopamine group when compared to predopamine infusion values and to the postinfusion values of the placebo group (P less than 0.001 and 0.025, respectively). Diastolic blood pressure increased to a small degree in the dopamine group. There was no significant change in heart rate or echocardiographically measured systolic time intervals. Low doses of dopamine increase cardiac performance and raise systolic blood pressure in the severely asphyxiated neonate.

Asphyxia Neonatorum↗