L. Jerome Krovetz

Cardiovascular Manifestations of the Hurler Syndrome Hemodynamic and Angiocardiographic Observations in 15 Patients

Hemodynamic observations in 15 cases of the Hunter-Hurler syndrome are reported. Required premedication was two to three times that usually needed. No major complications or deaths occurred. As a group, these individuals tend to have slight elevations of pulmonary artery pressures and more definite elevations of systemic arterial pressures. They generally have normal cardiac outputs and, thus, elevations of total systemic, total pulmonary, and pulmonary arteriolar resistances. In only one case was there clinical and angiocardiographic evidence of valvular involvement, that case having aortic insufficiency. In another case there was only angiocardiographic evidence of mitral insufficiency. Only one child was in congestive heart failure at the time of the study. There was no evidence of coronary artery disease. The ascending limb right ventricular pressure curve was frequently notched and the pressure rise time of the left ventricle (dp/dt) was low. Nocase had a congenital malformation of the heart.Those individuals who have normal vascular bed resistances appear to live longer than those who have elevated resistances early in life. The prognosis is also better for the male Hurler of the sex-linked variety than for those cases due to autosomal recessive inheritance.

Heart disease in the Laurence-Moon-Biedl-Bardet syndrome: A review and a report of 3 brothers

A summary of heart disease in the Laurence-Moon-Biedl-Bardet syndrome has not been published previously. Three male siblings, two of whom had congenital heart disease, are discussed. A review of the literature indicates that a variety of congenital cardiac malformations, occurring predominantly in males, may be found in this syndrome. Acquired heart disease, primarily hypertrophy of the left ventricle, is noted most frequently in association with renal disease and systemic hypertension. Necropsy reports suggest that the incidence of congenital and acquired heart disease is greater than one would surmise from the clinical reports of this entity.

A New Method Utilizing Indicator-Dilution Technics for Estimation of Left-to-Right Shunts in Infants

The short circulatory pathways of infants cause rapid systemic and pulmonary circulation times. So rapid are they that the usual methods for calculating left-to-right shunts frequently yield a considerable overestimation. A new formula was described for use with injections into the left heart and arterial sampling. This formula applied to 11 infants and 7 older patients showed a significantly better correlation with oximetric calculations than did the right heart injections. This method also will reveal the presence of a left-to-right shunt that is of insufficient magnitude to distort dye curves obtained following right heart injections.

The physiologic significance of body surface area.

I N W E a E S T in comparing the physiology of various sized animals has led to a long search for methods that would allow quantitative comparisons. This has been of particular interest in the field of pediatrics, where the pertinent range of body size is large, regardless of whether length, weight, or body surface area is considered. These measurements, as well as total body water, lean body mass, and equations combining two or more of these parameters have been utilized in attempts to develop meaningful normal physiologic standards, as well as to estimate fluid and drug dosages. Rather than attempt a complete discussion of all such parameters and their associated equations, this paper will restrict itself to discussion of body surface area as an index of normal physiologic standards, including a review of methods used for measuring or estimating surface area.

Hemodynamic effects of isotonic solutions rapidly injected into the heart and great vessels.

Abstract Rapid injections of 0.9 per cent NaCl, 5 per cent dextrose, and heparinized blood were made into the heart and great vessels of dogs under conditions mimicking those of angiocardiography. Injections into the right side of the heart were usually followed by alternating periods of systemic hypotension and hypertension, an increase in pulmonary arterial pressure, and an increase in cardiac output. Injections into the left ventricle and aorta were more striking. Systemic arterial pressures decreased by an average of 31 per cent of control values, whereas pulmonary arterial and pulmonary arterial capillary pressures increased approximately the same amount. Changes in cardiac output were variable, ranging from +35 to −40 per cent of control values. Left heart injections also produced tachypnea, bradycardia, and extrasystoles in several of the dogs. Similar effects were demonstrated in 11 patients undergoing diagnostic catheterizations. In view of the fact that these effects could be produced by the rapid injection of isotonic solutions, considerable caution should be observed in extrapolating physiologic data from angiocardiograms.

An Analysis of Factors Determining Delivery Rates of Liquids through Cardiac Catheters

To obtain optimal opacification of the cardiovascular system it is desirable to inject angiographic contrast medium at rapid and reproducible rates. All too often maximum diameter and minimum length of the catheter are determined by patient size. While this is a restriction, injection pressure, temperature, and the particular contrast agent are still available as variables. As opacification depends on the delivery rate of iodine rather than its volume, there are practical limitations to the benefits gained by increasing iodine content as opposed to losses incurred by-concomitant increases in viscosity. Previous attempts at analysis have relied on mathematical formulations for laminar and/or turbulent flow (1–4). As we shall see, since catheter flows encompass both ranges no one of these formulas is applicable for flows generated by pressure injectors. These flows are predictable by the standard engineering technic which plots friction factor and Reynolds number for tube flow. Material and Methods The cont...

Left Ventricular Function in Children Studied by Increasing Peripheral Resistance with Angiotensin

Response to angiotensin infusion was evaluated in 11 normal children and 24 patients with suspected left ventricular dysfunction. This included 12 patients with endocardial fibroelastosis, three with idiopathic myocardial hypertrophy, one with cardiac glycogenosis, and eight with the Hunter-Hurler syndrome. In 20 of these 24 patients, cardiac catheterization findings confirmed the clinical suspicion of decreased left ventricular function. In 11 instances left ventricular dysfunction was diagnosed by an elevation of resting left ventricular end-diastolic pressure. In eight instances this was ascertained by a decrease in resting left ventricular stroke work when compared to the expected range, derived by using a linear regression formula based on 24 normal subjects. In only four instances were both abnormal; that is, the resting left ventricular stroke work was decreased and the left ventricular end-diastolic pressure was increased. In five of these 20 patients, abnormal left ventricular function was elicited only on infusion of angiotensin. Thus this test is a significant adjunct in our ability to determine myocardial malfunction at cardiac catheterization.

Interruption of the aortic arch with complete transposition of the great vessels

Abstract This report concerns a Negro infant with interruption of the aortic arch and complete transposition of the great vessels. The presenting features were congestive heart failure and differential cyanosis. The marked cyanosis of the hands was in sharp contrast to the relatively normal color of the feet. The diagnosis was established during life by detailed hemodynamic and angiocardiographic studies. These are correlated with the salient clinical features of this case and with similar cases reported in the literature.

Relation of hemodynamics to height and weight percentiles in children with ventricular septal defects

Abstract Cardiac catheterization data of 90 children with isolated ventricular septal defects were analyzed for hemodynamic correlates with growth failure. Growth retardation (weight more than height) was a prominent feature in both sexes although girls were more severely affected. Of the hemodynamic parameters measured, one correlated well with growth failure. There was, however, a close association between pulmonary hypertension and poor weight gain. The antithesis was not true. Over half of the children below the tenth percentile for weight had pulmonary to systemic pressure ratios of 0.30 or less, precluding good correlation. It is believed that significantly abnormal hemodynamics in children with isolated ventricular septal defects in this age group do not account for a large proportion of “growth failures.”

Endocardial fibroelastosis in American Negro children: A distinct entity?

Abstract Of 24 cases of endocardial fibroelastosis (EFE), 18 (75 per cent) were in Negro children. Our general and cardiac pediatric admissions average slightly less than 25 per cent Negro, rendering this a statistically significant difference. Further analysis by race revealed that the Negro children had a later onset of symptoms and a higher incidence of iron-deficiency anemia. In addition, there was suggestive evidence of a higher incidence of prematurity. No further racial differences in signs, symptoms, or laboratory findings were noted. These differences suggest to us that EFE or a clinically indistinguishable disease is more common among the Negro children in our geographical area. Reference is made to the similarity and to the differences between our American Negro cases and the cardiomyopathy occuring in African Negro children, as well as to the higher incidence of myocardial and endomyocardial fibrosis in the Negro race, both in Africa and the United States. Recent speculation as to the etiology of EFE and the possible role of heredity, environment, and socioeconomic factors is mentioned.