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Biomedical subjects

J S Child

Publications and source records attributed to J S Child.

At least 55 records · Page 3Linked to original sources

Adults with cyanotic congenital heart disease: hematologic management.

Hematologic management of adults with cyanotic congenital heart disease has received little recent attention. The lack of practical therapeutic guidelines prompted us to consolidate our observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients. Specific attention focused on regulation of erythrocyte mass and concepts of compensated and decompensated erythrocytosis, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function. Cerebral infarction was not seen in any patient. Phlebotomy is best reserved for treatment of symptomatic hyperviscosity. Iron therapy is indicated for symptomatic iron deficient erythropoiesis. Abnormal hemostatic mechanisms are the rule. Antithrombotic medications have little or no role in treatment. Hyperuricemia is the result of abnormal renal uric acid excretion not urate overproduction, and serves as a marker of abnormal renal function. Drugs that promote urate excretion are the preferred maintenance treatment in symptomatic hyperuricemic patients.

Adolescent↗

Assessment of mitral flow velocity with exercise by an index of stress-induced left ventricular ischemia in coronary artery disease.

Exercise-induced myocardial ischemia results in both diastolic and systolic left ventricular (LV) dysfunction. To investigate the utility of Doppler assessment of LV diastolic function with exercise, 28 consecutive patients underwent digital stress echocardiography, including measurement of mitral flow velocity by pulsed-wave Doppler and simultaneous stress thallium imaging. The mean mitral flow velocity was measured as the integrated area under the LV diastolic inflow Doppler spectral display. The change in mean mitral flow velocity from baseline to immediate postexercise was compared among 3 patient groups: (1) patients with thallium redistribution or exercise-induced wall-motion abnormalities, or both, consistent with exercise-induced ischemia (n = 18), (2) patients with no evidence of stress-induced ischemia, with or without resting wall-motion abnormalities (n = 10), and (3) 10 control subjects of similar age with normal resting 12-lead electrocardiograms, normal resting and postexercise 2-dimensional echocardiograms and normal electrocardiographic treadmill stress testing. The percent increase in mean mitral flow velocity was 101% (+/- 59) for controls and 86% (+/- 53) for patients without stress-induced ischemia versus 33% (+/- 24) in patients with stress-induced ischemia (p less than 0.005). An increase in mean mitral flow velocity with exercise of greater than 50% correctly identified 9 of 10 nonischemic control patients. An increase in mean velocity of less than 50% predicted ischemia in 15 of 18 patients with evidence of stress-induced ischemia (p less than 0.005) Thus, Doppler assessment of LV diastolic function with exercise expressed as a change in the mean velocity of mitral flow is a useful indicator of stress-induced ischemia.

Blood Flow Velocity↗

Reassessment of echocardiographic criteria for diagnosis of mitral valve prolapse.

Commonly used echocardiographic criteria for mitral valve prolapse (MVP) include a sizable proportion of persons whose hearts are normal. Nevertheless, the echocardiogram is generally used as an independent standard for the diagnosis of MVP despite lack of consensus on the criteria to be used and the probability of interobserver variability. Conversely, there is a relatively uniform consensus that classic auscultatory signs establish the diagnosis of MVP beyond reasonable doubt. Accordingly, in 148 patients referred for evaluation of known or suspected MVP, the echocardiographic patterns that coincide with diagnostic auscultatory signs were studied prospectively to compare those patterns with criteria commonly used for the echocardiographic diagnosis of MVP and to determine interobserver variability in echocardiographic interpretation. Eighty patients (54%) had a classic mid- to late systolic click or an apical late systolic murmur, or both. Eleven patients (7%) had the apical holosystolic murmur of mitral regurgitation with no discernible clinical or echocardiographic cause other than the consideration of MVP. Doppler echocardiography was performed in 80 of the 148 patients. The degree of superior systolic bowing of each mitral leaflet and the location of leaflet coaptation relative to the presumed plane of the mitral anulus were graded on apical 4-chamber and parasternal long-axis views. The only patterns absolutely specific for auscultatory MVP were: severe bowing of the anterior or posterior leaflet; coaptation of leaflets on the left atrial side of the anular plane; moderate to severe Doppler mitral regurgitation accompanied by any degree of leaflet bowing; and mild Doppler mitral regurgitation accompanied by moderate bowing of a leaflet.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Hemodynamic responses to isolated increments in heart rate by atrial pacing after a Fontan procedure.

The physiologic role of the right ventricle has long been a subject of interest to physiologists. The Fontan operation provides a human model for studying the circulation in series devoid of a subpulmonic right ventricle. The hemodynamic response to isotonic exercise in this setting has been established, and differs appreciably from normal. However, the physiologic response to an increase in heart rate (atrial pacing) as an isolated variable has not been examined and compared to atrial pacing in hearts with two concordant subarterial ventricles. Accordingly, we compared the supine bicycle exercise response to rate-equivalent right atrial pacing in nine patients after atriopulmonary anastomoses (the Fontan operation) for single ventricle or tricuspid atresia. Cardiac index increased 77% with exercise (rest 2.6 L/min/m2; exercise 4.6 L/min/m2) but decreased 12% with atrial pacing (rest 2.5 L/min/m2; pacing 2.2 L/min/m2). Pulmonary arterial oxygen saturation declined significantly during exercise (rest 68%; exercise 31%) and during atrial pacing (control 72%; pacing 64%). The mean increment in pulmonary arterial pressure was 1.3 times greater with exercise (rest 14 mm Hg; exercise 20 mm Hg) than with pacing (control 12 mm Hg; pacing 16 mm Hg). Peak systemic arterial systolic pressure increased 14% with exercise but was unchanged by pacing. Systemic and pulmonary vascular resistances fell with exercise but changed insignificantly during atrial pacing. Stroke volume rose slightly with exercise but fell significantly with pacing.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

The value of echocardiography in blunt chest trauma.

All victims of blunt injury to the chest or precordium admitted to a Level I trauma center in a 1-year period were evaluated prospectively with two-dimensional echocardiography on the day of admission, serial determinations of creatine kinase (CK) and MB isoenzyme radioimmunoassay (CK-MB) over the first 24 hours, continuous electrocardiographic monitoring over at least the first day, and serial 12-lead electrocardiography (ECG) over the first 3 days. The patients were divided into four groups based upon the results of echocardiography. Group A (n = 35) had normal ECHO and ECG; Group B (n = 16), normal ECHO and abnormal ECG; group C (n = 14), ECHO showing abnormal wall motion and/or pericardial fluid; group D (n = 8), ECHO showing a nontraumatic valvular or wall motion abnormality. Nineteen patients required an operation under general anesthesia. Group C patients had significantly higher CK, CK-MB, numbers of associated injuries, and Injury Severity Scores; seven required invasive hemodynamic monitoring. No cardiac morbidity of general anesthesia was seen. We conclude that echocardiography is an important tool for diagnosis and triage which may be used to stratify a homogeneous patient population into groups with acute, chronic, and no cardiac disease. Cardiac injury occurs in a setting of multisystem trauma. Patients with normal echocardiogram and ECG on admission do not require intensive care monitoring.

Adolescent↗

Control of ventilation during exercise in patients with central venous-to-systemic arterial shunts.

The diversion of systemic venous blood into the arterial circulation in patients with intracardiac right-to-left shunts represents a pathophysiological condition in which there are alterations in some of the potential stimuli for the exercise hyperpnea. We therefore studied 18 adult patients with congenital (16) or noncongenital (2) right-to-left shunts and a group of normal control subjects during constant work rate and progressive work rate exercise to assess the effects of these alterations on the dynamics of exercise ventilation and gas exchange. Minute ventilation (VE) was significantly higher in the patients than in the controls, both at rest (10.7 +/- 2.4 vs. 7.5 +/- 1.2 l/min, respectively) and during constant-load exercise (24.9 +/- 4.8 vs. 12.7 +/- 2.61 l/min, respectively). When beginning constant work rate exercise from rest, the ventilatory response of the patients followed a pattern that was distinct from that of the normal subjects. At the onset of exercise, the patients' end-tidal PCO2 decreased, end-tidal PO2 increased, and gas exchange ratio increased, indicating that pulmonary blood was hyperventilated relative to the resting state. However, arterial blood gases, in six patients in which they were measured, revealed that despite the large VE response to exercise, arterial pH and PCO2 were not significantly different from resting values when sampled during the first 2 min of moderate-intensity exercise. Arterial PCO2 changed by an average of only 1.4 Torr after 4.5-6 min of exercise. Thus the exercise-induced alveolar and pulmonary capillary hypocapnia was of an appropriate degree to compensate for the shunting of CO2-rich venous blood into the systemic arterial circulation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

The restrictive cardiomyopathies.

In parallel with the rapidly developing interest in the diastolic properties of ventricular function in the 1970s, the restrictive cardiomyopathies have taken their place as the third major category of primary heart muscle disease. The restrictive cardiomyopathies are characterized by primary abnormalities of diastolic ventricular function with normal to near normal systolic performance and little or no increase in end-diastolic or end-systolic dimensions of either right or left ventricle. The restrictive abnormality of ventricular function can result from myocardial or endomyocardial disease, the etiologies of which may be known or unknown. Diastolic dysfunction that is essentially myocardial can be idiopathic (probably an enzymatic/metabolic disturbance), infiltrative (myocardial interstitium), or within myocardial cells (storage diseases). Diastolic dysfunction that results from endomyocardial disease is typified by endomyocardial fibrosis or the hypereosinophilic syndrome, although carcinoid, metastatic malignancies, radiation, and anthracycline toxicity may be accompanied by endomyocardial restriction. Echocardiography and Doppler ultrasound, MRI, and radionuclear techniques have been major advances in providing diagnostic precision, contributing materially to the clinical identification of the restrictive cardiomyopathies as well as discriminating certain of their etiologies.

Adult↗

Comparison of two-dimensional and Doppler echocardiography and intracardiac hemodynamics for quantification of mitral stenosis.

Forty-three patients with mitral stenosis (MS) were studied to assess the relation of catheter-derived pressure gradient half-time (P 1/2), mitral valve areas (calculated by the Gorlin formula and 2-dimensional echocardiography [2-D echo]) to mitral valve areas derived from Doppler pressure half-time (T 1/2) in order to establish an accurate line-drawing method in nonlinear velocity tracings and to revalidate the use of the empiric constant of 220 ms as the T 1/2 that predicts a 1.0-cm2 mitral valve area. Mitral valve area could be quantified by 2-D echo in 39 of 43 patients and by Doppler in 31 of 34 patients, for a success rate of 91%. A reliable technique for measuring Doppler T 1/2 in nonlinear Doppler velocity tracings was a "mid-diastolic" line-drawing method, validated with the "anatomic" mitral valve area by 2-D echo (r = 0.89) and with the "hemodynamic" mitral valve area by the Gorlin formula (in pure MS without regurgitation) (r = 0.95). By both Doppler T 1/2 and hemodynamic P 1/2, the use of 220 ms to predict a mitral valve area of 1.0 cm2 was validated. Each T 1/2 and P 1/2 had an exponential inverse relation to the mitral valve area by the Gorlin formula in pure MS. Doppler and 2-D echocardiographic quantification of MS are complementary. Reliable measurement of T 1/2 in nonlinear velocity tracings is achieved by a mid-diastolic line-drawing method and use of the equation 220 ms/T 1/2 = mitral valve area accurately quantifies MS.

Adult↗

Mitral regurgitation after cardiac transplantation.

The contribution of the left atrium to mitral valve competence was assessed using the model of altered atrial size and geometry created by atrial anastomosis during cardiac transplantation. Sixteen patients underwent Doppler and 2-dimensional echocardiography after orthotopic transplantation. Mitral regurgitation was present in 14 of 16 patients. Left atrial geometry was uniformly abnormal, in a "snowman" configuration. Compared with 16 normal control subjects, the transplanted left atria were dilated (23 +/- 6 vs 13 +/- 3 cm2 during ventricular systole, p less than 0.001). Mitral valve anular diameter indexes, anular systolic reduction and ventricular function were normal in both groups. Ventricular volumes were small in the transplanted heart relative to donor body size (15 +/- 5 vs 20 +/- 8 cm3/m2 in systole, p less than 0.05). The ratio between ventricular length and anular diameter was smaller in the transplant patients (0.87 +/- 0.1 vs 1.0 +/- 0.2, p less than 0.05). In the presence of abnormal left atria, mitral regurgitation may occur without other structural abnormalities of the mitral apparatus. This study suggests that the left atrium plays an important role in mitral valve competence for primary cardiac disease associated with left atrial enlargement, even in the absence of intrinsic mitral valve disease or left ventricular dysfunction.

Echocardiography↗

Predictability of mitral regurgitation detected by Doppler echocardiography in patients referred for cardiac transplantation.

Mitral regurgitation (MR) is common in patients with congestive heart failure (CHF), but the frequency in relation to the origin of ventricular dilatation has not been established. In 50 patients referred for cardiac transplantation, MR was assessed by Doppler echocardiography and the findings were compared with clinical information. Dilatation of the left ventricle, left atrium and mitral anulus was analyzed in 25 patients with respect to cause of CHF. All 50 patients had MR of at least moderate severity, regardless of cause (idiopathic dilated cardiomyopathy in 36, coronary artery disease in 14), length of symptoms (20 +/- 19 months, less than 6 months in 13 patients) or presence of murmurs (absent in 13 patients). Comparison of patients with nonischemic cardiomyopathy to those with ischemic heart disease revealed larger left ventricular volumes (215 +/- 81 vs 131 +/- 60 ml in systole, p less than 0.05) and left atrial volumes (124 +/- 70 vs 70 +/- 35 ml, p less than 0.05). Mitral anular dilatation was present only in patients with idiopathic cardiomyopathy (diameters 3.6 +/- 0.4 vs 3.1 +/- 0.2 cm, p less than 0.05). The frequency of significant MR in these patients with CHF suggests that it may have a major role in decompensation and in the therapeutic response.

Adolescent↗

Beneficial effect of residual anterograde flow on tissue viability as assessed by positron emission tomography in patients with myocardial infarction.

With the advent of therapeutic interventions to restore blood flow in patients with acute infarction, early identification of salvageable myocardium has gained clinical importance. It was the purpose of this study to define regional blood flow and glucose metabolism in patients with acute myocardial infarction as assessed by positron emission tomography (PET) and to correlate these findings with coronary anatomy. In 15 patients blood flow as determined by N-13 ammonia was reduced in 37 left ventricular segments. In 20 segments there was a similar decrease in FDG uptake, consistent with tissue necrosis. In contrast, 17 segments with reduced blood flow revealed maintained FDG uptake suggesting residual viability in the electrocardiographically defined 'infarct segment'. PET viability and necrosis was significantly associated with presence or absence of anterograde flow in the infarct artery. Evaluation of regional wall motion did not distinguish segments with PET criteria for viability from segments with necrosis. Thus, in patients without thrombolytic therapy during evolving myocardial infarction, spontaneous reperfusion appears to be beneficial for tissue survival. PET offers the unique possibility to assess tissue viability early after the ischaemic injury where evaluation of wall motion fails to identify salvageable myocardium.

Adult↗

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve.

We postulated that the abnormal shape, size, and function of the right heart and adjoining ventricular septum in adults with Ebstein's anomaly of the tricuspid valve might in turn alter the shape and function of the left ventricle. Seven adult patients with uncomplicated Ebstein's anomaly were studied. Left ventricular geometry was determined by two-dimensional echocardiography. Left ventricular function was assessed by treadmill exercise and radionuclide angiography at rest and with exercise. Paradoxic ventricular septal motion was consistently present. Left ventricular eccentricity (ratio of two minor axes in the short-axis view) was uniformly abnormal, averaging 1.35 +/- 0.23 (normal = 1.02 +/- 0.05). The ratio of right to left ventricular cavity size averaged 1.70 +/- 0.44 (normal 0.65 +/- 0.30), and tricuspid valve displacement into the right ventricular cavity averaged 52% (normal 8%). Functional right atrial size averaged 27.6 +/- 5.2 cm2 (normal right atrial area = 13.1 +/- 2.2 cm2). Resting left ventricular ejection fractions were below 50% in all but two patients. In response to Bruce protocol exercise stress, there were consistently appropriate increments in heart rate, blood pressure, and peak double product and, with one exception, radionuclide left ventricular ejection fraction. There were significant correlations between tricuspid valve displacement and functional right atrial size versus resting left ventricular ejection fraction and left ventricular eccentricity. These data support the hypothesis that derangements in right heart morphology and function in Ebstein's anomaly contribute to significant alterations in left ventricular geometry, but the geometric alterations are associated with tangible but less significant changes in left ventricular systolic function.

Adolescent↗

Cardiac tumors and thrombus: evaluation with MR imaging.

Thirty patients with a suspected cardiac or pericardial mass underwent MR imaging. Twenty-six also had two-dimensional (2D) echocardiography, and three also had CT; one patient had MR only. Overall, 18 (60%) of the 30 patients were found to have a mass lesion. The lesion was confirmed by biopsy, surgery, or unequivocal demonstration on CT, 2D echocardiography, and/or MR imaging. Fourteen of the lesions were soft-tissue or tumor masses, and four were thrombi. The findings on 2D echocardiography and MR were in agreement in 17 (65%) of 26 patients who had both studies. MR was equivocal or in error in two patients (7%), and 2D echocardiography was nondiagnostic in seven (27%). In all seven patients with equivocal 2D echocardiography, the diagnosis was made by MR. In the four patients who did not have 2D echocardiography, MR showed the mass clearly. MR imaging is useful in the diagnosis of cardiac mass lesions. It can be used effectively in addition to 2D echocardiography to increase the certainty of diagnosis, and it is useful when 2D echocardiography is equivocal or inadequate.

Adolescent↗

Chronic hypoxaemia and decompensated erythrocytosis in cyanotic congenital heart disease.

Among forty adults with cyanotic congenital heart disease there was a subset of eleven patients with especially pronounced erythrocytosis, repeatedly rising haematocrit, recurring symptoms of hyperviscosity, and little or no shift of the haemoglobin/oxygen-dissociation curve. These patients were iron deficient as a result of many therapeutic phlebotomies; nevertheless their red-cell mass was comparable to that in iron-replete patients with similar, but stable, haematocrits. Iron repletion in the deficient patients resulted in rapidly increasing haematocrit and hyperviscosity. In one extreme case, erythropoiesis remained persistently iron deficient despite normal serum iron and ferritin levels. "Decompensated erythrocytosis" is an apt term for the excessive erythrocytic response and the associated phenomena.

Adolescent↗

New guidelines for the clinical diagnosis of mitral valve prolapse.

Because the term mitral valve prolapse has pathologic connotations, considerable effort has been expended to establish acceptable diagnostic standards, but without general agreement. This report combines information from the history, physical examination, electrocardiogram, chest x-ray and 2-dimensional echocardiogram in an effort to avoid the artifice of using the 2-dimensional echocardiogram as a categoric reference standard and to establish new clinical guidelines that distinguish pathologic mitral valve prolapse--a primary connective tissue abnormality of leaflets, chordae tendineae and anulus--from normal superior systolic displacement of mitral leaflets. The objective is to avoid implications of heart disease in healthy young persons within the gaussian distribution of normal. To this end, and with the Jones criteria as a model, major and minor criteria are proposed for the clinical diagnosis of mitral valve prolapse as a pathologic entity.

Angiocardiography↗