Comparison of high pulse repetition frequency and continuous wave Doppler echocardiography for velocity measurement and gradient prediction in children with valvular and congenital heart disease.

To compare the ability of high pulse repetition frequency and continuous wave Doppler echocardiography to detect the peak velocity of a jet flow disturbance and to predict pressure gradients accurately, two groups of children with valvular or congenital heart disease were examined using both Doppler techniques. The use study group included 84 children or adolescents (aged 1 day to 19 years) who underwent examination in the echocardiography laboratory with both Doppler techniques in a randomized sequence. The peak velocity recorded with high pulse repetition frequency Doppler echocardiography was compared with the peak velocity recorded with the continuous wave technique. The accuracy study group included 41 children or adolescents (aged 1 day to 16 years) who underwent examination with both Doppler techniques at the time of cardiac catheterization. Doppler pressure gradients were calculated from the peak velocity using the simplified Bernoulli equation and were compared with peak instantaneous gradients and peak to peak gradients measured at catheterization. In the use study, a high correlation was found between peak velocities detected by high pulse repetition frequency and continuous wave Doppler echocardiography (r = 0.94, SEE = 0.28 m/s). In the accuracy study, close correlations were found between measured peak to peak pressure gradients and pressure gradients calculated from continuous wave (r = 0.95, SEE = 7.9 mm Hg) and high pulse repetition frequency Doppler echocardiography (r = 0.94, SEE = 8.7 mm Hg).(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiographic findings in Uhl's anomaly. Demonstration of diastolic pulmonary valve opening.

The echocardiographic findings in a case of Uhl's anomaly, or congenital hypoplasia of the right ventricular myocardium, are reported. Diastolic opening of the pulmonary valve is described. Comparison is made with echocardiograms in Ebstein's disease of the tricuspid valve, and it is suggested that echocardiography can help in differentiating these two entities. In addition to the pulmonary valve finding, increased right ventricular dimension, delayed tricuspid closure and abnormality (prolapse) of the mitral valve were noted. The echocardiographic findings are compared with cardiac catheterization data.

Cardiac localization of transvascular bioptome using 2-dimensional echocardiography.

Two-dimensional (2-D) echocardiography was added to standard fluoroscopic localization of transvascular endomyocardial biopsy in 7 children with heart muscle disease whose ages were 6 months to 18 years. One left ventricular and 11 right ventricular biopsies were carried ot without complications using the fluoroscopic-echocardiographic technique. Two-dimensional echocardiographic monitoring has the advantages of reducing radiation exposure and providing anatomic information about intracardiac structures as well as bioptome localization. Lateral beam spread and reverberation artifacts represent potential problems with 2-D echocardiography, but they did not cause significant difficulties in this study.

Interruption of the aortic arch: preoperative and postoperative clinical, hemodynamic and angiographic features.

Interruption of the aortic arch, studied in 10 patients, was associated with a variety of other cardiac anomalies in 8 patients and was an isolated anomaly in 2. Clinical and angiographic evaluation in the former group revealed congestive heart failure and generalized cyanosis in early infancy, pulmonary and systemic arterial hypertension and a variety of intracardiac and aortic shunt. The two patients with an isolated anomaly had clinical and hemodyanmic features not dissimilar from those of severe coarctation but interruption of the aortic arch was demonstrated on aortography. Surgical treatment was successful in four of the eight infants with associated anomalies and in both children with the isolated defect. Postoperative angiography revealed several related complications and the status of the aortic reconstruction. Clinical angiographic recognition of this lesion is important because operative intervention has been successful in an increasing number of patients.

Two dimensional echocardiographic assessment of patients with bioprosthetic valves.

The clinical utility of two dimensional echocardiography in assessing bioprosthetic and left ventricular function was studied in 40 consecutive patients 1 week to 60 months after valve replacement surgery. These patients were referred to obtain normal baseline studies as well as to evaluate complications:suspected endocarditis, embolic phenomena and congestive heart failure of unknown cause. Independent M mode echocardiograms were also obtained in each patient. Confirmation of ultrasonic studies was by cardiac catheterization with angiography, surgery and pathologic study in 10 patients; cardiac catheterization with angiography alone in 7 patients; surgery and pathologic study in 3 patients; autopsy in 3 patients; blood cultures to confirm or exclude endocarditis in 10 patients; and confirmation on clinical grounds in 7 patients. Technically adequate two dimensional studies were recorded in 39 of 40 subjects. Two dimensional echocardiography accurately assessed 15 of 16 patients with an abnormal bioprosthetic valve and a normal left ventricle (1 of 16 patients had a false positive two dimensional echocardiogram); 8 of 8 patients suspected to have prosthetic valve or left ventricular dysfunction but who were normal; 7 of 7 patients with a normal prosthesis and an abnormal left ventricle; the one patient with an abnormal valve and left ventricle; and 7 of 7 clinically normal patients who were referred for baseline studies. In summary, the two-dimensional echocardiogram demonstrated a 97 percent diagnostic accuracy rate which was significantly greater than the 67 percent (P less than 0.001) for M mode echocardiography in the same group of patients. It is concluded that two dimensional echocardiography has excellent diagnostic accuracy in assessing bioprosthetic and left ventricular function and is superior to M mode echocardiography in evaluating patients after such valve replacement.

Naturally corrected double-outlet right ventricle with biventricular outflow tract obstruction.

A 5-year-old girl had presyncopal episodes associated with exertion. Diagnostic evaluation revealed a combination of congenital abnormalities: double-outlet right ventricle with apparent "natural correction" of a large subaortic ventricular septal defect and severe biventricular outflow tract obstruction. Intraoperative and postoperative studies confirmed satisfactory relief of the biventricular outflow tract obstruction.

Two-dimensional echocardiographic features of bicuspid aortic valve.

The two-dimensional cross-sectional echocardiographic diagnosis of bicuspid aortic valves is described and compared with results of M-mode echocardiograms. Aortic valve anatomy was determined in 19 selected patients by angiography, and confirmed in five by direct surgical visualization. Using an eccentricity index (EI) of 1.3 or greater as diagnostic of bicuspid aortic valve, M-mode correctly identified anatomy in 14 of 19 valves (74 percent), although EI varied in several patients. For two-dimensional diagnosis of bicuspid aortic valve, short axis cross section was preferred, and criteria included number of cusps seen in real time motion, irregularity of folding of cusp margins, and location of commissural insertions. Two-dimensional echocardiography correctly identified anatomy in 18 of 19 valves (95 percent). Long axis cross section disclosed valvular doming in all 8 patients in whom doming was observed angiographically, correlating with hemodynamic findings. Two-dimensional echocardiography aids in the detection of bicuspid aortic valve in a suspected population, can give an estimate of valve gradients, and explains variability in M-mode findings. As such, two-dimensional echocardiography is a valuable tool in the noninvasive diagnosis of the bicuspid aortic valve.

Mechanical circulatory support for the treatment of children with acute fulminant myocarditis.

BACKGROUND: Viral myocarditis may follow a rapidly progressive and fatal course in children. Mechanical circulatory support may be a life-saving measure by allowing an interval for return of native ventricular function in the majority of these patients or by providing a bridge to transplantation in the remainder. METHODS: A retrospective chart review of 15 children with viral myocarditis supported with extracorporeal membrane oxygenation (12 patients) or ventricular assist devices (3 patients) was performed. RESULTS: All patients had histories and clinical findings consistent with acute myocarditis. The median age was 4.6 years (range 1 day-13.6 years) with a median duration of mechanical circulatory support of 140 hours (range 48-400 hours). Myocardial biopsy tissue demonstrated inflammatory infiltrates or necrosis, or both, in 8 (67%) of the 12 patients who had biopsies. Overall survival was 12 (80%) of 15 patients, with 10 (83%) survivors of extracorporeal membrane oxygenation and 2 (67%) survivors of ventricular assist device support. Nine (60%) of the 15 patients were weaned from support, with 7 (78%) survivors; the remaining 6 patients were successfully bridged to transplantation, with 5 (83%) survivors. All survivors not undergoing transplantation are currently alive with normal ventricular function after a median follow-up of 1.1 years (range 0.9-5.3 years). CONCLUSION: Eighty-percent of the children who required mechanical circulatory support for acute myocarditis survived in this series. Recovery of native ventricular function to allow weaning from support can be anticipated in many of these patients with excellent prospects for eventual recovery of full myocardial function.

Tetralogy of Fallot: selective staged management.

One hundred twenty-four patients with tetralogy of Fallot have undergone either primary total repair (61), shunt and later repair (30), or an initial shunt (33). The mean ratio of pulmonary anulus to descending thoracic aorta increased from 0.80 +/- 0.25 before the shunt to 1.22 +/- 0.26 before the repair (p less than 0.0001). The mean ratio in the primary repair group was 1.23 +/- 0.25. A transannular patch was necessary in only six of 91 patients (6.6%). Postrepair right ventricular/left ventricular pressure ratio averaged 0.50 +/- 0.11 in the shunt plus repair group and 0.43 +/- 0.12 in the primary repair group. Only four patients had a right ventricular/left ventricular pressure ratio less than 0.65. A significant inverse linear relationship existed between this ratio and the pulmonary anulus size measured at operation and normalized for the patient's height (p less than 0.01). Postoperative complications occurred in 21% of patients after a shunt and 20% of patients after open heart repair. The early mortality was 0.8% (1/124). An initial shunt in patients with a small pulmonary anulus can result in an increased anulus size and better hemodynamic result with frequent avoidance of a transannular patch. Staged repair may result in improved overall mortality rates.

Video viewing as an alternative to sedation for young subjects who have cardiac ultrasound examinations.

Sedation is often required for extended or quantitative echocardiographic examination of young patients. To test the hypothesis that patient viewing of entertaining videotapes during echocardiographic examinations would reduce the need for sedation and that Doppler pressure gradients so obtained would correspond satisfactorily with subsequent catheterization pressure gradients, 38 patients (age, 5 to 64 months; mean age, 18.6 months) with a variety of cardiac defects were studied. Because of excessive activity, resistance, fear, or crying, all of these patients would have normally been sedated. Instead, an age-appropriate videotape was used for patient viewing. In 35 of 38 patients, complete examinations were obtained with video viewing with no sedation. Twelve study patients later underwent catheterization while receiving sedation. The Doppler pressure gradients obtained while patients were viewing videotapes corresponded well with catheterization (r = 0.94). However, those pressure gradients were higher than those obtained with catheterization (mean, 8.3 mm Hg), but there was only one significant discrepancy of 22 mm Hg. In this series, complete examinations were obtained without sedation in 92% of subjects who would have normally required sedation (p less than 0.001), with minimal reduction in the accuracy of prediction of subsequent sedated catheterization pressure gradients. This suggests that near baseline conditions existed while patients were viewing television. Video viewing during echocardiographic examinations appears to be advantageous.

A child with valproic acid-associated carnitine deficiency and carnitine-responsive cardiac dysfunction.

Valproic acid enhances renal losses of carnitine esters and leads to decreased plasma free carnitine concentrations in many patients receiving valproic acid therapy. However, decreased serum carnitine levels are of unclear pathologic significance, and most children manifest no symptoms of carnitine deficiency. We report a child with valproic acid-associated carnitine deficiency who had severe cardiac dysfunction develop that resolved with carnitine replacement therapy.

APPLICATION OF T-TECHNIQUE FACTOR ANALYSIS TO THE STOCK MARKET.

Proportional changes in the prices of 425 individual common stocks were recorded for each of 44 quarter-year periods. After normalization of the data for each stock, sums of cross-products were computed among the time periods across stocks. Factor analysis of the cross-product matrix yielded seven factors. By cumulating the factor loadings successively over the 44 quarters, so as to simulate absolute values rather than change scores, six of these factors appear to be generated by the simplicial nature of the cross-product matrix, while the seventh was an expected factor representing general stock market strengths and weaknesses. Some possibilities for pre- diction are discussed, but little in the way of direct prediction of stock market fluctuations is supported by these findings.

MOTIVATIONAL TYPES AMONG COLLEGE STUDENTS.

College students were related to one another over a variety of measures associated with motivation to study in college. The inverse factor analysis of these relahionships revealed ten motivational types. Data for 12 hypothetical students, entering the analysis as an extension matrix, helped to interpret the types found among the real studenb. In a table of results, the average value on each measure for students of each type k compared with the average value for students who were found to be the opposites of each type. Consistencies among the measures of each type make good psychological sense. In addition, the actual studenb, all known personally to the author, seemed adequately to belong to the types into which the analysis placed them.

Variability of echocardiographic discontinuity in double outlet right ventricle and truncus arteriosus.

The widespread application of echocardiography to the field of congenital heart disease has led to the development of a concept of "echocardiographic discontinuity" for the diagnosis of some conditions. Although this is a valuable sign in differentiating such entities as tetralogy of Fallot, truncus arteriosus and double outlet right ventricle, the reported cases illustrate that the echocardiographic recognition of discontinuity may be complicated by technical factors. In addition, the differential diagnosis must include truncus arteriosus with coexistence of anterior and posterior discontinuity.

Dimensions of Cognitive Closure.

This study investigated the generality of the perceptual factors of speed and flexibility of closure in the cognitive domain. Several experimental measures of both flexibility and speed of closure employing perceptual or figural content, verbal or symbolic content, and semantic content were administered to 541 Naval Aviation Cadets, along with marker measures for several established factors. In addition to several of these marker dimensions, factors were obtained for both flexibility and speed of figural closure, of verbal or symbolic closure, and of semantic closure, along with a dimension tentatively interpreted as flexibility of grammatical closure. The substantial intercorrelations among these factors yielded four second-order dimensions, interpreted as analytical functioning or general reasoning, figural closure, symbolic closure, and semantic closure. The latter three factors each represent a combination of analytical and structuring skills (a coalescence of flexibility and speed of closure) and are distinguished by the type of content involved (figure, symbol, or meaning).

Myocarditis in juvenile rheumatoid arthritis.

Three children are described who have had myocarditis as part of juvenile rheumatoid arthritis (JRA). The diagnosis was established by the appearance of cardiomegaly or congestive heart failure or both in the absence of substantial pericardial effusion or extra cardiac cause. Myocarditis, in these cases, occurred on a background of severe, active systemic disease. No pathologic specimens from hearts of acute cases are available, but an autopsy specimen of one child who died after two months of treatment with high doses of steroids showed diffuse changes typical of the "dilated ventricle" type of cardiomyopathy. Treatment with high doses of adrenocorticosteroids has been rapidly successful in controlling the acute phase, while digoxin must be used with extreme care because of high incidence of toxicity to glycosides.

Different lipolytic effects of theophylline and dibutyryl cyclic AMP in vivo and in vitro.

Both dcAMP and theophylline are known to promote lipolysis in vitro by increasing intracellular cAMP. Although theophylline stimulates FFA mobilization in vivo as well, a report of low circulating FFA levels in the rat given dcAMP suggested that dcAMP may inhibit lipolysis in the intact animal. To explore this possibility, a comparison of the in vitro and in vivo lipolytic effects of theophylline and dcAMP was made in the young dog. Circulating glycerol and FFA levels rose following the administration of theophylline. While glycerol and FFA fell slightly in puppies given dcAMP, only the FFA change was significant. Epinephrine infusions given alone produced sustained elevations of glycerol and FFA. When theophylline was given in conjunction with ongoing epinephrine infusions, plasma glycerol and FFA levels remained high. On the other hand, epinephrine-stimulated lipolysis was markedly inhibited by dcAMP, as shown by pronounced falls of glycerol and FFA from the elevated levels found with epinephrine alone. In vitro studies involving fragments of puppy adipose tissue reveal that epinephrine, theophylline, and dcAMP promoted glycerol release. In contrast to the in vivo observations, lipolysis was also stimulated by combinations of both epinephrine and theophylline as well as by epinephrine and dcAMP. Thus, theophylline stimulates lipolysis in vitro and in vivo in the puppy. In contrast, dcAMP stimulates lipolysis in vitro but inhibits this action in the intact animal. This important difference in the two pharmacologic agents suggests the need for caution when using them in in vivo studies involving the action of cAMP.