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OBJECTIVE: To determine the effects positive pressure ventilation have on left ventricular diastolic function in neonates after the arterial switch operation. DESIGN: Prospective case series. SETTING: Pediatric cardiac and multidisciplinary intensive care units in two university-affiliated children's hospitals. PATIENTS AND PARTICIPANTS: The patient population consisted of 12 neonates weighing 2.5-4.2 kg with D-transposition of the great arteries (DTGA) who underwent arterial switch operation. INTERVENTIONS: All patients were mechanically ventilated in a volume-targeted mode with a square wave flow pattern. The positive end-expiratory pressure was held constant. A long inspiratory time was set by extending it over three cardiac cycles. MEASUREMENTS AND RESULTS: Pulsed Doppler measurements of left ventricular diastolic function were performed during the following cardiac cycles: (1) the last diastolic period of expiration (E(L)), (2) first, second and third diastolic periods of inspiration (I1, I2, I3) and (3) the first diastolic period of expiration (E(F)). Doppler measurements of peak E wave, peak A wave, E area/A area, E area fraction, A area fraction, 0.33 area fraction and the deceleration time were made. Doppler tracings were digitized and the data obtained from three sequential study periods were averaged. Data were statistically analyzed using the repeated measures analysis of variance procedure. During (I1), there was a 21% increase in the peak E wave (0.53+/-0.06 vs 0.64+/-0.08 m/s, p < 0.01) and 28% increase in peak A wave (0.47+/-0.07 vs 0.60+/-0.08 m/s, p < 0.01) compared to (E(L)). There was a 24% increase in total area under the E and A waves when I1 was compared to E(L) (0.059+/-0.008 vs 0.073+/-0.009, p < 0.01) and there was no change in mitral valve deceleration time. Compared to the initial diastolic period during inspiration (I1), the third diastolic period during inspiration (I3) had a 38% decrease in peak E (0.64+/-0.08 vs 0.40+/-0.05 m/s, p < 0.01) and 33% decrease in peak A (0.60+/-0.09 vs 0.40+/-0.05 m/s, p < 0.01). In addition, there was a 16% reduction in total area under the E and A waves (0.073+/-0.009 vs 0.061+/-0.008, p < 0.01). There were no changes in the other diastolic indexes that reflect changes in ventricular compliance or relaxation. CONCLUSIONS: In neonates with transposition of the great arteries (TGA) after the arterial switch operation, positive pressure ventilation augments left ventricular filling during the early phase of inspiration. Prolonging the inspiratory time over three cardiac cycles results in a reduction in left ventricular filling during the third diastolic period. There were no changes in the other diastolic indexes that reflect changes in ventricular compliance or relaxation.

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The power-weighted sum of velocities (PWS) is the sum of each velocity component of the Doppler signal multiplied by its power. The purpose of this study was to determine (1) whether PWS is linearly related to volume flow and (2) whether PWS can predict the regurgitant fraction in an in vitro pulsatile flow system simulating aortic regurgitation. Doppler analysis of aortic flow was performed with an intact valve and two regurgitant valves. For each valve a linear relation between the forward flow PWS and forward flow volume was demonstrated, with excellent correlation (r = 0.99). For the valves with regurgitant orifices, the values for the PWS-derived regurgitant fraction were compared with measured regurgitant fraction. A fair correlation was demonstrated (r = 0.59), with low accuracy in prediction (error 44% +/- 24%). The PWS was inaccurate in predicting flow ratios in our in vitro system despite the strong relation with forward flow volume. The error incurred may be due to effects of filters that remove low velocity and low amplitude information.

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To determine the usefulness of systemic venous flow patterns in patients with mild/moderate right ventricular hypertension, 17 patients with isolated mild/moderate pulmonic stenosis and 17 age-matched normal children were evaluated with pulsed Doppler echocardiography. Tricuspid valve, superior vena caval, and hepatic vein pulsed Doppler recordings were obtained with simultaneous respirometry and electrocardiography. Peak velocities and velocity-time integrals were measured for Doppler signals corresponding with ventricular systole, ventricular diastole, ventricular end systole, and atrial systole. The groups were similar in weight, heart rate, tricuspid inflow Doppler echocardiograms, and cardiac indexes. Compared with normal subjects, patients showed changes in respiratory variation for some superior vena caval and hepatic vein indexes. In addition, hepatic vein measurements made at ventricular end systole were significantly lower and measurements made at atrial systole were significantly higher in patients than in normal subjects. These changes in systemic venous flow patterns may provide a sensitive indicator of early right-sided heart dysfunction.

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The usefulness of two-dimensional and Doppler echocardiography during buttoned double-disk device closure of an atrial septal defect was evaluated in 20 consecutive patients at the time of interventional catheterization. Transesophageal echocardiography was used in 11 patients (ages 5 to 62 years, weights 20 to 91 kg). Because of the size of the available transesophageal echo probe, transthoracic echocardiography was used in the remaining 9 patients (ages 4 to 5.5 years, weights 14 to 21 kg). In the transesophageal echo group, 1 patient was found to have no atrial septal defect despite a previous diagnosis by transthoracic echocardiography, 3 patients had atrial septal defects too large for closure despite attempts in 2, and 7 patients had transesophageal echo guided device placement. All of these 7 patients had small residual shunts by color Doppler, 2 had unusual arm positions, and 2 had surgical removal of the device due to embolization to the pulmonary artery in 1 and failure to obtain close approximation of the occluder and counteroccluder in 1. In the transthoracic echo group, 2 patients had atrial septal defects too large for closure, 1 patient had no femoral venous access, and 6 patients had transthoracic echo guided device placement. All of these 6 patients had small residual shunts by color Doppler and 3 of the 6 had unusual arm positions. For atrial septal defect sizing, transesophageal echo measurements correlated with catheter balloon size more closely than did transthoracic echo measurements (r 2 = 0.97 vs 0.86).(ABSTRACT TRUNCATED AT 250 WORDS)

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Although the early mortality for repair of truncus arteriosus has decreased in the modern era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair, we reviewed 46 neonates and infants undergoing repair of truncus arteriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was performed beyond the first month of life in only 8 patients, because of late referral in 7 and severe noncardiac problems in 1. Associated cardiac anomalies were frequently encountered, the most common being interrupted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hypoplastic pulmonary arteries (n = 4), and major coronary artery anomalies (n = 3). Truncal valve replacement was performed in 5 patients with severe regurgitation, 3 of whom also had truncal valve systolic pressure gradients of 30 mm Hg or more. The truncal valve was replaced with a mechanical prosthesis in 2 patients and with a cryopreserved homograft in 3 patients. Right ventricle-pulmonary artery continuity was established with a homograft in 41 patients (range 8 mm to 15 mm), a valved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved polytetrafluoroethylene tube in the remaining patient (8 mm). There were 5 hospital deaths (11%, 70% confidence limits 7% to 17%). Multivariate and univariate analyses failed to demonstrate a relationship between hospital mortality and age, weight, or associated cardiac anomalies. Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-up from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac deaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted in excellent survival. We continue to recommend repair promptly after presentation, optimally within the first month of life.

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Ultrasound theory suggests that the volume of flow is directly related to the power and amplitude of the backscattered Doppler signals. To evaluate the accuracy of volume flow calculated with power-weighted and amplitude-weighted mean velocities (PWMV and AWMV), volume flows were measured in a pulsatile flow-tank system equipped with a 1.25 cm diameter simulated femoral artery. Analyses were performed throughout a range of physiologic flows, mean driving pressures, and pulse rates. At each hemodynamic setting, volume flow in the simulated artery was measured with an electromagnetic flow probe and with pulsed Doppler echocardiography by use of 7.0 and 3.5 MHz transducers. In addition, to determine the effects of vessel size and parabolic flow on the accuracy of the Doppler volumes, volume flow was evaluated in several differently sized vessels at sampling distances of 20 times the vessel diameter downstream from the orifice. On the ultrasound system, PWMV was calculated as the sum of the individual velocities multiplied by their respective power fractions (the fraction of the total instantaneous power represented by the individual signal power). The instantaneous PWMV was plotted continuously in time and superimposed on the spectral recording. Similarly, AWMV was calculated with amplitudes measured as the square root of the signal power. The PWMV and AWMV were integrated over the flow period and multiplied by the known cross-sectional area of flow to obtain the Doppler volume. In all analyses performed, volumetric flows calculated with Doppler echocardiography with PWMV and AWMV correlated extremely well with those measured with the electromagnetic flow probe. Thus, over a wide range of physiologic conditions, transducers frequencies, and vessel sizes, volume flow can be accurately calculated from PWMV and AWMV Doppler data. This technique provides an accurate, automatic method for on-line determination of volumetric flow.

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Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.

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To assess long-term femoral artery complications after aortic balloon valvuloplasty or coarctation balloon angioplasty, we examined 19 children who were 3 weeks to 21 years old (mean 7.6 years) at the time of catheterization. Two-dimensional and Doppler echocardiographic examinations of the common, superficial, and deep femoral arteries were performed at an average of 2.0 years after balloon dilatation. Pulsatility index (PI) was calculated as the maximum velocity minus the minimum velocity divided by the mean velocity. No patient was suspected clinically of having peripheral arterial disease prior to the echocardiographic examination. Fourteen patients had normal femoral arteries. Of these, 10 had normal two-dimensional and Doppler echocardiographic examinations of both femoral arteries. These patients had triphasic flow patterns (forward in systole, reverse in early diastole, forward in middiastole) and Pls of 3.7-41.6 (mean 9.5). Four of the 14 normal patients had abnormal pulsed Doppler examinations showing continuous forward flow and low Pls (1.7-3.5) reflecting residual coarctation (10-30 mmHg gradients). Five patients had abnormal femoral arteries. Of these, two had no visible obstruction by two-dimensional echocardiography and color-flow imaging but had abnormal pulsed Doppler patterns (continuous forward flow and low Pls of 2.5 and 2.9) only on the side of the balloon catheter insertion. Three of the five abnormal patients had visible obstructions by two-dimensional echocardiography and color-flow imaging and had abnormal pulsed Doppler patterns (continuous forward flow and low Pls from 1.1-3.6).(ABSTRACT TRUNCATED AT 250 WORDS)

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To evaluate the early diastolic peak filling rate of the left ventricle, three groups of children (normal children, patients with aortic valvular stenosis, and patients with aortic coarctation) were examined with the peak filling rate normalized to stroke volume calculated from the mitral valve inflow Doppler recording as the peak E velocity divided by the velocity time integral. The normal value for this index in children was 6.78 +/- 0.99 SV/sec and did not vary with age, weight, body surface area, or heart rate. Compared with normal subjects, both patients with aortic stenosis and patients with coarctation had increased left ventricular mass, but patients with aortic stenosis had decreased normalized peak filling rates (5.3 +/- 0.84 SV/sec, p < 0.01), while patients with coarctation had normal rates (6.79 +/- 0.98 SV/sec, p = 0.97). Compared with patients with aortic coarctation, patients with aortic stenosis had higher Doppler gradients. Thus the Doppler index of peak filling rate normalized to stroke volume is particularly useful in children because it is independent of heart rate, age, weight, and body surface area. Patients with coarctation may have normal peak filling rates normalized to stroke volume despite increased left ventricular mass because of milder obstruction or better coronary artery perfusion compared with that of patients with aortic stenosis.

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The development of two-dimensional and Doppler echocardiography has provided a noninvasive technique for the diagnosis and serial assessment of patients with subvalvular aortic stenosis. The clinical records and echocardiographic data were reviewed of all patients with subaortic stenosis diagnosed between 1983 and 1991. Of the 77 patients identified (45 male and 32 female), 28 had isolated subaortic stenosis and 49 had associated cardiac lesions. The most frequently encountered associated lesions were ventricular septal defect (n = 19) and coarctation of the aorta/interrupted aortic arch (n = 14). Serial echocardiographic studies, performed in 38 of the 77 patients, documented significant progression of the left ventricular outflow tract gradient in 25 patients (66%) and development of aortic regurgitation in 25 patients (66%). Surgical resection was performed in 36 patients. The preoperative outflow tract peak gradient was 62.9 +/- 31 mm Hg (range 0 to 153), whereas the immediate postoperative gradient was 14.4 +/- 14 mm Hg (range 0 to 67). The two patients with a significant residual gradient (37 and 67 mm Hg, respectively) in the immediate postoperative period had severe subaortic stenosis preoperatively with marked left ventricular hypertrophy and intracavitary gradient. The immediate postoperative echocardiograms demonstrated no worsening of aortic regurgitation in any patient and regression of regurgitation in one patient from mild to none. Intermediate-term follow-up studies were available for review in 13 postoperative patients at a mean of 4 years postoperatively. In 2(15%) of these 13 patients, subaortic stenosis recurred; however, the degree of aortic regurgitation did not increase in any patient.(ABSTRACT TRUNCATED AT 250 WORDS)

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An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.

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In atrioventricular (AV) septal defect, the common AV valve can have a common orifice or can be divided by bridging leaflet tissue into two separate orifices. To determine the accuracy of a two-dimensional echocardiographic technique devised specifically for evaluation of the number of AV valve orifices, all 69 children undergoing surgical repair of AV septal defect from April 1987 to August 1990 were examined prospectively. The presence of bridging leaflet tissue and the number of AV valve orifices were determined with use of a subcostal imaging plane. From a standard subcostal four-chamber view, the plane of sound was rotated 30 degrees to 45 degrees clockwise until the AV valve was seen en face. The plane of sound was then tilted from a superior to an inferior direction so that cross-sectional views of the AV valve were examined from the inferior margin of the atrial septum to the superior margin of the ventricular septum. Of the 69 patients, 6 (9%) were excluded because the appropriate subcostal images were not obtained (in 3 because of obesity and in 3 as a result of operator failure). The remaining 63 children, ranging in age from 1 day to 13.5 years and in weight from 1 to 55 kg, constituted the study group. Echocardiographic results were compared with surgical observations in 62 patients and with autopsy findings in 1 patient. With the two-dimensional echocardiographic technique, 32 of 33 patients with a common orifice and 28 of 30 patients with two separate AV valve orifices were correctly identified.(ABSTRACT TRUNCATED AT 250 WORDS)

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To determine the usefulness of echocardiographic indexes of left ventricular (LV) function as possible predictors of cardiac rejection, 12 transplant recipients (ages 3 to 17 years) underwent a total of 52 serial echocardiographic examinations and cardiac biopsies. The results were compared to those of 12 normal children (ages 2 to 17 years). Biopsies were graded as no rejection (n = 23), mild rejection (cellular infiltrate, n = 13), and moderate rejection (myocyte necrosis, n = 16). LV dimensions, percent shortening fraction, indexed LV mass, and ejection fraction were measured from M-mode and two-dimensional echocardiography. From the mitral valve Doppler tracing, the following measurements were made: isovolumic relaxation time, peak E and peak A velocities, and the fraction of filling under the E and A waves as well as in the first third of diastole. Compared with normal subjects, transplant recipients with no rejection had higher heart rates (95 +/- 15 vs 80 +/- 17 beats/min), longer isovolumic relaxation time (68.8 +/- 11.2 vs 51.5 +/- 13.6 msec), decreased first third area fraction (0.48 +/- 0.10 vs 0.57 +/- 0.10), and similar shortening fraction, LV mass, and peak E and A velocities (p less than 0.03). Compared with transplant recipients with no rejection, patients in whom mild rejection developed also had decreased shortening fraction (31% +/- 10% vs 37% +/- 8%) and decreased peak E velocity (0.68 +/- 0.19 vs 0.88 +/- 0.15 m/s) (p less than 0.03). From mild to moderate rejection, no further changes were noted in any echocardiographic indexes measured.(ABSTRACT TRUNCATED AT 250 WORDS)

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