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From 1979 through 1983, 328 of 1,388 pediatric cardiac operations involved patients undergoing their first procedure at less than 4 months of age. Of these, 220 patients had 265 nonductal procedures, and their case histories are reviewed for results and total hospital cost. Initial operative mortality was 20% (43 patients). Infants with lower operative age and operative weight tended to have closed procedures. Mortality and cure were not related to gestational age, birth weight, age at operation, number of operations, or type of operation. Lower operative weight was associated with a greater mortality. Evaluated survivors (142 patients) were followed for a mean of 24 months. Fifteen percent (33 patients) died during follow-up. Of survivors, 80% (114 patients) had optimized general health; a subset of 29% had normal cardiac function, and 17% were cured. Lower birth weight was associated with curable lesions and normalcy (p less than 0.04). Longer preoperative hospital stay and lower weight at operation were associated with higher hospital cost (p less than 0.05). Hospital cost was not related to type of operation, gestational age, birth weight, age at operation, mortality, cure, or normalcy. Acquired neurologic dysfunction and long-term disability were uncommon. The mean hospital cost for surviving infants was +80,000 (1984 dollars). Effective hospital cost per survivor was +110,000. Mortality, cure, and normal function after cardiac operations in infants less than 4 months of age were not related to gestational age, birth weight, or age at operation. Mortality was higher in patients with a lower weight at operation. Separation into distinct fiscal cost groups is not reasonable in this series. Because most survivors are in normal or optimized cardiac health, intensive cardiovascular care in this population is justified.

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Frank-lead vectorcardiograms (VCGs) were obtained from 1222 normal infants and children. By means of the Pipberger computer system, 176 different scaler and vector measurements obtained from each VCG were correlated with height, weight, race, sex, torso length, chest circumference, and chest diameters to determine the effect of anthropometric indices on the VCG wave forms. Because 5509 tests of statistical significance were performed, correlation coefficients and tests of statistical significance are reported only with p less than 0.001. Height, weight, torso length, and chest circumference show good correlation with QT interval, but these findings are reflective of the decrease in heart rate with age. The VCG does not show consistent correlations with constitutional variables when stratified by age, sex, or race. There were six instances of VCG parameters significantly greater in black children, and four instances of VCG parameters significantly greater in white children. All racial differences, while statistically significant, are small by clinical, hand measurement standards. There were 22 VCG values in boys which exceeded those in girls and only one VCG value in girls which exceeded those in boys. In the pediatric age group, racial differences in VCG wave forms are small and clinically insignificant. The Frank-lead system adequately corrects for constitutional variables in infants and children. Adequate evaluation of pediatric VCGs requires stratification of data according to age and sex.

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From 1980 through 1984, 28 children younger than 19 years (mean 7.9) underwent cardiac valve replacement with 30 mechanical prostheses. Patients were followed for a total of 471 months (mean 15.7) and received either warfarin (mean 0.16 mg/kg/day) or acetylsalicylic acid and dipyridamole (mean 6.1 and 1.9 mg/kg/day, respectively) as thromboembolism prophylaxis. The frequency and incidence of thromboembolism and hemorrhage were compared. Warfarin-treated patients were at increased risk of hemorrhage (5 of 20 [25%], or 22 per 100 patient-years, vs 0 of 10 [0%], or 0 per 100 patient-years, p less than 0.05). Three of the 5 hemorrhagic episodes were mild, and in no case was hemorrhage life-threatening. Patients who did not receive warfarin had a greater risk of thromboembolism (2 of 10 [20%], or 12 per 100 patient-years, vs 0 of 20 [0%], or 0 per 100 patient-years, p less than 0.05). Both episodes of thromboembolism were life-threatening and necessitated emergency valve replacement. Although warfarin is associated with greater risk of hemorrhage than is acetylsalicylic acid and dipyridamole, warfarin is better than antiplatelet drugs in thromboembolism prophylaxis and is indicated for anticoagulation therapy in children with mechanical cardiac prostheses.

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Morphometric measurements of 22 hearts with total anomalous pulmonary venous connection (TAPVC) were compared with measurements of 8 matched control specimens without heart disease. Each of the TAPVC specimens had a shorter left atrium, smaller left atrial surface area and larger diameter of the fossa ovalis. In addition to increased length of the right ventricle and larger circumferences for tricuspid and pulmonary valve anuli, the left ventricular contour of the ventricular septum was flat or convex in 18 of the 22 hearts; the septum was significantly longer than normal in these specimens and wider at its midportion. Because mitral and aortic valve anuli were normal in circumference, the data suggest that left ventricular volume is not decreased despite change in ventricular shape.

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Anomalous origin of the left coronary artery from the pulmonary artery is associated with myocardial infarction, left ventricular dysfunction, mitral valve dysfunction and, occasionally, intracardiac congenital abnormalities. A technique that utilizes a flap of the anterior wall of the pulmonary artery to serve as a neocoronary artery to direct aortic flow from a created aortopulmonary window to the pulmonary artery orifice of the anomalous left coronary artery was used in five patients aged 2.5 months to 4.75 years. Two patients were less than 4 months of age at operation. There was one death 2 days after operation and one late death. The two youngest patients required mitral valve replacement. Two of the three surviving patients are well at follow-up at 7 to 44 months. One patient has been lost to follow-up study. One patient had postoperative catheterization which showed an intact repair. The pulmonary artery neocoronary procedure is applicable to infants and small patients with anomalous origin of the left coronary artery from the pulmonary artery.

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Successful surgical repair of a 4-year-old boy with situs inversus [I, D, D] and corrected transposition of the great vessels (TGV) as well as hemodynamically significant ventricular septal defect (VSD), systemic atrioventricular (tricuspid) valve regurgitation, and atrial septal defect is described. Unique technical aspects of VSD and tricuspid valve repair in association with corrected TGV and situs solitus or inversus are discussed and clarified.

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During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

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A 12-year-old girl with multifocal premature ventricular contractions had two fixed-coupled ventricular ectopic foci. A third focus with variable coupling interval and a common interectopic interval satisfied criteria for ventricular parasystole, an infrequently recognized arrhythmia pattern in children.

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A left ventricular, mass-like echocardiographic density (a pseudotumor), present only during diastole and not attached to any intracardiac structure, was present in 77 of 1,820 consecutive 2-dimensional echocardiograms performed with a rotating-head transducer. Evaluation of the patients with a pseudotumor demonstrated that aortic insufficiency of mild to moderate degree was the most commonly associated lesion. Of the 61 patients with aortic insufficiency in our series, 69% had a left ventricular pseudotumor. Of the cases with a pseudotumor and aortic insufficiency, none had severe aortic insufficiency. Of the 1,759 patients without aortic insufficiency, a left ventricular pseudotumor was present in 2%. In our series, the sensitivity of left ventricular pseudotumor for aortic insufficiency was 69% and the specificity was 98%.

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Balloon atrial septostomy is an accepted method for palliation of certain types of congenital heart disease. However, malposition of the balloon may lead to cardiac perforation, avulsion of an atrioventricular valve, or laceration of the systemic or pulmonary veins. Inasmuch as single-phase fluoroscopy may not identify balloon position correctly and as biplane fluoroscopy adds significant radiation exposure, two-dimensional echocardiography has been used to assist in balloon atrial septostomy in ten infants. The catheter is advanced from the inferior vena cava to the right atrium across the foramen ovale to the left atrium with the echo transducer in the subxiphoid position. The balloon is inflated and its position within the left atrium is confirmed by echo. The catheter is withdrawn according to the technique of Rashkind. Withdrawal is halted when the balloon traverses the atrial septum. Adequate septostomy is indicated on echo by a defect at least 5 mm in diameter and by flapping of the inferior rim of the atrial septum. There were no complications using this technique and a clinically adequate septostomy was achieved in each patient. Two-dimensional echocardiography-assisted balloon atrial septostomy minimizes risk of complications and decreases exposure to ionizing radiation.

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Two infants, 101/2 and 11 1/2 months of age, underwent mitral valve replacement with Ionescu-Shiley bovine bioprosthesis for congenital cardiac defects. Both patients had early valve failure, 19 months and 4 months after implantation, due to the growth of excessive collagen on the ventricular surface of the bioprosthesis with adherence of the collagen primarily to the sewing rings and struts of the valves. The cause of this excessive collagen reaction is unknown. Further study is needed to document the true incidence of this problem.

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Two-dimensional echocardiography (2DE) was performed on 13 bioprosthetic mitral valves in 11 pediatric patients, 1 week to 46 months following implantation. Cardiac catheterization confirmed the echocardiographic findings of seven valves. The 11 normally functional bioprosthetic mitral valves by 2DE had leaflet thickness not exceeding 2.0 mm, freely moving leaflets, and normal sewing ring motion. The left atrium (LA) was enlarged only in the presence of mitral valve obstruction, atrial fibrillation, or left ventricular (LV) dysfunction. One bovine valve was stenotic due to fibrous tissue beneath normal valve leaflets; 2DE demonstrated limited leaflet motion, tissue beneath a thin valve leaflet, increase rocking motion of the sewing ring, and LA enlargement. In another patient, a bovine valve produced subaortic LV obstruction due to growth of fibrous tissue outside the stent; 2DE showed that the valve leaflets were thin and moved normally without excessive rocking of the valve ring, and identified the abnormal fibrous tissue in the LV outflow tract.

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This paper presents our experience with two-dimensional echocardiography in the detection of 61 intravascular catheters or wires in a series of 39 infants and children. It was possible to identify intravascular wires as small as 0.5 mm in diameter and intravascular catheters as small as 0.9 mm in diameter. In no instance, was an intravascular catheter not detected. The two-dimensional echocardiogram is demonstrated to be a tool to assist the physician in placement of intravascular catheters. In the catheterization laboratory, the two-dimensional echocardiogram is useful in determining catheter location, particularly in the presence of complex congenital defects. Two-dimensional echocardiographic guidance of catheters minimizes exposure to ionizing radiation.

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Thirteen patients, ranging in age from 10 months to 19 years (mean 7.8 years) and in weight from 6.6 to 60 kg (average 29.5 kg) underwent 14 operations for mitral valve replacement with a heterograft prosthesis between January 1, 1976 and July 1, 1979 for a variety of congenital or acquired lesions. Preoperative indications included severe refractory congestive heart failure in each patient with growth retardation, which was especially prominent in the younger patients. Operative mortality was 14% (2/14) with both deaths occurring within 48 hours of operation in patients less than 6 years of age. All surgical survivors had clinical improvement as manifested by relief of symptoms, decrease in heart size, and significant growth. Routine postoperative catheterization in five patients revealed good initial postoperative results in those studied, with one patient having a second study 20 months after operation. He was found to have had degeneration of his bovine prosthesis and had subsequent successful reoperation with a porcine prosthesis. Further long-term serial catheterizations are needed to further document the history of heterograft prosthesis in children, but they are preferred to mechanical valves became of the lack of need for long-term anticoagulants and the absence of thromboembolism complications. This series suggests that mitral valve replacement, when indicated by refractory congestive heart failure and growth retardation, can be successfully performed even in infants and small children. Surgery should not be postponed to allow for subsequent patient growth if the natural history of the disease is of progression.

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Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.

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