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BACKGROUND: Cardiopulmonary bypass induces a systemic inflammatory response. This study investigated, in a pediatric population, cytokine-induced responses and their potential modification by intraoperative steroid administration. METHODS: Markers of the acute-phase response were measured perioperatively in 24 children weighing less than 10 kg undergoing cardiac operations. Those having operations with cardiopulmonary bypass were randomized to receive either no steroid (group I, n = 8) or 10 mg/kg methylprednisolone in the pump prime (group II, n = 10); patients undergoing nonbypass procedures were controls (group III, n = 6). RESULTS: In all groups, plasma interleukin-6 level was elevated (p < 0.01) above baseline throughout the post-operative period, peaking earlier in group I. Levels of C-reactive protein peaked at 48 hours, and postoperative core temperature was raised in all groups. Levels of interleukin-6 from 2 to 6 hours and C-reactive protein at 24 hours postoperatively were greater (p < 0.05) in group I than in group II. Maximum interleukin-6 level, C-reactive protein level, and temperature were all significantly greater in group I than in group III. Maximum interleukin-6 level correlated with maximum C-reactive protein level in group I only (rs = 0.76; p < 0.05) and showed no association with temperature. Duration of bypass did not correlate with levels of interleukin-6. CONCLUSIONS: This study demonstrated a marked acute-phase response to operation; the greater response to procedures with cardiopulmonary bypass was abrogated by intraoperative steroid administration. The importance of interleukin-6 as an inducer of acute phase proteins after bypass is supported by its association with C-reactive protein levels, but other factors must be important in the induction of pyrexia.

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The endocrine phase of the stress response to cardiopulmonary bypass in children is known to be subtly different from that seen in adults. The aim of this investigation was to determine whether there are similar differences in the acute phase response. Thirteen children were studied (mean age 2.65 years). Each child had congenital heart disease and underwent corrective cardiac surgery. Blood samples taken two days prior to operation and at 6, 9, 12, 24, 48 and 120 hours after were analysed for C-reactive protein, albumin, caeruloplasmin, zinc and copper concentrations. Metal:carrier protein molar ratios were also calculated. Results demonstrate changes which, although similar to those seen in adults, differed both quantitatively and qualitatively. This is explained by the concept of immaturity leading to a generally poor capacity for protein synthesis and a relative inability to respond to altered circumstances.

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BACKGROUND AND AIM OF THE STUDY: The Carpentier-Edwards supra-annular valve was introduced in 1982 and was designed to incorporate the perceived benefits of low pressure fixation and supra-annular configuration. We report an eleven year experience with the valve, concentrating on its use for single valve replacement in the aortic position. METHODS: The prosthesis was implanted in 395 patients at 399 operations between January 1984 and October 1993. Myocardial revascularization was additionally performed in 122 (31%) patients. The mean age of the patient population was 65.4 years (range 22-84 years). Sex distribution was predominantly male (62%). Three hundred and seventy-two patients left hospital (30 days operative mortality 5.8%). The mean follow up extended to 3.4 years per patient, and the cumulative follow up for the series was 1264.3 patient years. RESULTS: Overall actuarial survival at six years was 75.8% +/- 4.1%. The incidence of valve-related complications expressed in terms of actuarial freedom from complication at six years resulted in a freedom from reoperation of 95.1% +/- 1.8%, from prosthetic endocarditis of 97.2% +/- 1.0%, from thromboembolism and anticoagulant-related hemorrhage of 95.9% +/- 1.3% and 95.8% +/- 1.6%, respectively. Freedom from periprosthetic leak was 99.1% +/- 0.7%, whilst freedom from structural valve deterioration was 97.6% +/- 1.5%. CONCLUSIONS: We conclude that the Carpentier-Edwards supra-annular valve compares favorably with other similar bioprostheses and that changes in its design have not impaired its structural integrity.

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BACKGROUND: A reduction in liver function is common after cardiac operations, particularly in children with preexisting cardiac failure. The etiology is multifactorial, but the redistribution of organ blood flow that occurs during cardiopulmonary bypass implicates ischemia as one of the principal causes of injury. Dopamine hydrochloride is known to have specific effects on the renal circulation, and the aim of this study was to investigate its effects on hepatic perfusion. METHODS: Eight children with congenital heart disease were studied 6 hours after the end of cardiopulmonary bypass when they were fully rewarmed and hemodynamically stable. Using noninvasive auricular densitometry, we determined the percent disappearance rate of indocyanine green as an index of liver blood flow both before and 1 hour after commencing an infusion of dopamine at 4 micrograms.kg-1.min-1. RESULTS: Results showed an increase of approximately 31% in the percent disappearance rate of indocyanine green with the addition of low-dose dopamine (4 micrograms.kg-1.min-1) (p < 0.01). CONCLUSIONS: Dopamine may have a therapeutic role in increasing hepatic perfusion and minimizing any loss in liver function.

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The aim of this study was to measure total body water in children with congenital heart disease before and after cardiac surgery and to compare the results of deuterium and 18oxygen dilution methods. Seventeen children (aged 4 to 33 months) were given aliquots of isotopically labeled water 1 week before and 6 hours after cardiac surgery. Isotope equilibration and analysis of the declining enrichment of daily urine samples allowed calculation of the total body water content. Before operation, total body water was significantly elevated (p < 0.001, Wilcoxon test); after operation it fell to approximately normal values. This finding is in contrast to those of previous reports, but may be explained in that the method used for calculation depended on measurements taken over a 7-day period rather than on a single measurement of isotope dilution as used elsewhere. Nevertheless, these results do suggest that surgery can correct the preoperative fluid overload. Comparison of deuterium and 18oxygen dilution methods showed a 2% to 2.5% overestimation of the total body water content with deuterium sampling.

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The clinical measurement of hepatic perfusion is complicated by a dual blood supply and the invasive nature of the majority of techniques available. The aim of this study was to validate indocyanine green clearance and noninvasive auricular densitometry as a measure of hepatic perfusion in the context of paediatric cardiac surgery. The effects of different dye concentrations on densitometer recording were assessed in vitro and found to have a linear relationship. Similarly, variations in haematocrit, within the range 21-47%, also had little effect on accuracy. Comparison of densitometry and direct blood sampling with plasma spectrophotometry in six postoperative, normothermic children showed no significant difference between the noninvasive and invasive techniques (r = 0.968; p > 0.05, t-test). Comparison in 10 hypothermic children during cardiopulmonary bypass also showed no significant difference between the two methods, provided that no further cooling or rewarming took place (r = 0.83; p > 0.05, Wilcoxon test). Noninvasive auricular densitometry can, therefore, provide a reliable assessment of hepatic perfusion in children undergoing cardiac surgery.

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Children with congenital heart disease may have some degree of hepatic impairment, with further impairment developing shortly after surgical correction of the cardiac defect. The redistribution of organ blood flow that occurs during cardiopulmonary bypass implicates ischaemia as one of the principal causes of injury. The aim of this study was to measure liver blood flow in children with congenital heart disease and to determine both the effects of cardiopulmonary bypass and the consequences of corrective surgery. Indocyanine green clearance and auricular densitometry, were used in 31 children. In 83% we demonstrated a reduced liver blood flow, with a mean percentage disappearance rate (PDR) of 12.9% (SEM +/- 1.2). This finding was unrelated to the patient's age, the type of congenital heart defect or the presence or absence of cyanosis. During cardiopulmonary bypass, hepatic perfusion was further reduced in 77% of children, by an average of 67%, out of proportion with the iatrogenic reduction in total body flow. Six hours after surgery, liver blood flow had increased significantly above preoperative levels (p < 0.001; t-test) to approximately normal values with a mean PDR of 20.4% (SEM +/- 1.5).

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We present a single pericardial patch repair of the sinus venosus defect with anomalously connected pulmonary veins, incorporating enlargement of the superior vena cava. In our small series to date this procedure has been carried out without morbidity or mortality. Noninvasive follow-up by echocardiography and electrocardiography, over the short term, has not detected any stenosis of the venous pathways or sinus node dysfunction.

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OBJECTIVE: To assess the nutritional status of children with congenital heart disease. DESIGN: Six anthropometric, 24 biochemical, and five haematological markers of nutritional wellbeing were measured in children with congenital heart disease. SETTING: The west of Scotland. PATIENTS: 48 children admitted consecutively for surgical correction of congenital heart disease. MAIN OUTCOME MEASURES: Height, weight, and triceps and subscapular skin fold thicknesses were considered abnormal if they were below the third centile compared with standard reference data for age matched British children. Mid-arm circumference and arm muscle circumference were considered abnormal if they fell below the fifth centile compared with standard data. Biochemical and haematological data were compared with age matched and locally validated laboratory normals. RESULTS: A marked degree of undernutrition was evident in all children; 52% had weight less than the third centile, 37% were below the third centile for height, and 12.5% were below the third centile for triceps skin fold thickness and 18.8% for subscapular skin fold thickness. Mid-arm circumference and arm muscle circumference were below the fifth centile in 20.1% and 16.7% of children respectively. Five or more of the 29 biochemical and haematological measurements were abnormal in 83.3% of patients; 10 or more were abnormal in 12.5% of patients. CONCLUSIONS: Children with congenital heart disease are frequently undernourished, irrespective of the nature of cardiac defect and the presence or absence of cyanosis.

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Failure to thrive is a common feature of children with congenital heart disease. Whether this is the result of poor nutrition or an abnormally high basal metabolic rate is unknown, yet the state of nutrition has a profound effect on the metabolic response to injury and strongly influences the outcome of surgical treatment. The aim of this study was therefore to measure the preoperative and postoperative energy requirements of children with congenital heart disease. Eighteen children (aged 4 to 33 months) were given two oral doses of doubly labeled water (H2(18)O and 2H2O), the first 1 week before operation and the second 6 hours after the end of cardiac surgery. By measuring the relative loss of each isotope from the body water pool, we were able to calculate the rate of carbon dioxide production and therefore total energy expenditure. In five patients, energy expenditure was clearly elevated, suggesting that a raised basal metabolic rate is an important factor in the observed failure to thrive in at least a proportion of such children. Postoperatively, energy expenditure fell to values below normal for healthy children (not having an operation), which suggests that the stress of surgery leads to smaller energy requirements than have previously been thought.

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During an 8-month period, 86 consecutive infants and children under 2 years of age underwent palliative or corrective cardiac surgery, of whom 11 subsequently developed phrenic nerve injury (PNI). This was seen most frequently following classic or modified Blalock-Taussig shunts. The diagnosis was established by ultrasound screening of the diaphragm, and patients were initially managed expectantly with ventilatory support. In nine patients no further management was necessary with demonstrated return of diaphragmatic function. The remaining two patients underwent plication of the diaphragm. The mean time to diaphragmatic recovery was 40.8 days and was more prolonged in patients with paradoxical, as opposed to absent, diaphragmatic movement. There were no deaths in the series. A further retrospective review of 241 patients of similar age undergoing similar surgery over the preceding 2 years revealed evidence of PNI in 11 (4.6%). Recovery of diaphragmatic function was documented in all except one patient who died. Based on these results we believe that although PNI is associated with considerable morbidity, and frequently a long stay in Intensive Care, there is evidence of spontaneous recovery of diaphragmatic function in 90% of the patients. Consequently, plication of the diaphragm can usually be avoided. Ultrasound scanning is extremely useful in establishing the diagnosis and offers assistance in predicting prognosis and deciding management.

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Triiodothyronine is an important regulator of cellular metabolism and may have potential use as an inotropic agent. The aim of this study was to determine the effects of cardiopulmonary bypass on thyroid function in infants weighing less than 5 kg. Serial measurements of triiodothyronine, thyroxine, and thyroid-stimulating hormone were made in 10 infants and corrected for the effects of hemodilution. We demonstrated a fall in triiodothyronine and thyroxine levels, with some recovery after 3 to 6 hours. An additional decrease then occurred, reaching a trough at 48 hours (representing a fall of 78% for triiodothyronine and 57% for thyroxine) before hormone levels returned to normal at 5 to 7 days. Thyroid-stimulating hormone concentrations increased and decreased, predating and complementing exactly the changes in triiodothyronine and thyroxine. These results are quantitatively and, for thyroid-stimulating hormone, qualitatively different from those previously reported in adults. In two patients who died, however, and in one who had a particularly difficult postoperative course, no increase in triiodothyronine, thyroxine, or thyroid-stimulating hormone concentrations was found after a trough had been reached at 48 to 72 hours, which suggests abnormal function at the hypothalamopituitary level.

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We have investigated the role of the lungs in the extraction of atrial natriuretic factor (ANF) by measuring plasma levels in samples taken from the central circulation in 12 patients (mean age 59 years; range 43 to 68) undergoing cardiac surgery. We also investigated the effects of cardiopulmonary bypass on ANF levels. ANF levels (mean +/- SD) were lower in pulmonary venous samples (41 +/- 20 pg/ml) than in pulmonary arterial samples (54 +/- 18 pg/ml; p less than 0.001), demonstrating 24% extraction of ANF by the lungs. Both left atrial (47 +/- 23 pg/ml) and systemic arterial levels (52 +/- 22 pg/ml) were higher than pulmonary venous levels (both p less than 0.05), indicating secretion of ANF into the left side of the heart. During cardiopulmonary bypass, plasma ANF concentration fell from 68 +/- 23 pg/ml before aortic cross-clamping to 35 +/- 13 pg/ml 10 minutes after and 28 +/- 9 40 minutes after the application of clamps (both p less than 0.001). A rebound rise to 122 +/- 33 pg/ml followed the release of the clamp (p less than 0.001). This study demonstrates that ANF is extracted by the lungs and secreted directly into the left side of the heart. The considerable fall in plasma levels that was observed during aortic cross-clamping might contribute to the neurohumoral activation and increased peripheral resistance observed after prolonged cardiopulmonary bypass and to the risk of renal ischemic injury.

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Povidone-iodine is an effective antiseptic, but its topical use has been associated with a number of adverse reactions in burn patients and in neonates as a result of transcutaneous absorption. In particular, high plasma iodine concentrations are known to cause renal failure, metabolic acidosis, and thyroid suppression. Because of the permeable nature of the skin in small infants and the large areas cleaned before cardiac operations, it is possible that significant transcutaneous iodine absorption might occur in this situation. We have studied 17 infants, less than 3 months of age, who were undergoing closed cardiac or thoracic procedures. After povidone-iodine skin preparation in 15 (covering 20% to 30% of body surface area), plasma total iodine concentrations rose fourfold (range, 160% to 1,440%). This increase was significantly different from the preoperative level at 6, 12, 18, and 24 hours. There was no increase in plasma iodine concentration in 2 patients who were not exposed to povidone-iodine or any other iodine-containing compound. We discuss the implications for a topical antisepsis policy in infants.

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OBJECTIVE: To evaluate the clinical usefulness of transoesophageal echocardiography in the assessment of children with fixed left ventricular outflow tract stenosis. PATIENTS AND METHODS: Eight consecutive children, aged over 5 years, with fixed subaortic stenosis and one child with fixed subpulmonary left ventricular outflow tract stenosis were prospectively assessed by precordial and transoesophageal echocardiography. RESULTS: Transoesophageal images of the left ventricular outflow tract were much clearer than precordial images in all patients except one with a prosthetic mitral valve. Improved visualisation provided further information on the nature of the lesion (additional chordal attachment of the mitral valve in one, accessory atrioventricular valve tissue with aneurysm formation in one), on the extent of the lesion (circumferential in three), and on the very close relation of a ridge to the aortic valve leaflets in one. Transoesophageal Doppler did not provide any additional information on aortic regurgitation and was unreliable for gradient estimation across the left ventricular outflow tract. CONCLUSIONS: Transoesophageal imaging provides an excellent means of visualising lesions in the left ventricular outflow tract and can be useful in a few children and adolescents in whom precordial echocardiography does not provide adequate information. The technique can also be used intraoperatively to define the full extent of the obstructive lesion and to assess residual lesions after surgery.

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We compared the ability of transthoracic and transoesophageal echocardiography to determine the presence and site of an atrial septal defect and associated anomalous pulmonary venous connexions in 13 school age children (aged 5 to 15 years) and 12 adults (aged 25 to 68 years). Transthoracic echocardiography detected atrial septal defects in 12 children and 6 adults. Transoesophageal echocardiography confirmed the position of 16 (13 secundum, 3 primum) of these 18 defects but altered the diagnosis from a secundum defect to a sinus venosus defect in one and from a sinus venosus defect to a high secundum defect in another. In addition to these 18, transoesophageal echocardiography diagnosed a defect in 5 adults (3 secundum and 2 sinus venosus defects) and 1 child (secundum defect). In an adult with inconclusive transthoracic findings, transoesophageal echocardiography enabled clear visualisation of the atrial septum and excluded an atrial septal defect. Transoesophageal echocardiography showed anomalous attachment of a pulmonary vein into the region of a sinus venosus defect (n = 3) but did not show anomalous connexions to the superior caval vein (n = 3) or the inferior caval vein (n = 1). Transoesophageal echocardiography provides a reliable method of diagnosing or excluding an atrial septal defect in patients with inconclusive transthoracic findings and is of particular diagnostic value in sinus venosus defects.

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Bullet emboli are an interesting but rare occurrence and their management when situated in the venous system remains controversial. Two cases of venous bullet embolism to the right heart are reported due to airgun pellet injuries. Both cases required bullet embolectomy using cardio-pulmonary bypass.

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Pulmonary artery sling is a rare abnormality that usually presents in infancy with wheeze or stridor. Diagnoses by cross sectional echocardiography, computed tomography, and magnetic resonance imaging have been described but pulmonary arteriography was regarded as essential for a definite diagnosis. The use of colour Doppler adds to the diagnostic certainty of cross sectional echocardiography and colour Doppler provided a definite diagnosis in an infant that allowed surgical repair without the need for cardiac catheterisation. Postoperative flow through the left pulmonary artery was also assessed by Doppler, and this avoided the need for repeat catheterisation or perfusion lung scanning.

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