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OBJECTIVES: We investigated the incidence of thrombocytopenia in pediatric patients with Down syndrome following cardiac surgery for congenital heart disease. STUDY DESIGN: We retrospectively evaluated 162 patients (81 girls, 81 boys; mean age 26.1 ± 39.5 months) who underwent total surgical correction for congenital heart disease. The patients were divided into two groups with respect to the presence (n=118) or absence (n=44, controls) of Down syndrome. Platelet counts were performed preoperatively and on days 1 to 7 after surgery and thrombocytopenia was defined as a platelet count of less than 100,000/mm3. RESULTS: The incidence of thrombocytopenia was significantly higher in patients with Down syndrome compared to controls (61.9% vs. 34.1%, p=0.002). Severe thrombocytopenia was observed in 22% and 4.6% of cases with and without Down syndrome, respectively. Postoperative platelet counts showed sharp decreases in both groups, bottoming out on day 3 and with more significant decreases in patients with Down syndrome; they started to rise on day 4, but remained lower than baseline levels on day 7. The only significant differences between the two groups in preoperative and postoperative variables were higher incidences of reintubation (26.3% vs. 9.1%, p=0.003) and complications during intensive care (50.9% vs. 27.3%, p=0.007) in cases with Down syndrome. Comparison of patients with and without thrombocytopenia independent of Down syndrome yielded significant differences for thrombocytopenic patients with respect to age, operation age, the presence of cyanosis, type of surgery and operation time, aortic clamp and bypass times, lengths of intensive care and hospital stay, intubation and chest tube drainage times, and the incidence of postoperative complications (p<0.05). CONCLUSION: Despite higher incidence of thrombocytopenia, the presence of Down syndrome was not associated with significant differences other than increased reintubation requirement and higher complication rate during intensive care.

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We report the somatic growth characteristics of 60 infants who underwent corrective surgery for congenital heart disease. Patients were assigned to the following groups: Group 1, cyanosis with pulmonary hypertension (PH); Group 2, cyanosis without PH; Group 3, large left-to-right shunt and PH; and Group 4, left-to-right shunt or obstructive heart lesion and no PH. Weight, length, and head circumference measurements and z scores were obtained before the operation, at 45 days, and 3, 6, and 12 months after the operation. Details about dietary intake, socioeconomic status at presentation, length of stay in the intensive care unit, hospitalization period, and perioperative events were noted. The endpoint was reaching a z score > -1 for all anthropometric measurements. At presentation, 51 patients (85%) had malnutrition. The family income, dietary intake, and presence of preoperative chronic malnutrition were interrelated and influenced the weight of the patient at all times during the postoperative follow-up (p < 0.05 for all values). The severity of the heart defect had no significant influence on the postoperative anthropometric measurements (p > 0.05). The lowest preoperative z scores for weight and height were observed in Group 3. Seven patients could not achieve the endpoint at the end of 12 months (4 in Group 3 and 3 in Group 2). Catch-up growth is attained mostly in the first year after corrective surgery. Delays in reaching z scores > -1 are observed in the chronically malnourished children. If adequate calories are provided and early corrective surgery is performed, the normal growth potential may be fulfilled.

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Budd-Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Although there are no guidelines for treatment of patients with Budd-Chiari syndrome, thrombolytic therapy may be useful in patients with acute Budd-Chiari syndrome. In this report, a boy with Budd-Chiari syndrome due to membranous obstruction of the inferior vena cava treated with systemic and local administration of recombinant tissue plasminogen activator is described. We would like to emphasize the role of systemic and local fibrinolytic treatment in these patients.

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Acute renal failure (ARF) is a major complication in infants who undergo cardiac surgery. The aim of this investigation was to identify possible risk factors for ARF and mortality in this patients group. Out of 64 patients, 21 (32.8%) cases developed acute renal failure and overall mortality rate was 25%. The mortality rate was higher in the infants who developed ARF than those who did not (66.7% and 4.7%, respectively, p<0.05). Also, ARF was positively correlated with mortality (r:0.70, p<0.0001). The nonsurvivors had lower mean serum albumin than did the survivors (p<0.05), and serum albumin level was negatively correlated with mortality (r= -0.34, p< 0.05). For the patients with serum albumin level <3.5 g/dL, the unadjusted odds ratio for mortality was 4.3 (CI 95%:1.05-17.86). Total bypass time and aorta clamping time were significantly longer in the nonsurvivor group than in the survivor group (p<0.05 for both). In conclusion, the significant risk factors for mortality in these patients were development of ARF, low serum albumin level, and long total bypass and aorta clamping times, which may be predictive of poor prognosis.

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OBJECTIVE: Corrective surgery for congenital heart defects in children frequently requires cardiopulmonary bypass (CPB). Serum and bronchoalveolar levels of interleukin-6 (IL-6) may be useful in assessing the severity of the systemic inflammatory response after CPB. In the present study, the authors aimed to compare the effects of ketamine anesthesia and isoflurane anesthesia with respect to serum and tracheobronchial aspirate (TBA) IL-6 levels in infants undergoing CPB for cardiac surgery. DESIGN: Prospective and randomized controlled study. SETTING: University-based teaching hospital. PARTICIPANTS: Thirty-four infants aged 2 to 24 months were randomized into 2 groups. INTERVENTIONS: In group K (n = 17), anesthesia was induced with intravenous (IV) ketamine, 1 to 2 mg/kg, and fentanyl, 1 to 2 microg/kg, and was maintained with infusions of ketamine, 25 to 75 microg/kg/min, and fentanyl, 10 microg/kg/h. In group I (n = 17), induction was achieved with IV thiopental sodium, 3 to 5 mg/kg, and fentanyl, 1 to 2 microg/kg, and was maintained with 1% isoflurane and fentanyl, 10 mug/kg/h. Blood and TBA samples were obtained at 6 and 4 stages, respectively. MEASUREMENTS AND MAIN RESULTS: Serum IL-6 and TBA IL-6 levels were similar in the 2 groups at all stages (p > 0.05). CONCLUSION: The present results show that ketamine anesthesia does not provide superiority over isoflurane anesthesia with respect to serum and TBA IL-6 levels.

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OBJECTIVE: The authors compared the effects of remifentanil with fentanyl on the hemodynamic and respiratory variables in children with left-to-right shunting and pulmonary hypertension. DESIGN: A prospective, randomized, and controlled design. SETTING: University hospital. PARTICIPANTS: Children aged between 3 months and 6 years undergoing pediatric cardiac surgery for correction of left-to-right intracardiac shunting. INTERVENTIONS: Children were assigned to 1 of the 2 opioids for intraoperative use. Fentanyl was given as a 20 microg/kg intravenous bolus followed by infusion at a rate of 20 microg/kg/h in group 1 (control, n=15), and remifentanil was given as a 2 microg/kg intravenous bolus followed by infusion at a rate of 2 microg/kg/min in group 2 (n=18). MEASUREMENTS AND MAIN RESULTS: Mean arterial pressures at 30 to 40 minutes postbypass and the first 2 hours postsurgery were higher in the remifentanil group (p<0.05). Heart rates, pulmonary artery pressures, and airway pressures did not differ at any time between groups. Peripheral oxygen saturation values were lower at 30 and 45 minutes in the prebypass period and higher at 1 to 4 hours in the intensive care unit in the remifentanil group (p<0.05). After protamine administration, transient peripheral oxygen desaturation was observed with 10 children in the remifentanil group and with 3 children in the fentanyl group without any hemodynamic deterioration (p=0.029). CONCLUSION: There were no clinically important differences in hemodynamic and respiratory measurements intraoperatively and during the initial 24 hours postoperatively between fentanyl and remifentanil in pediatric patients undergoing surgical repair of defects with left-to-right shunts.

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We describe a patient whose features represent a new entity within the oculo-auriculo-vertebral spectrum. The boy had right microtia, atresia of the external auditory canal, growth retardation, a complex heart defect, and extra-lobar pulmonary sequestration. The cardiac anomalies were persistent left superior vena cava, aortic stenosis, bicuspid aortic valves and subaortic membrane. Spinal films revealed complete fusion of the C2-C3 and C5-C6 vertebrae, and scoliosis of the lumbar spine. The patient's mental development was normal, and there were no abnormalities on ophthalmological examination. This report, reviews features of similar published cases, and argues why this may represent a 'new' entity within the oculo-auriculo-vertebral spectrum. The cardinal features are microtia, atresia of the external auditory canal, complex cardiac defects, growth retardation, normal mental and motor development in most cases and vertebral anomalies. All six of the patients reviewed were male raising the possibility of X-linked inheritance.

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Bronchial compression due to pulmonary artery dilation is an important problem in infants with congenital heart disease, and can complicate the postoperative course. In recent years, airway stenting has become a popular treatment for these cases. We achieved success with endobronchial stenting in a two-month-old infant with bronchial compression caused by a dilated pulmonary artery.

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Coarctation of the aorta can manifest in a number of ways. Differential diagnosis is particularly difficult during the neonatal period, especially in newborns presenting with a sepsis-like or metabolic disease-like condition. We describe five newborns who presented with metabolic acidosis, renal insufficiency, or ischemic hepatitis and stress the importance of early diagnosis to prevent adverse outcome.

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