First experience on bilirubin removal with a hemoadsorption column (Lixelle®) in a child with cardiogenic liver injury.

INTRODUCTION: Hyperbilirubinemia may have deleterious effects on many organs, even after the neonatal age. Blood purification is effective in the treatment of hyperbilirubinemia. Recently some reports suggest the potential role of hemoadsorption columns in this setting. METHODS: We present the case of a 6-year-old child with severe hyperbilirubinemia due to congestive liver dysfunction, complicated by persistent inflammation, immunosuppression and catabolism syndrome (PICS). The patient was treated with a hemoadsorption column (Lixelle®) in combination with continuous veno-venous hemodiafiltration (CVVHDF). RESULTS: During treatment, a significant and rapid decrease in total bilirubin (TB) and other indices of cholestasis was observed. Furthermore, a progressive reduction in the inflammatory biomarkers (Procalcitonin, C-reactive protein) occurred. These results persisted at the discontinuation of therapy. CONCLUSIONS: To our knowledge this is the first case in which hemoadsorption with the Lixelle® adsorbing column in combination with CVVHDF has been used to manage pediatric hyperbiliribinemia secondary to cardiogenic liver injury.

Beneficial effect of fenoldopam mesylate in preventing peak blood lactate level during cardiopulmonary bypass for paediatric cardiac surgery.

OBJECTIVES: To evaluate the efficacy of fenoldopam mesylate (dose 0.2 µg/kg/min) in reducing the occurrence of hyperlactataemia (i.e. peak level of blood lactate >2.0 mM/l) during cardiopulmonary bypass (CPB) in paediatric cardiac surgery. Hyperlactataemia occurring during CPB for paediatric cardiac surgery is considered an early biomarker of an increased risk of poor outcome. METHODS: This was a dose/effectiveness clinical study applying Simon's two-stage optimal design with 5% type I error rate and 90% statistical power. Following parents' written informed consent, 53 children undergoing elective cardiac surgery with CPB between March 2009 and February 2012 were enrolled. Inclusion criteria were children weighing 3-15 kg scheduled for elective cardiac surgery and with expected CPB time of 60-180 min. Patients requiring surgery with total circulatory arrest were excluded. All patients received fenoldopam infusion at a dose of 0.2 µg/kg/min from the beginning of surgery until the end of CPB. RESULTS: The primary end-point was the evaluation of response to fenoldopam, i.e., blood lactate levels ≤2.0 mM/l. A total of 53 children, median age 5.7 months (range 11 days to 48 months) were enrolled. In the first stage, 18 of 19 (95%) children achieved normalization of lactate values. Then the study was continued to stage II by enrolling an additional 34 patients. At study conclusion, 96.2% of patients showed normalized lactate values. Fenoldopam infusion was well tolerated in all patients. No adverse events were observed. CONCLUSIONS: In this study, fenoldopam at a dose of 0.2 µg/kg/min was well tolerated in paediatric patients undergoing elective cardiac surgery with CPB. In 96.2% of patients, infusion of fenoldopam was associated with intraoperative blood lactate <2.0 mM/l.

Dexamethasone prophylaxis in pediatric open heart surgery is associated with increased blood long pentraxin PTX3: potential clinical implications.

Glucocorticoid administration before cardiopulmonary bypass (CPB) can reduce the systemic inflammatory response and improve clinical outcome. Long pentraxin PTX3 is a novel inflammatory parameter that could play a protective cardiovascular role by regulating inflammation. Twenty-nine children undergoing open heart surgery were enrolled in the study. Fourteen received dexamethasone (1st dose 1.5 mg/Kg i.v. or i.m. the evening before surgery; 2nd dose 1.5 mg/kg i.v. before starting bypass) and fifteen children served as control. Blood PTX3, short pentraxin C-reactive protein (CRP), interleukin-1 receptor II (IL-1RII), fibrinogen and partial thromboplastin time (PTT) were assayed at different times. PTX3 levels significantly increased during CPB in dexamethasone-treated (+D) and dexamethasone-untreated (-D) subjects, but were significantly higher in +D than -D patients. CRP levels significantly increased both in +D and -D patients in the postoperative days, with values significantly higher in -D than +D patients. Fibrinogen and PTT values were significantly higher in -D than +D patients in the 1st postoperative day. IL-1RII plasma levels increased in the postoperative period in both groups. Dexamethasone prophylaxis in pediatric patients undergoing CPB for cardiac surgery is associated with a significant increase of blood PTX3 that could contribute to decreasing inflammatory parameters and improving patient clinical outcome.

Discrete subaortic stenosis: incidence, morphology and surgical impact of associated subaortic anomalies.

BACKGROUND: The association between discrete subaortic stenosis and other subaortic anomalies is a well known but rarely reported occurrence. The aim of this study is to define the incidence, morphology, and surgical impact of associated anomalies of the left ventricular outflow tract in children operated on for discrete subaortic stenosis. METHODS: Between 1994 and 2000, 45 consecutive children were operated on for discrete subaortic stenosis. Patients were divided in two groups according to the obstructive lesion detected by echocardiography. RESULTS: A localized shelf was found as an isolated lesion in 31 patients (group A), whereas additional subaortic anomalies were found in 14 cases (31%) and were multiple in 5 cases (group B). The anomalies included anomalous septal insertion of mitral valve (7 cases); accessory mitral valve tissue (2 cases); anomalous papillary muscle (2 cases); anomalous muscular band (8 cases); and muscularization of the anterior mitral valve leaflet (1 case). Cardiopulmonary bypass and aortic cross-clamping times were significantly shorter in group A. There were no operative deaths nor major complications or deaths during follow-up. A gradient of 15 mm Hg or more was found at follow-up in 5 cases whereas aortic regurgitation was estimated to be not clinically significant in all but 1 patient. Six cases of recurrent subaortic stenosis were found in our series, 3 of them with other subaortic anomalies. CONCLUSIONS: This study shows that discrete subaortic stenosis can often be associated with other subaortic abnormalities. Surgical treatment of these anomalies produces excellent early and mid-term relief of obstruction without any increase in mortality and morbidity.