Anisocytosis as a possible predictor of low cardiac output syndrome in children undergoing mitral valve surgery.

PURPOSE: Mitral valve surgery in children involves correcting congenital and acquired pathologies, with a reported mortality rate of 0.9%. Low cardiac output syndrome (LCOS) is a serious complication with the incidence of 20-25%. The aim of the study was to estimate possible prognostic factors of LCOS in children undergoing mitral valve procedure. MATERIAL AND METHOD: This single-center retrospective analysis enrolled children aged <18 years who underwent mitral valve surgery during 24 year period. Preoperative clinical and laboratory parameters, and operative factors were analyzed. RESULTS: Thirty consecutive pediatric patients (11 (37%) males and 19 (63%) females) in median (Q1 - Q3) age of 57 (25-115) months, who underwent mitral valve replacement, were included. The 30-day mortality was 7% (2 patients) and was related to postoperative multiorgan failure. LCOS occurred in 8 (27%) children. The receiver operator curve (ROC) analysis established parameters that have predictive value for LCOS occurrence: cardiopulmonary bypass (CPB) time, with 89 â€‹min as optimal cut-off point (AUC â€‹= â€‹0.744, p â€‹= â€‹0.011) yielding sensitivity of 100% and specificity of 42.9%; left ventricular ejection fraction (LVEF) â€‹< â€‹60 % (AUC â€‹= â€‹0.824, okp â€‹= â€‹0.001) with sensitivity of 62.5% and specificity of 93.75%; and red blood cell distribution width (RDW) above 14.5 % (AUC â€‹= â€‹0.840, p â€‹< â€‹0.001; sensitivity of 87.5% and specificity of 75%). CONCLUSIONS: In mitral valve replacement in pediatric patients, CPBtime above 89 â€‹min, preoperative LVEF below 60% and preoperative RDW above 14.5% can be regarded as the potential predictors of LCOS.

The beneficial role of simple inflammatory blood indices in pediatric cardiology.

Simple whole blood analysis can effectively demonstrate complex changes in inflammatory responses to cardiovascular disorders in adults and enable the prediction of adverse outcomes or diminished survival. Such inflammatory activation has also been detected in the pediatric population. Blood analysis results are repeatable and readily available, which gives the method an advantage over others. Inflammatory phenomena such as a high leukocyte count and an increased neutrophil-to-lymphocyte ratio (NLR) are related to a poor prognosis of advanced heart defects and worse outcomes after pediatric cardiac surgery in the advanced stages of the disease. Surgery-associated inflammation exacerbates these diseases, and the inflammatory response may further complicate the postoperative period. Simple blood cell counts and indices may be beneficial for evaluating cardiac surgery outcomes and cardiovascular disorder prognosis in infants and children. This review summarizes current knowledge on inflammatory markers in pediatric cardiovascular diseases and surgery.

Neutrophil Count as Atrioventricular Block (AVB) Predictor following Pediatric Heart Surgery.

Neutrophils play a significant role in immune and inflammatory reactions. The preoperative inflammatory activation may have a detrimental effect on postoperative outcomes. The aim of the study was to investigate the relation between preoperative hematological indices on postoperative complications' risk in pediatric cardiac congenital surgery. The retrospective single center analysis included 93 pediatric patients (48 (65%) males and 45 (35%) females), mean age of 7 (3−30) months referred for cardiac surgery in cardiopulmonary bypass due to functional single ventricle disease (26 procedures), shunts lesions (40 procedures) and cyanotic disease (27 procedures). Among simple hematological indices, the receiver-operating-characteristic curve showed that a neutrophil count below 2.59 K/uL was found as an optimal cut-off point for predicting postoperative atrioventricular block following pediatric cardiac surgery (AUC = 0.845, p < 0.0001) yielding a sensitivity of 100% and a specificity of 65.62%. Preoperative values of neutrophil count below 2.59 K/uL in whole blood analysis can be regarded as a predictive factor (AUC = 0.845, p < 0.0001) for postoperative atrioventricular block in pediatric cardiac surgery.

Circulating brain-derived neurotrophic factor, leptin, neuropeptide Y, and their clinical correlates in cystic fibrosis: a cross-sectional study.

INTRODUCTION: Cystic fibrosis (CF) involves chronic inflammation and decreased pulmonary function, which increase caloric demand. Yet, sufficient energy provision is hindered by reduced appetite and fat malabsorption. Brain-derived neurotrophic factor (BDNF), leptin, and neuropeptide Y (NPY) belong to energy balance-regulating factors. We aimed to assess their concentrations in CF patients in order to search for potential clinical correlates. MATERIAL AND METHODS: This was an exploratory, cross-sectional study. Patients' weight and height Z-scores, forced expiratory volume in 1 s (FEV1%), exocrine pancreatic status (fecal elastase-1), genotypes, and other characteristics were assessed. Serum concentrations of BDNF, leptin, NPY, IL-6, and TNF-α were measured using ELISA. RESULTS: The study enrolled 56 patients, of whom 29 (52%) were female and 17 (30%) were younger than 16 years. Median (1st-3rd quartile) mass Z-score was -0.85 (-1.56-(-0.36)); median FEV1 was 70.5% (45.0-89.5); 48 (86%) patients had exocrine pancreatic insufficiency and 8 (14%) diabetes. Overall, median concentrations were: BDNF: 33.91 ng/ml (26.40-40.43), leptin: 12.05 ng/ml (8.93-17.77), NPY: 2.86 ng/ml (1.75-4.42). None of these factors correlated with mass Z-score, FEV1%, IL-6 or TNF-α. Leptin and NPY correlated negatively (ρ = -0.62, p = 3 × 10-7); BDNF/NPY ratio was associated with leptin (ρ = 0.54, p = 2 × 10-5), BDNF/leptin ratio correlated with NPY (ρ = 0.60, p = 1 × 10-6). In a multivariable regression analysis NPY was weakly, but independently, associated with FEV1%, and leptin with age. CONCLUSIONS: BDNF and leptin were not associated with weight Z-score or FEV1%. Serum NPY concentrations seemed to be lower in CF patients with reduced pulmonary function independently of malnutrition and inflammation.

[Radiofrequency ablation of accessory pathway from noncoronary cusp of aorta after failed cryoablation]. / Zabieg ablacji pradem o wysokiej czestotliwoscidrogi dodatkowej przednio−przegrodowejw niewiencowym platku zastawki aortalnejpo nieskutecznym zabiegu krioablacji.

Successful ablation of atrial or ventricular arrhythmia from aortic sinuses of Valsalva has been well documented with low complication rate. Radiofrequency (RF) ablation of anteroseptal pathway is always challenging for the risk of atrioventricular block. Thus cryoablation is widely accepted approach. We present a 27 year-old patient with WPW syndrome and frequent palpitations referred for cryoablation of anteroseptal pathway. Extensive mapping from high anterseptal region and from noncoronary aortic cusp of aorta revealed a sharp pathway potential but several applications of cryomapping were unsuccessful. Finally, the pathway was permanently ablated with RF energy from noncoronary cusp of aorta. The mapping of aortic valve should be performed during difficult anteroseptal pathway ablation and unsuccessful cryomapping dose not preclude successful RF ablation at the same location.

[12-year follow-up of a patient after successful ablation of incessant fascicular tachycardia with biventricular heart failure and severe sinus node dysfunction]. / 12−letnia obserwacja pacjenta po skutecznej ablacji ustawicznego czestoskurczu wiazkowego przebiegajacego z obukomorowa niewydolnoscia serca i dysfunkcja osrodków automatyzmu.

We present a case of a 14 year-old boy with a incessant fascicular tachycardia with retrograde conduction and tachycardiomyopathy. Verapamil, adenosine, lignocaine and repeated cardioversions were unsuccessful in termination of tachycardia. Amiodarone however caused sinus node dysfunction and temporary electrode pacing had to be used. The patient underwent successful radiofrequency catheter ablation in the area of the left posterior fascicle. Because of ventricular extrasystoles (with QRS morphology resembling that of fascicular VT) the patient underwent second ablation which significantly reduced the number of ventricular beats. The course of subsequent 12-year follow-up was uneventful.

Mutation of the MYH7 gene in a child with hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome.

Familial hypertrophic cardiomyopathy (HCM) displays autosomal dominant inheritance with incomplete penetration of defective genes. Data concerning the familial occurrence of ventricular preexcitation, i.e. Wolff-Parkinson-White (WPW) syndrome, also indicate autosomal dominant inheritance. In the literature, only a gene mutation on chromosome 7q3 has been described in familial HCM coexisting with WPW syndrome to date. The present paper describes the case of a 7-year-old boy with HCM and coexisting WPW syndrome. On his chromosome 14, molecular diagnostics revealed a C 9123 mutation (arginine changed into cysteine in position 453) in exon 14 in a copy of the gene for beta-myosin heavy chain (MYH7). It is the first known case of mutation of the MYH7 gene in a child with both HCM and WPW. Since no linkage between MYH7 mutation and HCM with WPW syndrome has been reported to date, we cannot conclude whether the observed mutation is a common cause for both diseases, or this patient presents an incidental co-occurrence of HCM (caused by MYH7 mutation) and WPW syndrome.

Beneficial in vitro effect of N-acetyl-cysteine on oxidative stress and apoptosis.

Chronic renal failure (CRF) is usually accompanied by abnormalities of both humoral and cellular immune response. The aim of the study was to investigate the influence of N-acetyl-cysteine (NAC) on intracellular oxidative stress and apoptosis rate of T lymphocytes in children with CRF. Twenty-two children (aged 4-16, mean 7.4) with CRF treated with dialysis were enrolled in the study. Intracellular reactive oxygen species (ROS) production was quantified by mean rhodamine 123 (RHO) fluorescence intensity with flow cytometry. Annexin V FITC was used for identifying apoptotic cells. Mean fluorescence intensity (MFI), which reflected intracellular oxidative stress in T lymphocytes, was increased in patients with CRF compared with the controls (CD3+: 31.58+/-11.58 vs 22.55+/-4.97, p = 0.043; CD3+CD4+: 32.50+/-8.59 vs 27.75+/-12.76, NS; CD3+CD8+: 32.10+/-11.85 vs 20.77+/- 4.89, p =0.012). Apoptotic T lymphocytes occurred more frequently in patients with CRF treated with hemodialysis (HD) (11.36+/-6.96%) than in the controls (6.14%+/-3.36%; p = 0.025). After 24 h incubation with NAC MFI and apoptosis rate decreased significantly in all subpopulations of lymphocytes. NAC, as a strong antioxidant, has a favorable effect on intracellular oxidative stress and apoptosis rate of T lymphocytes in patients with CRF. A decreased apoptosis rate may have positive effect on functional abnormalities of T cells already found in patients with CRF.

[Intracellular synthesis of cytokines as the index of function of peripheral blood lymphocytes and monocytes in children with first relapse of nephrotic syndrome]. / Wewnatrzkomórkowa synteza cytokin jako wykladnik funkcji limfocytów i monocytów u dzieci z I rzutem zespolu nerczycowego.

BACKGROUND: Nephrotic Syndrome (NS) is a disease in which immune system function disorders play an important role. The aim of the study was an assessment of intracellular expression of cytokines in primary NS including assessment of intracellular expression of monokines (TNF-alpha, IL-8) in non-specific LPS stimulation system and intracellular expression of lymphokines (IL-2, INF-gamma, IL-4, IL-6) in experimental system with application of stimulation with ionomycin and PMA. METHODS: The study included 47 children with NS aged 2-16 years. After activation of whole blood cells in the presence of brefeldin A the cells were labelled with monoclonal antibodies against surface antigens. In the second phase the cells underwent permeabilisation and were labelled with monoclonal antibodies against individual cytokines. RESULTS: The studies performed demonstrated in children with NS relapse an increase of intracellular synthesis of cytokines characteristic of Th1 system (IL-2) and a decrease of synthesis of cytokines characteristic of Th2 system (IL-4, IL-6). In these patients an increase was also found of intracellular synthesis of proinflammatory monokines (TNF-alpha and IL-8). The studies performed, confirmed the earlier observations concerning abnormal cellular response in NS and demonstrated that differences in serum concentrations of individual cytokines may result from disturbances in their intracellular synthesis.

Echocardiographic parameters of left ventricular systolic and diastolic function in infants, children and adolescents before and after surgical correction of secundum atrial septal defect.

BACKGROUND: Left ventricular (LV) dysfunction has been described in adults with secundum atrial septal defect (ASD II) and heart failure (CHF). This anomaly has been rarely regarded as a cause of CHF in pediatric patients with ASD II. AIM: To assess LV systolic and diastolic function in patients with ASD II before and after cardiosurgery as well as to establish the prognostic value of these parameters in infants, children and adolescents with ASD II. METHODS: LV systolic (LVEF) and diastolic function parameters (E/A, DCT, IVRT) were studied using Doppler echocardiography in 104 patients aged between 1-18 years with ASD II before cardiosurgery and 4 years afterwards. These parameters were compared with similar variables in 150 healthy infants, children and adolescents. RESULTS: Before surgery LVEF was significantly lower only in infants with ASD II and CHF when compared with healthy controls. Relaxation abnormalities of LV diastolic function were observed before surgery in 40.4% of patients, especially in infants and adolescents with CHF. These disturbances were still present in about 20% of patients after surgery, especially in infants. Before and after surgery both LV and RV end-diastolic volumes were normal in about 6-10% of patients with LV diastolic dysfunction. CONCLUSIONS: In patients with ASD II and CHF diastolic dysfunction of LV is more common than systolic dysfunction. RV volume overload mainly affects LV diastolic function but it is not the only cause of CHF in patients with ASD II, especially in infants and adolescents. After surgery, normalisation of LV diastolic function is not observed in about 20% of patients with ASD II, especially in those who had CHF before operation. Special monitoring of the circulatory system is necessary in patients with ASD II operated in infancy and adolescence.