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Abstract: Low output syndrome significantly increases morbidity and mortality of cardiac surgery and lengthens the durations of intensive care unit and hospital stays. Its treatment by catecholamines can lead to undesirable systemic and cardiac complications. Levosimendan is a calcium sensitiser and adenosine triphosphate (ATP)-sensitive potassium channel (IK,ATP) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O2 supply and demand, and possesses cardioprotective and organ protective properties as well. Based on the scientific literature and experts’ opinions, a European recommendation was published on the perioperative use of levosimendan in cardiac surgery in 2015. Along this line, and also taking into consideration cardiac surgeon, anaesthesiologist and cardiologist representatives of the seven Hungarian heart centres and the children heart centre, the Hungarian recommendation has been formulated that is based on two pillars: literature evidence and Hungarian expert opinions. The reviewed fields are: coronary and valvular surgery, assist device implantation, heart transplantation both in adult and pediatric cardiologic practice. Orv Hetil. 2018; 159(22): 870–877. more...

OBJECTIVES Fluid overload after pediatric cardiac surgery is common and has been shown to increase both mortality and morbidity. This study explores the risk factors of early postoperative fluid overload and its relationship with adverse outcomes. DESIGN Secondary analysis of the prospectively collected data of children undergoing open-heart surgery between 2004 and 2008. SETTING Tertiary national cardiac center. PATIENTS One thousand five hundred twenty consecutive pediatric patients ( more...

Background: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery. Methods: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center. Occurrence, treatment options and long term outcomes were analyzed. Chylothorax patients less than 2 years of age were analyzed using propensity-matched statistical analysis in regard to postoperative complications after discharge. Results: During the 10-year period, 48 patients had chylothorax after pediatric cardiac surgery. The highest incidence was observed on the second postoperative day (7 patients, 14.6%). Seven patients (14.6% of the chylothorax population) died. During the follow up period, 5 patients had additional thromboembolic complications (2 had confirmed thrombophilia). Eleven patients had a genetic abnormality (3 had Down’s syndrome, 3 had Di-Giorge’s syndrome, 1 had an IgA deficiency and 4 had other disorders). During the reoperations (49 cases), no chylothorax occurred. After propensity matching, the occurrence of pulmonary failure (P=0.001) was significantly higher in the chylothorax group, and they required prolonged mechanical ventilation (P=0.002) and longer hospitalization times (P=0.01). After discharge, mortality and neurologic and thromboembolic events did not differ in the matched groups. Conclusions: Chylothorax is an uncommon complication after pediatric cardiac surgery and is associated with higher resource utilization. Chylothorax did not reoccur during reoperations and was not associated with higher mortality or long-term complications in a propensity matched analysis. more...

The pediatric-modified Risk, Injury, Failure and Loss, and End-Stage (pRIFLE) criteria and a different but conceptually similar system termed Acute Kidney Injury Network (AKIN) were created to standardize the definition of acute kidney injury (AKI) in children. Kidney Disease: Improving Global Outcomes (KDIGO) currently recommends a combination of AKIN and pRIFLE in AKI. This study aimed to compare the three classifications for predicting AKI in pediatric patients undergoing cardiac operations.We analyzed the prospectively collected data of 1,489 consecutive pediatric patients undergoing cardiac operations between January 2004 and December 2008. AKI presence and severity was assessed for each classification using the change in serum creatinine and estimated creatinine clearance levels calculated by the Schwartz equation.AKI was present in 285 (20%), 481 (34%), and 409 (29%) patients according to the AKIN, pRIFLE, and KDIGO systems, respectively. The KDIGO classification categorized 121 patients (8%) who were placed in the AKIN 0 category, whereas the pRIFLE system categorized 74 (5%) in KDIGO 0 and 200 (14%) in AKIN 0 stages as having an AKI. The overall mortality rate was 3.9%. The KDIGO stage III (odds ratio [OR], 18.8; 95% confidence interval [CI], 9.6 to 36.6, p0.001), the AKIN stage III (OR, 38.3; 95% CI, 20.6 to 70.9, p0.001), and pRIFLE failure group (OR, 13.6, 95% CI, 7 to 26.3; p0.001) were associated with increased mortality.The pRIFLE system was the most sensitive test in detecting AKI, and this was especially so in the infant age group and also in the early identification of AKI in low-risk patients. The AKIN system was more specific and detected mostly high-risk patients across all age groups. The KDIGO classification system fell between pRIFLE and AKIN in performance. All three had increasing severity of AKI associated with mortality. more...

Measurement of the global end-diastolic volume index (GEDI) by transpulmonary thermodilution (TPTD) has become a useful technique for measuring preload in adults. This study aimed to investigate the hemodynamic changes in neonates during the postoperative period after arterial switch surgery. Over a 13-month period, the postoperative data of 12 neonates with transposition of the great arteries were retrospectively investigated. Arterial and central venous blood pressures were monitored, Cardiac index (CI), stroke volume index (SVI), systemic vascular resistance index (SVRI), GEDI, and extravascular lung water index (ELWI) were measured by thermodilution. The CI was significantly correlated with the SVRI only in the closed chest condition (r = −0.92; P < 0.001). The CI and SVI values were significantly lower and the ELWI and SVRI values significantly higher in both the open and closed chest conditions than the postextubation values. The relationship between change in GEDI and change in CI was stronger in the open chest condition (r = 0.93; P < 0.006) than in the closed chest condition (r = 0.75; P = 0.055). However, the latter just missed statistical significance. According to the findings, TPTD seems to be a useful tool for assessing cardiac function after neonatal arterial switch surgery. Establishment of normal values will be essential for proper guidance of therapy for this population using volumetric parameters. more...

Objective: To investigate the associations of blood glucose (BG) parameters and postoperative complications following paediatric cardiac surgery in the presence and absence of insulin treatment. Methods: Prospectively collected perioperative data on 810 consecutive patients who underwent surgery for congenital heart disease were retrospectively analysed. A combined outcome of death and multiple organ dysfunction (any two of the followings: infectious, cardiac, pulmonary, renal or neurological complications) was considered as the endpoint. Results: In total, 110 patients developed the combined endpoint and 32 of these patients died during the perioperative period. Patients treated with insulin were younger, smaller and underwent more complex procedures. They had higher peak BG levels and higher daily BG fluctuation, however, BG parameters were not associated with adverse outcome. In patients without insulin treatment, peak BG values higher than 250 mg/dl (OR, 7.65; 95% CI, 1.06–55.17; p=0.043) and BG fluctuation exceeding the level of 150 mg/dl (10.72; 1.74–65.90 p=0.010) on the first postoperative day were independently associated with the combined endpoint. Conclusions: Peak BG level and BG fluctuation on the first postoperative day were associated with the combined endpoint of complications and death but these results were only confined to patients without insulin treatment. more...

Summary Background: Natriuretic peptide levels are associated with cardiac output and ventricular function. We hypothesized that concomitant measurement of the peptide fragments and the hemodynamic parameters could elucidate the associations of these parameters after pediatric cardiac surgery. Methods: After approval of the institutional review board and parents’ informed consent, we investigated the clinical data of eight neonates undergoing correction of transposition of the great arteries. We measured the level of N-terminal fragments of prohormones of atrial and brain natriuretic peptides (NT-proANP, NT-proBNP) preoperatively, postoperatively and 12, 24, 48, and 72 h after arrival in the intensive care unit. The hemodynamic status was assessed by transpulmonary thermodilution at the same time points. Creatinine and other laboratory values were analyzed in the first 48 h postoperatively. Results: NT-proBNP levels were inversely correlated with cardiac index (CI, r = −0.47, P = 0.030), stroke volume index (r = −0.65, P = 0.005), and global end-diastolic volume index (GEDI; r = −0.63, P = 0.011). There was strong inverse correlation between the change of NT-proBNP levels and the change of CI between two consecutive measurements during the postoperative period (r = −0.79, P = 0.001). The NT-proBNP level 12 h after surgery was strongly correlated with the creatinine level of the postoperative 24th hour (r = 0.81, P = 0.014). Conclusions: NT-proBNP correlated with the hemodynamic parameters and with the severity of renal dysfunction. Therefore, NT-proBNP is a reliable indicator of the circulatory state and the severity of a low output syndrome after arterial switch operation in neonates. more...

INTRODUCTION The aim of this study was to investigate the role of the insulin and glucose content of the maintenance fluid in influencing the outcomes of pediatric patients undergoing heart surgery. METHODS A total of 2063 consecutive pediatric patients undergoing cardiac surgery were screened between 2003 and 2008. A dextrose and an insulin propensity-matched group were constructed. In the dextrose model, 5% and 10% dextrose maintenance infusions were compared below 20 kg of weight. RESULTS A total of 171 and 298 pairs of patients were matched in the insulin and glucose model, respectively. Mortality was lower in the insulin group (12.9% vs. 7%, p = 0.049). The insulin group had longer intensive care unit (ICU) stay [days, 10.9 (5.8-18.4) vs. 13.7 (8.2-21), p = 0.003], hospital stay [days, 19.8 (13.6-26.6) vs. 22.7 (17.6-29.7), p < 0.01], duration of mechanical ventilation [hours, 67 (19-140) vs. 107 (45-176), p = 0.006], and the incidence of severe infections (18.1% vs. 28.7%, p = 0.01) and dialysis (11.7% vs. 24%, p = 0.001) was higher. In the dextrose model, the incidence of pulmonary complications (13.09% vs. 22.5%, p < 0.01), low cardiac output (17.11% vs. 30.9%, p < 0.01), and severe infections (10.07% vs. 20.5%, p < 0.01) was higher, and the duration of the hospital stay [days, 16.4 (13.1-21.6) vs. 18.1 (13.8-24.6), p < 0.01] was longer in the 10% dextrose group. CONCLUSIONS Insulin treatment appeared to decrease mortality, and lower glucose content was associated with lower occurrence of adverse events. more...

OBJECTIVES: The incidence of congenital heart disease is 50%, mostly related to endocardial cushion defects. The aim of our study was to investigate the postoperative complications that occur after paediatric cardiac surgery. METHODS: Our perioperative data were analysed in paediatric patients with Down syndrome undergoing cardiac surgery. We retrospectively analysed the data from 2063 consecutive paediatric patients between January 2003 and December 2008. After excluding the patients who died or had missing data, the analysed database (before propensity matching) contained 129 Down patients and 1667 non-Down patients. After propensity matching, the study population comprised 222 patients and 111 patients had Down syndrome. RESULTS: Before propensity matching, the occurrences of low output syndrome (21.2 vs 32.6%, P= 0.003), pulmonary complication (14 vs 28.7%, P< 0.001) and severe infection (11.9 vs 22.5%, P= 0.001) were higher in the Down group. Down patients were more likely to have prolonged mechanical ventilation [median (interquartile range) 22 (9–72) h vs 49 (24–117) h, P= 0.007]. The total intensive care unit length of stay [6.9 (4.2–12.4) days vs 8.3 (5.3–13.2) days, P= 0.04] and the total hospital length of stay [17.3 (13.3–23.2) days vs 18.3 (15.1–23.6) days, P= 0.05] of the Down patients were also longer. Mortality was similar in the two groups before (3.58 vs 3.88%, P= 0.86) and after (5.4 vs 4.5%, P= 1.00) propensity matching. After propensity matching, there was no difference in the occurrence of adverse events. CONCLUSIONS: After propensity matching Down syndrome was not associated with increased mortality or complication rate following congenital cardiac surgery. more...

Background The objective of this study was to identify the postoperative risk factors associated with the conversion of colonization to postoperative infection in pediatric patients undergoing cardiac surgery. Methods Following approval from the Institutional Review Board, patient demographics, co-morbidities, surgery details, transfusion requirements, inotropic infusions, laboratory parameters and positive microbial results were recorded during the hospital stay, and the patients were divided into two groups: patients with clinical signs of infection and patients with only positive cultures but without infection during the postoperative period. Using propensity scores, 141 patients with infection were matched to 141 patients with positive microbial cultures but without signs of infection. Our database consisted of 1665 consecutive pediatric patients who underwent cardiac surgery between January 2004 and December 2008 at a single center. The association between the patient group with infection and the group with colonization was analyzed after propensity score matching of the perioperative variables. Results 179 patients (9.3%) had infection, and 253 patients (15.2%) had colonization. The occurrence of Gram-positive species was significantly greater in the colonization group (p = 0.004). The C-reactive protein levels on the first and second postoperative days were significantly greater in the infection group (p = 0.02 and p = 0.05, respectively). The sum of all the positive cultures obtained during the postoperative period was greater in the infection group compared to the colonization group (p = 0.02). The length of the intensive care unit stay (p Conclusions Based on our results, we uncovered independent relationships between the conversion of colonization to infection regarding positive S. aureus and bloodstream results, as well as significant differences between the two groups regarding postoperative C-reactive protein levels and white blood cell counts. more...

Transpulmonary thermodilution (TPTD) is an increasingly popular method used to monitor the complex hemodynamic changes in critically ill children. The purpose of our study was to examine the relationship between transthoracic echocardiographic (TTE) parameters and global hemodynamic variables derived from TPTD and those derived from conventional measurements in infants and neonates undergoing corrective cardiac surgery.After approval from the Ethics Committee of Gottsegen György Hungarian Institute of Cardiology and individual parental consent were obtained, patients were prospectively enrolled. In parallel with continuous postoperative conventional monitoring, TPTD was measured four times daily, and TTE was performed once per day. Conventional hemodynamic, TPTD and TTE parameters were compared with weighted linear regression statistics and a Pearson correlation.One hundred forty-five TPTD measurements and 35 TTE examinations of thirteen enrolled patients were analyzed. Global end-diastolic volume index (GEDVI) was correlated with the fractional shortening (SF, r=0.67, P=0.001) measured by TTE. Among the preload parameters, the percentage change of GEDVI between two consecutive time points showed a pertinent correlation with changes of cardiac index (r=0.67, P=0.001) and changes of stroke volume index (r=0.57, P=0.008). Percentage changes in SF demonstrated a strong negative correlation with changes of left ventricular end-systolic diameter (r=-0.86, P0.001). There was no significant relationship between alterations in arterial or central venous pressure values with TTE or TPTD parameters.Both TPTD and TTE may be used in the estimating volumetric preload parameters. The time course of TPTD-derived parameters may have clinical relevance in pediatric critical care practice. more...

The RIFLE (risk, injury, failure, loss, and end-stage renal disease) classification system was developed to standardize the definition of acute kidney injury (AKI) in adults. We hypothesized that AKI was associated with increased mortality and morbidity.Acute kidney injury was defined as a decrease in the amount of estimated creatinine clearance based on pediatric-modified RIFLE (pRIFLE) criteria. Using propensity score analysis, 325 patients who had AKI were matched to 325 patients who did not have AKI from a database of 1,510 consecutive pediatric patients who underwent cardiac surgery between January 2004 and December 2008 at a single center. The association between AKI and outcome was analyzed after propensity score matching of perioperative variables.Four hundred eighty-one patients (31.9%) had AKI according to the RIFLE categories. Of those 1,510, 173 (11.5%) reached pRIFLE criteria for risk; 26 (1.7%) reached the criteria for injury; and 282 (18.7%) reached the criteria for failure. Fifty-five patients (3.6%) died. The 2 matched groups were well balanced in terms of measured perioperative variables. Mortality rate was 5.2% in the AKI and 2.5% in the matched control group (p=0.09). Occurrence of low cardiac output syndrome (p=0.002), need for dialysis (p0.001), and infection (p=0.03) were significantly higher, and duration of mechanical ventilation (p0.001) and length of intensive care unit stay (p0.001) were significantly longer compared with the matched control group.Acute kidney injury was independently associated with an increased occurrence of postoperative complications but not with mortality after pediatric cardiac surgery. more...

Background Blood transfusion in adults is associated with increased mortality and morbidity after cardiac operations. The aim of this study was to identify the main predictors of blood transfusion and explore the relationship between blood transfusion and adverse outcomes in a pediatric population. Methods We retrospectively analyzed a prospectively collected database (January 2002 to December 2003) of 657 consecutive pediatric patients undergoing open heart procedures in a tertiary pediatric cardiac center. Risk models were calculated for each blood product and for the total amount of blood transfused during the operation and in the first 24 hours. Postoperative adverse events were investigated after propensity score adjustment. Results During the postoperative period, 30 patients (4.6%) died, 80 (12.2%) sustained nonvascular pulmonary complications, and 113 (17.2%) had infection. The risk model for the total amount of blood transfusion included weight, preoperative creatinine clearance, preoperative mechanical ventilation, duration of operation and cross-clamp, surgeon, delayed chest closure, inotropic dose, and nitric oxide administration. Univariate analyses demonstrated significant associations between blood transfusion and occurrence of every complication except of neurologic events. After adjustment for propensity score and disease severity, the total amount of blood transfusion was independently associated with an increased risk for infections (odds ratio, 1.01; 95% confidence interval, 1.002 to 1.02; p = 0.01). Transfusion of platelets was associated with lower incidence of nonvascular pulmonary complications (odds ratio, 0.89; 95% confidence interval, 0.79 to 0.99; p = 0.049). Conclusions The amount of blood transfusion is independently associated with infections but not with mortality. more...

At 28th week of gestation a conotruncal malformation with ventricular septal defect was diagnosed by fetal echocardiography. Postnatal echocardiographic and angiocardiographic examinations confirmed the diagnosis of conotruncal malformation (pulmonary atresia, ventricular septal defect, patent ductus arteriosus, aortopulmonary collateral arteries). The unifocalization (age: 11 months) and total correction with aortic homograft (age: 7 years) were performed. To our knowledge our case is the first whose intrauterine diagnosis of complex congenital heart disease was confirmed after delivery and had successful two-stage surgical management. more...

The total cavopulmonary anastomosis is one of the alternative surgical procedures which can be performed in the management of the most complicated congenital heart diseases. It was the first time in Hungary that this surgical management was performed successfully in a girl with univentricular heart, pulmonary valve stenosis, malposition of the great arteries, who was operated on palliative procedure previously. more...

After palliative procedure a 2 years old girl with congenital heart disease was corrected with aortic homograft. Truncus arteriosus is a rare congenital heart disease and this is the first successfully corrected case in our country. Using homograft in this age group improves the early and late result of this type of corrective surgery. more...

"In vitro" sensitivity of lectin (PHA, Con A)-stimulated lymphocytes to antitumor drugs (ARA-C, ADR, VM26, MTX, CP, VCR, Vepesid, ACLA) and the clinical efficiency of the complex therapy was compared in 7 patients with ALL and 2 patients with NHL. H3-thymidin incorporation of lymphocytes labelled prior to the drug exposure was used as "in vitro" method. A fairly good correlation was found between the "in vitro" test and the clinical response to the drug administered. These results suggest that this "in vitro" test is useful in choosing the drugs to be administered in case of malignancies of children. more...

Summary Background: Early extubation after cardiac surgery in children is feasible; however, predictors of prolonged mechanical ventilation (MV) should be recognized as soon as possible. Methods: At a tertiary pediatric cardiac center, prospective case series analyses were carried out with a total of 411 patients within 1 year of cardiac surgery. Perioperative factors were evaluated for strength of association with duration of MV >61 h (medium, MMV) and >7 days (long, LMV). Two multiple regression models were performed for both cut-off points: one model considered factors identified until 24 h postoperation, the other was performed with all parameters. Results: One hundred and three patients (25%) were still intubated after 61 h; 38 patients required LMV and they occupied 33% of total intensive care unit (ICU) bed days. If factors occurring until 24 h after surgery were analyzed, duration of cardiopulmonary bypass (CPB), intraoperative transfusion, post-CPB arterial oxygen tension (PaO2/FiO2), and fluid intake on the first day were found to be associated with MMV. Urea nitrogen value, nitric oxide treatment, delayed sternal closure, and tracheobronchomalacia, measured at the same point of time, were independent predictors of LMV. Of all the studied clinical predictors, MMV was associated with pulmonary hypertensive events, delayed sternal closure, peritoneal dialysis, nonvascular pulmonary problems, low output syndrome and fluid intake, while urea nitrogen (24 h), postsurgical neurological events, nitric oxide, tracheobronchomalacia, pulmonary hypertensive events and cardiac reoperations were identified as determinants of LMV. Conclusions: Causes of MV after surgery are heterogeneous, vary with time, and have variable impact on the duration of MV. more...

The results of surgical intervention are reported in 10 cases of critical valvular aorta stenosis diagnosed with echocardiography in neonatal age and infancy. The significance of echocardiography (2-dimensional, M-mode, Doppler) which does not mean stress for the patient in critical state and provides correct diagnosis is emphasized. Correct diagnosis contributes to the performance of operation in appropriate time as well as the use of immediately performable and correct surgical technique is considered necessary. These are the first cases in Hungary where open heart operations have been carried out on the basis of echocardiography in neonatal age and infancy. The good results proved the adequacy of the solution. more...

Thrombotic lesions on the leaflets of the tricuspid valve in a premature infant with an anatomically normal heart are described. The diagnosis was made by two-dimensional and Doppler echocardiography. No etiologic explanation could be obtained from the history and clinical findings. The newborn had no infection, no antithrombin deficiency, and no indwelling catheter through either a peripheral, central or umbilical vein. The multiple masses were successfully removed by surgery under deep hypothermic circulatory arrest at 1 month of age. The child is well, without neurological deficit, and is developing normally. more...

A total of 417 individuals were examined by the Strauss-Albertini method, which is useful to detect the lack of hypoxanthin-guanine-phosphoribosil-transferase (HPRT) located on the X-chromosome. The damage of this locus leads to the loss of the sensitivity of the T-lymphocytes to 6-thioguanine (TG) therefore in the presence of this antimetabolite (TG) the cells will respond to the lectin's (phytohaemagglitinine: PHA) growth stimulatory effect. This defect in the cells can cause mutation, making the cells to be resistant against TG-treatment and during their blastogenesis they will be able to incorporate 3 (H)-thymidine. The number of these labeled cells will give the frequency of the mutant (TG-resistant) cells. The results showed a significant elevation in the number of TG-resistant lymphocytes among uranium miners and among industrial workers exposed to different mutagenic or carcinogenic chemicals when compared to the negative controls. The mutation frequency was elevated also among primary lung cancer patients (positive controls) up to the level measured among industrial workers. more...