Surgery For Congenital Heart Defects Research Articles

Factors determining outcomes in grown up patients operated for congenital heart diseases.

Background:The number of grown ups with congenital heart diseases (GUCHs) is steadily increasing.Aims:To analyze factors predicting early cardiac morbidity following cardiac surgery in GUCH at a tertiary care center.Setting and Design:Retrospective study at a multispeciality tertiary referral center.Methods:Between January 2004 and December 2014, 1432 patients ≥13 years of age (acyanotic defects: 843, cyanotic defects: 589) underwent surgery for congenital heart defects. Factors associated with early cardiac morbidity were analyzed.Statistical Analysis:Univariable and multivariable analysis of all factors affecting outcomes.Results:On multivariate analysis, previous sternotomy, aortic cross-clamp time >45 min, cyanosis, and emergency procedure were independent predictors of early morbidity with respective odds ratios (ORs) of 12.4, 3.6, 2.6, and 8.1. For more precise estimation, a risk score was generated. Taking the log odds with each of these four as respective weights, a score was generated. The variables were previous sternotomy (2.5), aortic cross-clamp >45 min (1.3), emergency procedure (2.1), and cyanosis (0.9), if the respective condition is present, zero otherwise. The score ranged from 0 to 4.5. The average value of the score based on the four variables was significantly higher in cases with morbidity (1.85 ± 1.17) vs. (0.75 ± 0.88), P < 0.001. Distribution of scores was significantly different between patients with and without morbidity. Sixty-seven percent patients without any morbidity had score <1 compared to 24.6% with morbidity. Only 0.9% patients without morbidity had score of ≥3 compared to 16.4% patients with morbidity. Compared with patients having score <1, patients with scores 1-2 had OR of 3.4, 2-3 had OR of 6.0, and >3 had OR of 48.7.Conclusion:GUCH can be safely operated when adequate caution is taken in the presence of independent predictors such as previous sternotomy, aortic clamp time >45 min, cyanosis, and emergency procedure.

Presence of Aggregatibacter actinomycetemcomitans in saliva and cardiac tissue samples of children with congenital heart disease.

The purpose of this study was to analyze the presence of Aggregatibacter actinomycetemcomitans in saliva and cardiac tissue samples of children requiring cardiac surgery in Istanbul, Turkey. Twenty-five patients (mean age: 6.24 ± 2.93) undergoing surgery for congenital heart defects (CHDs) and an age/gender-matched control group of 25 healthy children were enrolled in the study. Saliva samples were collected from all children; plaque index (PI) and gingival index (GI) were also determined. In CHD group, cardiac tissue samples were received during surgery. All samples were evaluated for the presence of A. actinomycetemcomitans and its highly leukotoxic JP2 clonal strains using polymerase chain reaction. The findings were analyzed by Mann-Whitney U, Chi-square, and Fisher's exact tests. No significant differences were found in PI and GI values between the groups. A. actinomycetemcomitans was not detected in cardiac tissue samples. A. actinomycetemcomitans in saliva was detected in 2 (8%) of the CHD and 5 (20%) of the control children (p > 0.05). A. actinomycetemcomitans JP2 clonal strains were determined from 1 (4%) of the control group while it was not determined from the samples of the CHD group. Early colonization of A. actinomycetemcomitans in oral cavities could be assessed as a risk marker for periodontal disease. Periodontal pathogens may enter bloodstream through bacteremia; thus, the presence of periodontal pathogens in the oral cavity of children should be assessed as a risk marker for cardiac diseases in older ages.

Abstract 18284: Incidence of Epilepsy is Elevated in Children and Young Adults With Congenital Heart Defects Compared With the General Population: A Nationwide Cohort Study

Perioperative seizures related to surgery for congenital heart defects (CHD) are well described; however, few data exist on the long-term risk of epilepsy in patients with CHD. We aimed to estimate the incidence of epilepsy in children and young adults with CHD compared with the general population. Methods: Utilizing data from the Danish National Registry of Patients (DNRP) we identified all patients diagnosed with CHD before the age of 15 years between 1980 and 2010 who were born during the same period. The DNRP is a nationwide hospital discharge registry covering all Danish hospitals. Previously validated methodology using the DNRP was applied to measure the outcome, epilepsy, as well as presence of extra cardiac defects (ECD) and/or syndromes. We used the Danish Medical Birth Registry to identify preterm birth (gestational age&lt;37 weeks). For each CHD subject, we identified 10 controls from the general population using the Danish Civil Registration System, matched by sex and birth year. A unique personal identifier assigned at birth and used in all Danish public registries enabled virtually complete follow up for migration, death, or epilepsy until January 1, 2013. We computed cumulative incidences and hazard ratios (HR) (split at 5 years of age to obtain proportional hazards) of time from CHD diagnosis (index date for controls) to epilepsy. Results: We identified 14,665 CHD subjects with a median age at diagnosis of 2 (IQR 19) months. By 15 years of age, the cumulative incidence of epilepsy was 4% among CHD subjects. The HR of epilepsy among CHD subjects compared with the control cohort was 3.7 (95% CI: 3.2-4.3) below 5 years of age, and 2.4 (95% CI: 2.1-2.7) from 5 to 33 years of age. In the older age group, HR for patients with severe CHD was 2.8 (95% CI: 2.3-3.5), and for mild and moderate CHD was 2.2 (95% CI: 1.8-2.6). After exclusion of all subjects with ECDs and/or syndromes and preterm birth, corresponding HRs were 2.2 (95% CI: 1.6-3.0) and 1.7 (95% CI: 1.3-2.2), respectively. Conclusion: The epilepsy risk was markedly increased in CHD subjects compared with the age and gender matched controls. These findings add evidence to support the importance of developing neuro-protective measures and potentially long-term epilepsy surveillance strategies in the CHD population.

Effect of 22q11.2 deletion on bleeding and transfusion utilization in children with congenital heart disease undergoing cardiac surgery.

Postsurgical bleeding causes significant morbidity and mortality in children undergoing surgery for congenital heart defects (CHD). 22q11.2 deletion syndrome (DS) is the second most common genetic risk factor for CHD. The deleted segment of chromosome 22q11.2 encompasses the gene encoding glycoprotein (GP) Ibβ, which is required for expression of the GPIb-V-IX complex on the platelet surface, where it functions as the receptor for von Willebrand factor (VWF). Binding of GPIb-V-IX to VWF is important for platelets to initiate hemostasis. It is not known whether hemizygosity for the gene encoding GPIbβ increases the risk for bleeding following cardiac surgery for patients with 22q11.2 DS. We performed a case-control study of 91 pediatric patients who underwent cardiac surgery with cardiopulmonary bypass from 2004 to 2012 at Children's Hospital of Wisconsin. Patients with 22q11.2 DS had larger platelets and lower platelet counts, bled more excessively, and received more transfusion support with packed red blood cells in the early postoperative period relative to control patients. Presurgical genetic testing for 22q11.2 DS may help to identify a subset of pediatric cardiac surgery patients who are at increased risk for excessive bleeding and who may require more transfusion support in the postoperative period.

Relationship of thymic changes and complications after congenital heart surgery.

The true role of thymic function in children with congenital heart defects is largely unknown. To study the possible role of thymic CD3 (T-lymphocyte marker) and CD20 (B-lymphocyte marker) expression and thymic histopathological changes in complications after surgery for congenital heart defects. Between January and July 2014, thymic tissue samples were obtained from 13 (69% male, 31% female, mean age 10.9 ± 2 years) of 25 Iraqi patients who underwent open heart surgery with partial thymectomy for correction of congenital heart defects. The samples were evaluated for thymic expression of CD markers (CD3 and CD20) and histopathological changes. For up to 6 months after surgery, data on complications were collected from each patient, including wound infection, cardiac arrhythmias, heart failure, rehospitalization, chest infection, and death. The prevalence of thymic hypoplasia was 15% and it occurred more frequently in patients with absent thymic CD3 expression (p = 0.005). Only wound infection (n = 2) and atrial fibrillation (n = 1) comprised the postoperative complications in our patients. Absence of thymic CD20 expression correlated significantly with postoperative complications and obstructive cardiac defects (p = 0.04), whereas no significant correlations were found between thymic hypoplasia with CD3 expression and postoperative complications (p > 0.05). Patients with absent thymic CD20 expression had significantly more postoperative complications and cardiac obstructive defects than those with positive CD20 expression.

Cardiopulmonary Bypass Increases Plasma Glial Fibrillary Acidic Protein Only in First Stage Palliation of Hypoplastic Left Heart Syndrome

BackgroundUniventricular congenital heart defects require open-heart surgery soon after birth, and are associated with risk of brain injury and poor neurologic outcome. MethodsThis is a prospective, observational study on children undergoing cardiac surgery. Plasma glial fibrillary acidic protein (GFAP), as an early marker of brain injury, was measured by ELISA at the end of anaesthesia induction, initiation of cardiopulmonary bypass (CPB), the end of cooling, the end of rewarming, the end of CPB, and after protamine administration. We recorded clinical and surgical parameters to assess which CPB phase and clinical parameters were associated with a GFAP increase. ResultsWe studied 13 children less than 50 months of age: 8 underwent Norwood or Damus-Kaye-Stansel palliation (group 1) and 5 underwent Fontan procedure (group 2). A GFAP increase was only observed in group 1, with the highest median value at the end of rewarming. No quantifiable levels of GFAP were measured at pre-bypass and the start of CPB stages in all patients. End of cooling and CPB-end GFAP, GFAP maximum value, and GFAP area under the curve all correlated with the CPB time spent at a cerebral regional saturation < 45% (P = 0.021, 0.028, 0.007, 0.021, respectively). ConclusionsChildren with univentricular heart defects exhibit a CPB plasma-GFAP increase only after stage 1 palliation. The maximum GFAP increase occurred at the end of rewarming. Further studies are needed to identify which clinical or surgical parameter(s) could reflect a GFAP increase during surgery for congenital heart defects, and whether GFAP levels correlate with the neurologic outcome.

Epidemiology and outcome of Congolese children who had surgery for heart defects

to determine the principal heart defects for which children underwent surgery and to determine the survival rate. this retrospective cohort study involves Congolese babies treated surgically from September 1989 to September 2010 in France for congenital heart defects (through "Mécénat chirurgie cardiaque" and "Chaîne de l'espoir"). It includes only 110 of the 182 recorded patients during the study period. The sex ratio for the 110 subjects included in the analysis was 1. Their mean age at surgery was 77.4 ± 57.6 months old (range: 8 to 204 months). The main congenital heart defects for which surgery was performed were ventricular septal defect (21.9%), tetralogy of Fallot either isolated (22.8%) or associated with patent foramen ovale (1.8%) or coronary anomalies (1.8%), atrial septal defect associated with other malformations (8.2%), pulmonary atresia with ventricular septal defect (5.5%), aortic stenosis (3.7%), atrioventricular septal defect (0.9%), and Laubry-Pezzi syndrome (0.9%). The median length of follow-up was 42.4 ± 35.6 months (range, 3-240 months). Patients' mean age at the study's end was 121.1 ± 86.3 months (range 20-372 months). The 5-year survival rate was 90% and the 20-year survival, 83.3%. Heart surgery for congenital heart defects has improved survival.

Achievements in Congenital Heart Defect Surgery

Background— This article presents an update of the results achieved by modern surgery in congenital heart defects (CHDs) over the past 40 years regarding survival and the need for reoperations, especially focusing on the results from the past 2 decades. Methods and Results— From 1971 to 2011, all 7038 patients &lt;16 years of age undergoing surgical treatment for CHD at Rikshospitalet (Oslo, Norway) were enrolled prospectively. CHD diagnosis, date, and type of all operations were recorded, as was all-cause mortality until December 31, 2012. CHDs were classified as simple (3751/7038=53.2%), complex (2918/7038=41.5%), or miscellaneous (369/7037=5.2%). Parallel to a marked, sequential increase in operations for complex defects, median age at first operation decreased from 1.6 years in 1971 to 1979 to 0.19 years in 2000 to 2011. In total, 1033 died before January 1, 2013. Cumulative survival until 16 years of age in complex CHD operated on in 1971 to 1989 versus 1990 to 2011 was 62.4% versus 86.9% ( P &lt;0.0001). In the comparison of patients operated on in 2000 to 2004 versus 2005 to 2011, 1-year survival was 90.7% versus 96.5% ( P =0.003), and 5-year cumulative survival was 88.8% versus 95.0% ( P =0.0003). In simple versus complex defects, 434 (11.6%) versus 985 (33.8%) patients needed at least 1 reoperation before 16 years of age. In complex defects, 5-year cumulative freedom of reoperation among patients operated on in 1990 to 1999 versus 2000 to 2011 was 66% versus 73% ( P =0.0001). Conclusions— Highly significant, sequential improvements in survival and reductions in reoperations after CHD surgery were seen. A future challenge is to find methods to reduce the need for reoperations and further reduce long-term mortality.

Grown up congenital hearts.

The exploits and successes of cardiac surgery for congenital heart defects over six decades has created a new population of cardiac patients – the Grown Up Congenital Hearts (GUCHs) which has many names in different countries. From success-ful cardiac surgery comes the refinement of other specialties – paediatric cardiology, anaesthesia, intensive care, sophis-ticated imaging, who looks after the GUCHs, and where are the optimal medical services for the adults (adolescents) with congenital heart diseases. At least 90% of children born with such defects can be expected to reach adulthood in regions/countries or areas where there has been established good paediatric cardiac surgery. Indeed most lesions now can be treated with success and survival. The notion of “total cor-rection” should be dispelled as a dream of surgeons for many years. There are conditions such as closure of the persistent duct, certain ventricular septal defects and a few other simple defects which can be considered as cured of the need for future medical care. Most long-term survivors with congenital heart disease, particularly the complex, need expert medical care for life. Thus their needs must be recognised with provision of funded and staffed specialised units with necessary expertise, taking on the role of education of the problem in the region. Not every region needs a unit but with support services, research and provision of the various levels of needs of the patients.Numbers of units depend on the size and geographical features of the country or area as well as the size of the GUCH population. Too many units lead to loss of expertise and ed-ucation by diluting numbers. No one in any country knows the true numbers of GUCH patients. Predictions are not true anywhere. John Keith in the late 1950s recognised the need to have a service as his children survived, and so sent John Evans to the Toronto General Hospital to establish such a service, separate from paediatric cardiology in the Children’s Hospital. So wise. In actual fact not much was established and the concerned doctor left to be a business magnate. Joe Perloff, influenced deeply by Paul Wood in his London training, fascinated by congenital heart disease, recognised GUCH needs in Los Angeles, established with difficult a service as did the National Heart Hospital in London with the opening of an adolescent cardiac unit in 1975 receiving the patients from Dr Richard Bonham-Carter, operated by Dr David Waterston in Hospital for Sick Children (Great Ormond Street). Services grew into Grown Up Congenital Heart unit – called after Paul Wood and rebuilt following the destruction when the Heart Hospital merged into the Brompton Hospital. Establishing such a unit has always been difficult for different reasons in different countries and remains so. It was facilitated by having a funded Health Service. It was easy in Malta because the principal doctors wanted it. It remains diffi-cult in the USA where provision is only in a few isolated units. Recognition even that there is this sub specialty of cardiology has been nearly impossible in many countries; even when they have good paediatric cardiac services or because they have, has helped GUCHs since the paediatric cardiologists frequently reserve a parental right to continue looking after their patients into adulthood. It cannot be imagined that paediatricians would consider that an adult trained consultant could have a right to care for an infant. Most would not wish to do so in any spe-cialty, so why should the paediatricians consider their ability (right) to care for adults, because they know the anatomy. In Canada, Japan and a number of European countries including the United Kingdom, it has been recognised that there needs to be a national service controlled and funded for the specialty of Grown Ups with congenital heart abnormalities as part of adult cardiology which is useful as many will acquire the cardiovascular diseases of the population.

Validation of association of the apolipoprotein E ε2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants

Apolipoprotein E (APOE) genotype is a determinant of neurologic recovery after brain ischemia and traumatic brain injury. The APOE ε2 allele has been associated with worse neurodevelopmental (ND) outcome after repair of congenital heart defects (CHD) in infancy. Replication of this finding in an independent cohort is essential to validate the observed genotype-phenotype association. The association of APOE genotype with ND outcomes was assessed in a combined cohort of patients with single-ventricle CHD enrolled in the Single Ventricle Reconstruction and Infant Single Ventricle trials. ND outcome was assessed at 14 months using the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. Stepwise multivariable regression was performed to develop predictive models for PDI and MDI scores. Complete data were available for 298 of 435 patients. After adjustment for preoperative and postoperative covariates, the APOE ε2 allele was associated with a lower PDI score (P = .038). Patients with the ε2 allele had a PDI score approximately 6 points lower than those without the risk allele, explaining 1.04% of overall PDI variance, because the ε2 allele was present in only 11% of the patients. There was a marginal effect of the ε2 allele on MDI scores (P = .058). These data validate the association of the APOE ε2 allele with adverse early ND outcomes after cardiac surgery in infants, independent of patient and operative factors. Genetic variants that decrease neuroresilience and impair neuronal repair after brain injury are important risk factors for ND dysfunction after surgery for CHD.

Health related quality of life after corrective surgery for congenital heart disease.

After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. A standardised questionnaire deve-loped for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. 173 patients (40% female, 60% male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8±3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n=50, Tetralogy of Fallot n=51, univentricular heart n=26, transposition of great arteries n=40). The results were compared with those of an age-matched control group (n=169). Patients with CHD reported a better HRQoL than the controls for all items (p<0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of "body image", had become very similar compared to controls. Pa-rents assessed their children significantly worse in 3 domains (friends, body image and emotion; p<0.01). Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better.

ABSTRACT 564

Background and aims: Children undergoing open heart surgery for congenital heart defect develop some form of neuro developmental impairment post surgery. The degree of impairment depends on the cardiopulmonary bypass factors. In such a scenario, is open heart surgery for congenital heart disease justified in infants with pre existing severe neurological injury? We report our experience in a series of 5 cases. Aims: To see the outcome of childrem with preexisting severe brain damage undergoing cardiac surgeries. Methods: Retrospective single unit study of 5 children undergoing cardiac surgery in the presence of severe pre existing brain damage in the last 24 months. The outcome of these children was noted based on worsening of existing brain damage. Results: All 5 cases underwent successful cardiac surgeries (4 on Cardiopulmonary bypass and one closed heart). Post surgery they all had a long hospital stay and there was no further deterioration in the neurological status. One patient required anticoagulation for metallic valve. Conclusions: Open heart surgery in a child with pre existing neurological injury has risk of aggravation of pre existing neurological injury. Children with pre existing severe neurological injury can undergo cardiac surgery. The decision for such a step must be multidisciplinary and according to the merit of each patient.

Dynamics of Factor XIII Levels After Open Heart Surgery for Congenital Heart Defects: Do Cyanotic and Acyanotic Patients Differ?

Cardiopulmonary bypass (CPB) reduces coagulation factor levels through hemodilution and consumption. Differences in CPB-induced alterations of factor XIII (FXIII) levels in children with cyanotic and acyanotic congenital heart defects (CHDs) are not well characterized. FXIII activity (determined by Berichrom assay), prothrombin index, activated partial thromboplastin time, and fibrinogen were measured before open heart surgery with CPB and 5 days postoperatively for children older than 3 years with acyanotic (n = 30) and cyanotic (n = 30) CHDs. The preoperative FXIII levels did not differ significantly among the children of the compared groups. The cyanotic patients showed a significantly longer duration of CPB (111.4 ± 45.8 vs 71.5 ± 33.6 min; p = 0.026) and aortic cross-clamp (68.0 ± 27.1 vs 45.4 ± 31.4 min; p = 0.034). The drop in FXIII levels after termination of CPB was more profound for the children with cyanotic CHDs (87.1 ± 13.4 to 49.1 ± 13.2 vs 81.5 ± 12.6 to 58.6 ± 11.1 %, respectively; p = 0.018). The cyanotc patients also were restored to their baseline FXIII levels later than the children with acyanotic CHDs (at 48 vs 24 h). The post-CPB dynamics of the majority of the other coagulation parameters in the compared groups of patients were similar. The cyanotic patients experienced significantly greater postoperative blood loss than the acyanotic patients (12.6 ± 4.9 vs 5.0 ± 2.1 mL/kg; p < 0.001) and were transfused with larger volumes of red blood cells (10.4 ± 6.5 vs 4.2 ± 2.5 mL/kg; p = 0.007). The decrease in FXIII levels after CPB is more profound and lasts longer in children with cyanotic CHDs than in acyanotic patients. The rational strategy of postoperative FXIII replacement therapy for these categories of patients needs to be determined.

Pediatric Cardiothoracic Surgery in Patients With Unilateral Pulmonary Agenesis or Aplasia

Unilateral pulmonary agenesis or aplasia (UPA), a rare developmental defect of the lung, is sometimes associated with congenital heart and tracheal diseases. The purpose of this study was to assess our experience of pediatric cardiothoracic surgery in UPA patients. Cardiothoracic surgery for congenital heart defect or tracheal stenosis performed between 1981 and 2010 in 8 UPA patients (agenesis in 5 and aplasia in 3) was reviewed retrospectively. Associated cardiac anomalies included ventricular septal defect, double outlet right ventricle with pulmonary atresia, total anomalous pulmonary venous connection, and interrupted aortic arch complex. For 7 patients with right UPA and 1 patient with left UPA, 12 cardiothoracic operations were performed, including 8 cardiac procedures in 4 patients and 4 tracheal procedures in 4 patients. Cardiac palliative repairs included Blalock-Park anastomosis, systemic-to-pulmonary artery shunt, and pulmonary artery banding. Cardiac definitive repairs included ventricular septal defect closure, subaortic membrane resection, modified Konno procedure, total anomalous pulmonary venous connection repair, and Rastelli-type operation. Tracheal repairs were costal cartilage tracheoplasty and slide tracheoplasty. The median age at surgery was 8 months and median body weight was 6.2 kg; the median operative time was 6.5 hours. There were 3 hospital deaths and 1 late death, with the 1-year mortality rate of 25%. Cardiopulmonary bypass-induced acute lung injury has occurred in 3 cases, 2 of which required extracorporeal membrane oxygenation support. Younger age of less than 1 month and prolonged cardiopulmonary bypass time of more than 200 minutes were related to operative risk factors for hospital mortality and morbidity. Most of the pediatric cardiothoracic operations in UPA patients were successfully performed through an optimal surgical approach and procedure, but they still presented surgical risks of high mortality and morbidity. Perioperative management of UPA patients should be provided with a precise understanding of anatomic configuration and a careful consideration of underlying risk factors.

Blood transfusion during versus after cardiopulmonary bypass is associated with postoperative morbidity in neonates undergoing cardiac surgery

Cardiac surgery on neonates for the correction of congenital heart defects is usually associated with the transfusion of packed red blood cells (PRBCs) into the cardiopulmonary bypass (CPB) circuit. We hypothesised that such transfusions of stored PRBCs directly into the arterial system may increase postoperative morbidity when compared to intravenous transfusion. In this retrospective cohort study, data from 122 consecutive neonates who received transfusions of PRBCs in the course of corrective surgery for congenital heart defects were analysed. Group assignment was according to the timing of the first transfusion: during CPB (On-CPB) or after weaning from CPB (Post-CPB). Chi Square and rank sum tests were applied to compare clinical characteristics. Times until extubation and release from the intensive care unit were analysed by Kaplan-Meier curves and by multivariate Cox regression. Transfusion of PRBCs during CPB was associated with greater 48 hour blood loss (mean±standard deviation, 86±125 versus 32±16 mL/kg, p<0.001), longer duration of mechanical ventilation (214±268 versus 99±75 h, p<0.001) and longer stay on the intensive care unit (10.9±12.1 versus 5.3±3.5 days, p<0.001). However, the groups also differed in many characteristics, such as bodyweight, complexity of surgery or preoperative haemoglobin concentration, which may also affect outcome. Yet, multivariate analyses confirmed an independent association of transfusion On-CPB with an adverse clinical outcome. Our results are consistent with the hypothesis that the transfusion of PRBCs during CPB may increase postoperative morbidity. However, due to the limitations of this retrospective analysis, further studies are needed to confirm a causal relationship between the timing of the transfusion and the clinical outcome and to elucidate putative mechanisms of such an association.

EPA-0306 – Psychomotor development of infants with congenital heart disease after cardiopulmonary bypass surgery

The decreased cerebral blood supply and hypoxia can impair the brain development of children with congenital heart defects (CHD) in utero. We explored the impact of cardiopulmonary bypass (CPB) surgery on neurodevelopment outcomes in infants with CHD. To assess psychomotor development of infants that underwent CPB surgery for CHD using the autologous placental umbilical cord blood (APUCB) in the first hours of life. To prevent psychomotor retardation in infants after CPB surgery and implement early psycho-diagnosis and psycho-correction measures. We analyzed a database of children enrolled in prospective studies on surgical support techniques from 2010 to 2012. We used the Bayley Scales of Infant Development (BSID-II) to study the cognitive and motor development. The study involved 90 infants of 1 to 2.5 years of age with CHD. Study group included 28 infants that underwent surgery with CPB and APUCB, conventional group included 62 infants that underwent surgery with CPB and donor blood. Although the aggregate group of infants after CPB surgery displayed the mental retardation and/or psychomotor development in 40% of infants the average mental (MDI) and psychomotor (PDI) indices were within normal age limits in study and conventional group of infants respectively. The study group tended to show higher MDI 94.5±15.8 and PDI 91.4±15.3 compared with conventional group MDI 90.3±13.4 and PDI 86.3±14.5. The considerable number of infants with cognitive retardation implies the development of early psychosocial programme of assistance to patients and the optimization of surgery using APUCB instead of donor blood.

Relation of Prenatal Diagnosis With One-Year Survival Rate for Infants With Congenital Heart Disease

Prenatal diagnosis of congenital heart defects (CHDs) is increasingly common, but it is still unclear whether it translates to improved postoperative outcomes. We performed a retrospective cohort study of all infants (aged <1 year) who underwent surgery for CHDs from 2006 to 2011 at a single institution. Primary outcomes were in-hospital and 1-year mortality rates. Secondary outcomes were readmission within 30 days of discharge, postoperative length of intensive care unit and hospital stay, unplanned reoperation, and extracorporeal membrane oxygenation use. We used chi-square analyses, Wilcoxon rank-sum tests, Kaplan-Meier survival curves, and adjusted Cox proportional hazards models to compare outcomes. Of the 1,642 patients with CHDs, 539 (33%) were diagnosed prenatally. Patients with prenatal diagnoses were of a younger age and less weight at the time of surgery, had greater Risk Adjustment for Congenital Heart Surgery scores, and were more likely to be white, to have an identified syndrome, or to be born at term. Compared with those diagnosed postnatally, those diagnosed prenatally had a significantly higher unadjusted 1-year mortality rate (11% vs 5.5%, respectively, p = 0.03). Controlling for weight, surgical severity, race, age at surgery, prematurity, and the presence or absence of genetic syndrome, patients with prenatal diagnoses had significantly greater mortality at 1 year (adjusted hazard ratio 1.5, p = 0.03), as well as significantly longer intensive care unit and hospital stays. Infants with CHDs diagnosed prenatally had worse outcomes compared with those diagnosed postnatally. Prenatal diagnosis likely captures patients with more severe phenotypes within given surgical risk categories and even within diagnoses and thus may be an important prognostic factor when counseling families.

Prevalence of Salivary Streptococcus mutans Serotype k in Children Undergoing Congenital Heart Surgery

The prevalence of Streptococcus mutans serotype k, which was speculated that might be associated with the development of cardiovascular diseases, has been reported in adult cardiovascular surgery patients. There is no information about presence of serotype k in children with cardiac disease. The aim of this study was to determine the salivary prevalence of S. mutans serotype k in children with congenital heart disease. Salivary samples of 25 patients undergoing elective surgery for congenital heart defects with cardiopulmonary bypass and an age and gender matched control group of 25 healthy children were enrolled in the study. Species-specific 16SrRNA gene sequences were used for S. mutans and serotype-specific rgpF gene sequences were used for S. mutans serotype k determination in stimulated saliva samples. S. mutans was detected in 19 (76%) of the study and 15 (60%) of the control children. The difference was not shown to be statistically significant. Serotype k was determined from 3 (12%) of the study group, while it was not determined from the samples of the control group. Our results indicate that those children with congenital heart disease may possess S. mutans serotype k in oral cavity at a higher frequency as similar with the adult cardiac surgery patients.

Update: Advances in Pediatric Cardiac Intensive Care

Update: Advances in Pediatric Cardiac Intensive Care

Ventricular Performance After Surgery for a Congenital Heart Defect as Assessed Using Advanced Echocardiography: From Doppler Flow to 3D Echocardiography and Speckle-Tracking Strain Imaging

A varying degree of impairment of ventricular performance is observed over the long-term after surgery for a congenital heart defect (CHD). Impaired ventricular performance has been shown to be of prognostic value for increased risk of cardiovascular events in adult CHD patients. This emphasizes the importance of delineating the timing and cause of this postoperative impairment. Impairment of ventricular performance could develop over time as a consequence of residua, sequelae and complications of the CHD or surgical procedure. Yet, impaired ventricular performance has also been observed immediately after surgery and can persist and/or worsen over time. This postoperative impairment of ventricular performance is the focus of this review. This article provides an overview of echocardiographic techniques currently used to assess ventricular performance. Furthermore, we review current literature describing ventricular performance, as assessed using echocardiography, after correction of a CHD. In general, a decrease in ventricular performance is observed directly after surgery for CHD’s. Subsequent follow-up of ventricular performance is characterized by a varying degree of postoperative recovery. A consistent observation is the persistent impairment of right-ventricular performance after repair in several different subgroups of CHD patients ranging from ventricular septal defect repair to surgery for Tetralogy of Fallot.