School age and adolescent heart failure following the Norwood procedure.

BACKGROUND: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. METHODS: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). RESULTS: Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. CONCLUSIONS: Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age.

Factors Associated With Attendance for Cardiac Neurodevelopmental Evaluation.

BACKGROUND AND OBJECTIVES: Neurodevelopmental evaluation of toddlers with complex congenital heart disease is recommended but reported frequency is low. Data on barriers to attending neurodevelopmental follow-up are limited. This study aims to estimate the attendance rate for a toddler neurodevelopmental evaluation in a contemporary multicenter cohort and to assess patient and center level factors associated with attending this evaluation. METHODS: This is a retrospective cohort study of children born between September 2017 and September 2018 who underwent cardiopulmonary bypass in their first year of life at a center contributing data to the Cardiac Neurodevelopmental Outcome Collaborative and Pediatric Cardiac Critical Care Consortium clinical registries. The primary outcome was attendance for a neurodevelopmental evaluation between 11 and 30 months of age. Sociodemographic and medical characteristics and center factors specific to neurodevelopmental program design were considered as predictors for attendance. RESULTS: Among 2385 patients eligible from 16 cardiac centers, the attendance rate was 29.0% (692 of 2385), with a range of 7.8% to 54.3% across individual centers. In multivariable logistic regression models, hospital-initiated (versus family-initiated) scheduling for neurodevelopmental evaluation had the largest odds ratio in predicting attendance (odds ratio = 4.24, 95% confidence interval, 2.74-6.55). Other predictors of attendance included antenatal diagnosis, absence of Trisomy 21, higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category, longer postoperative length of stay, private insurance, and residing a shorter distance from the hospital. CONCLUSIONS: Attendance rates reflect some improvement but remain low. Changes to program infrastructure and design and minimizing barriers affecting access to care are essential components for improving neurodevelopmental care and outcomes for children with congenital heart disease.

Quality of life after Ross procedure in children.

INTRODUCTION: Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD. METHOD: In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined. RESULTS: 68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively. CONCLUSION: Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.

Impact of tight glycemic control and hypoglycemia after pediatric cardiac surgery on neurodevelopmental outcomes at three years of age: Findings from a randomized clinical trial.

BACKGROUND: Studies examining the impact of randomization As per standard instruction, city is required for affiliations; however, this information is missing in affiliation 6. Please check if the provided city is correct and amend if necessary. to tight glycemic control (TGC) and resultant hypoglycemia on later neurodevelopmental outcomes have produced mixed results. Our study examined this association in children undergoing cardiac surgery. METHODS: Participants who were enrolled in the Safe Pediatric Euglycemia after Cardiac Surgery (SPECS) trial returned for neurodevelopmental (ND) follow-up between 30 to 42.5 months of age. ND outcomes were assessed using the Bayley Scales of Infant and Toddler Development, Third Edition. ND scores were compared between the TGC and standard care treatment groups and between patients with moderate to severe and no to mild hypoglycemia. As a secondary analysis, to increase sample size and power, we combined the three-year-old assessments with previously collected assessments done at < 30 months of age to further examine differences between groups longitudinally. RESULTS: Among the 269 participants who completed neurodevelopmental evaluation (in-person testing or questionnaires) at three years of age (follow-up rate, 31%), there were no statistically significant differences in ND outcomes according to treatment group or hypoglycemia status. In the combined analysis of all evaluations (from 9 to 42.5 months of age), we found no treatment group differences. However, in these longitudinal analyses, children who experienced moderate to severe hypoglycemia had lower scores on the Bayley-III cognitive and motor domains compared to children with no to mild hypoglycemia. CONCLUSIONS: For infants undergoing cardiac surgery, there was no impact of tight glycemic control on neurodevelopmental outcomes. Moderate to severe hypoglycemia was associated with worse ND outcomes in longitudinal analyses. TRIAL REGISTRATION: ClinicalTrials.gov NCT00443599. Registered: November 2016.

Utilisation of early intervention services in infants with congenital heart disease following open-heart surgery.

OBJECTIVE: To examine the use of early intervention services in infants with CHD after open-heart surgery and identify factors associated with receipt of services. STUDY DESIGN: Surveys were administered to caregivers of infants who underwent open-heart surgery before 1 year of age at a single institution between July, 2017 and July, 2018. Information regarding the infant's use of early intervention services and the caregiver's experience with the programme was obtained. Clinical data were retrieved from the medical record review. Logistic regression identified factors associated with receipt of services. RESULTS: The study included 158 eligible infants. Ninety-eight caregivers (62%) completed the surveys. Of those surveyed, 53.1% of infants were currently or previously enrolled in early intervention services. Infants most frequently received physical therapy (76.9%). The majority of caregivers found services to be moderately/very helpful (92.3%) and sufficient for their child (76.9%). In the univariate analysis, single-ventricle disease, known syndrome/genetic abnormality, extracardiac anomaly, and longer intensive care and hospital length of stay were associated with receipt of services. Single-ventricle disease (p = 0.004) and known syndrome/genetic abnormality (p < 0.0001) remained independently associated with receipt of services in the multivariable analysis. CONCLUSION: Amongst infants at risk for neurodevelopmental deficits, approximately half received services after open-heart surgery. Caregivers expressed satisfaction with the programme. While infants with single-ventricle disease and a known syndrome/genetic abnormality were more likely to receive early intervention services, many at-risk infants with CHD failed to receive services. Further research is needed to identify barriers to early intervention services and promote developmental outcomes.

The Bayley-III scale may underestimate neurodevelopmental disability after cardiac surgery in infants.

OBJECTIVES: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II). We compared BSID-II and Bayley-III scores in infants undergoing cardiac surgery. METHODS: We evaluated 2198 infants who underwent operations with cardiopulmonary bypass between 1996 and 2009 at 26 institutions. We used propensity score matching to limit confounding by indication in a subset of patients (n = 705). RESULTS: Overall, unadjusted Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores (90.7 ± 17.2 vs 77.6 ± 18.8, P < 0.001), and unadjusted Bayley-III composite cognitive and language scores were higher than BSID-II Mental Development Index scores (92.0 ± 15.4 vs 88.2 ± 16.7, P < 0.001). In the propensity-matched analysis, Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores [absolute difference 14.1, 95% confidence interval (CI) 11.7-17.6; P < 0.001] and the Bayley-III classified fewer children as having severe [odds ratio (OR) 0.24; 95% CI 0.14-0.42] or mild-to-moderate impairment (OR 0.21; 95% CI 0.14-0.32). The composite of Bayley-III cognitive and language scores was higher than BSID-II Mental Development Index scores (absolute difference 4.0, 95% CI 1.4-6.7; P = 0.003), but there was no difference between Bayley editions in the proportion of children classified as having severe cognitive and language impairment. CONCLUSIONS: The Bayley-III yielded higher scores than the BSID-II and classified fewer children as severely impaired. The systematic bias towards higher scores with the Bayley-III precludes valid comparisons between early and contemporary cardiac surgery cohorts.

Transplant-Free Survival and Interventions at 6 Years in the SVR Trial.

BACKGROUND: In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. METHODS: Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. RESULTS: Transplant-free survival for the RVPAS versus modified Blalock-Taussig shunt groups did not differ at 6 years (64% versus 59%, P=0.25) or with all available follow-up of 7.1±1.6 years (log-rank P=0.13). The RVPAS versus modified Blalock-Taussig shunt treatment effect had nonproportional hazards (P=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48-0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86-2.17; P=0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33-1.74; P=0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year, P<0.001), primarily because of more interventions between the stage II and Fontan procedures (HR, 1.72; 95% confidence interval, 1.00-3.03). Complications did not differ by shunt type; by 6 years, 1 in 5 patients had had a thrombotic event, and 1 in 6 had had seizures. CONCLUSIONS: By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock-Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Prevalence and risk factors associated with non-attendance in neurodevelopmental follow-up clinic among infants with CHD.

BACKGROUND: Neurodevelopmental impairment is increasingly recognised as a potentially disabling outcome of CHD and formal evaluation is recommended for high-risk patients. However, data are lacking regarding the proportion of eligible children who actually receive neurodevelopmental evaluation, and barriers to follow-up are unclear. We examined the prevalence and risk factors associated with failure to attend neurodevelopmental follow-up clinic after infant cardiac surgery. METHODS: Survivors of infant (<1 year) cardiac surgery at our institution (4/2011-3/2014) were included. Socio-demographic and clinical characteristics were evaluated in neurodevelopmental clinic attendees and non-attendees in univariate and multivariable analyses. RESULTS: A total of 552 patients were included; median age at surgery was 2.4 months, 15% were premature, and 80% had moderate-severe CHD. Only 17% returned for neurodevelopmental evaluation, with a median age of 12.4 months. In univariate analysis, non-attendees were older at surgery, had lower surgical complexity, fewer non-cardiac anomalies, shorter hospital stay, and lived farther from the surgical center. Non-attendee families had lower income, and fewer were college graduates or had private insurance. In multivariable analysis, lack of private insurance remained independently associated with non-attendance (adjusted odds ratio 1.85, p=0.01), with a trend towards significance for distance from surgical center (adjusted odds ratio 2.86, p=0.054 for ⩾200 miles). CONCLUSIONS: The majority of infants with CHD at high risk for neurodevelopmental dysfunction evaluated in this study are not receiving important neurodevelopmental evaluation. Efforts to remove financial/insurance barriers, increase access to neurodevelopmental clinics, and better delineate other barriers to receipt of neurodevelopmental evaluation are needed.

Effect of Fontan operation on liver stiffness in children with single ventricle physiology.

OBJECTIVES: Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. METHODS: Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available. Changes in mean liver shear wave speed (SWS) were assessed using a mixed-effect model with post hoc Tukey correction. Changes in IVC pressure were evaluated using the Wilcoxon signed-rank test. A p value less than 0.05 was considered significant. RESULTS: Mean age at enrolment was 33.5 ± 10.5 months. Baseline mean liver SWS was normal at 1.18 ± 0.29 m/s, increased to 2.28 ± 0.31 m/s at 2.5 ± 1.2 days (p < 0.0001) and to 2.22 ± 0.38 m/s at 7.5 ± 1.4 days (p < 0.0001). Five subjects returned at a mean of 185 ± 28 days, and mean liver SWS remained elevated at 2.08 ± 0.24 m/s (p < 0.0001). Mean IVC pressure increased from 7.2 ± 2.6 mmHg at baseline to 16.44 ± 3.3 mmHg at 2.2 ± 0.8 days post-surgery (p = 0.004). CONCLUSION: The Fontan operation immediately and chronically increases liver stiffness and IVC pressure. Our study provides further evidence that congestion is a key driver of Fontan-associated liver disease. KEY POINTS: • The Fontan operation triggers immediate hepatic congestion and marked liver stiffening. • Congestion, not fibrosis, drives early increased liver stiffness in Fontan patients. • Hepatic congestion persists chronically for months after the Fontan operation. • Congestion confounds shear wave elastography as a post-Fontan liver fibrosis biomarker.

Severe Acute Kidney Injury Following Stage 1 Norwood Palliation: Effect on Outcomes and Risk of Severe Acute Kidney Injury at Subsequent Surgical Stages.

OBJECTIVES: To identify associations of severe acute kidney injury early after stage 1 (Norwood) operation with risk of severe acute kidney injury and comorbidities at subsequent palliative stages in patients with hypoplastic left heart syndrome and other single ventricle lesions with left-sided obstruction. DESIGN: Retrospective cohort study. Severe acute kidney injury defined as Kidney Disease Improving Global Outcomes stage 3. SETTING: Single pediatric cardiac center. PATIENTS: Infants less than or equal to 28 days old with single ventricle physiology and left-sided obstruction undergoing stage 1 operation between September 2007 and November 2012 (n = 136). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The occurrence rate of severe acute kidney injury was 21% (28/136) following stage 1, 12% (12/98) following stage 2 palliation (superior cavo-pulmonary anastomosis), and 10% (7/73) following stage 3 palliation (total cavo-pulmonary anastomosis). Severe acute kidney injury early after stage 1 operation was significantly associated with continuous intravenous loop diuretic infusion, need for extracorporeal membrane oxygenation, and in-hospital death (all p < 0.05). Gestational age at birth was associated with severe acute kidney injury at stage 2 (p = 0.04) and stage 3 (p = 0.01). Severe acute kidney injury at stage 1 was an independent risk factor for severe acute kidney injury at stage 2 (adjusted odds ratio, 4.3; 95% CI, 1.1-16.9; p = 0.04). Development of severe acute kidney injury after stage 1 was associated with longer mechanical ventilation time after stage 3 (p = 0.047). CONCLUSIONS: Severe acute kidney injury after stage 1 palliation was an independent risk factor for developing severe acute kidney injury at stage 2, and was associated with prolonged duration of mechanical ventilation following stage 3. Information on the incidence and associated risk factors for postoperative acute kidney injury in hypoplastic left heart syndrome patients from multiple congenital heart centers is a necessary next step to further understand the long-term burden of severe acute kidney injury after staged palliation.

Fluid Overload Is Associated With Late Poor Outcomes in Neonates Following Cardiac Surgery.

OBJECTIVES: Acute kidney injury is a severe complication of cardiac surgery associated with increased morbidity and mortality; yet, acute kidney injury classification for neonates remains challenging. We characterized patterns of postoperative fluid overload as a surrogate marker for acute kidney injury and as a risk factor of poor postoperative outcomes in neonates undergoing cardiac surgery. DESIGN: Retrospective cohort study. SETTING: Single, congenital heart center destination program. PATIENTS: Four hundred thirty-five neonates undergoing cardiac surgery with cardiopulmonary bypass from January 2006 through December 2010. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Demographics, diagnosis, and perioperative clinical variables were collected, including daily weights and serum creatinine levels. A composite poor clinical outcome (death, need for renal replacement therapy or extracorporeal life support within 30 postoperative days) was considered the primary outcome measure. Twenty-one neonates (5%) had a composite poor outcome with 7 (2%) requiring renal replacement therapy, 8 (2%) requiring extracorporeal life support, and 14 (3%) dying between 3 and 30 days post surgery. Neonates with a composite poor outcome had significantly higher maximum fluid overload (> 20%) and were slower to diurese. A receiver-operating characteristic curve determined that fluid overload greater than or equal to 16% and serum creatinine greater than or equal to 0.9 on postoperative day 3 were the optimal cutoffs for significant discrimination on the primary outcome (area under the curve = 0.71 and 0.76, respectively). In multivariable analysis, fluid overload greater than or equal to 16% (adjusted odds ratio = 3.7) and serum creatinine adjusted odds ratio 0.9 (adjusted odds ratio = 6.6) on postoperative day 3 remained an independent risk factor for poor outcome. Fluid overload greater than or equal 16% was also significantly associated with cardiac arrest requiring cardiopulmonary resuscitation, prolonged ICU stay, and chest reexploration. CONCLUSIONS: This study highlights the importance of monitoring fluid balance in the neonatal cardiac surgical population and suggests that daily fluid overload, a readily available, noninvasive marker of renal function, may be a sensitive and specific predictor of adverse outcomes.

Parental Preferences Regarding Outpatient Management of Children with Congenital Heart Disease.

The objective of this study was to identify patterns of health care usage among children with congenital heart disease (CHD) and determine predictors for contacting cardiologists for routine care. Parents of children with CHD completed surveys, indicating which provider they would contact first for 12 concerns. Predictors for preference for cardiologist evaluation were identified by multivariable logistic regression. Surveys were completed by 307 of 925 (33.2%) parents. Median patient age was 9.4 years [interquartile range (IQR) 5.1-14.4 years] with a median of 1 cardiac surgery (IQR 1-3). Most parents agreed primary care physicians (PCPs) could identify problems related to CHD (70.0%) and when to refer to cardiologists (89.6%). More felt PCPs best understood their values (63.2 vs. 29.6%, P < 0.001) and were more accessible (63.5 vs. 33.6%, P < 0.001) than cardiologists. Parents preferred first evaluation by PCPs for 9 of 12 concerns. Preference for cardiology evaluation was independently associated with the number of cardiac catheterizations [adjusted odds ratio (AOR) for ≥2 catheterizations 2.4, 95% confidence interval (CI) 1.1-4.9], belief the cardiologist better knew the child's medical history (AOR 2.4, 95% CI 1.3-4.6), and provider accessibility (AOR 3.6, 95% CI 1.8-7.4). Parents of CHD patients reported close alignment with PCPs and would contact PCPs first for most routine care. However, some populations continue to contact cardiologists for routine care. Further study is needed to clarify best practices for clinician and parent education.

Neurodevelopmental outcomes after cardiac surgery in infancy.

BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI). RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02). CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Risk factors for prolonged length of stay after the stage 2 procedure in the single-ventricle reconstruction trial.

BACKGROUND: The single-ventricle reconstruction trial randomized patients with single right ventricle lesions to a modified Blalock-Taussig or right ventricle-to-pulmonary artery shunt at the Norwood. This analysis describes outcomes at the stage 2 procedure and factors associated with a longer hospital length of stay (LOS). METHODS: We examined the association of shunt type with stage 2 hospital outcomes. Cox regression and bootstrapping were used to evaluate risk factors for longer LOS. We also examined characteristics associated with in-hospital death. RESULTS: There were 393 subjects in the analytic cohort. Median stage 2 procedure hospital LOS (8 days; interquartile range [IQR], 6-14 days), hospital mortality (4.3%), transplantation (0.8%), median ventilator time (2 days; IQR, 1-3 days), median intensive care unit LOS (4 days; IQR, 3-7 days), number of additional cardiac procedures or complications, and serious adverse events did not differ by shunt type. Longer LOS was associated (R(2) = 0.26) with center, longer post-Norwood LOS (hazard ratio [HR], 1.93 per log day; P < .001), nonelective timing of the stage 2 procedure (HR, 1.78; P < .001), and pulmonary artery (PA) stenosis (HR, 1.56; P < .001). By univariate analysis, nonelective stage 2 (65% vs 32%; P = .009), moderate or greater atrioventricular valve (AVV) regurgitation (75% vs 24%; P < .001), and AVV repair (53% vs 9%; P < .001) were among the risk factors associated with in-hospital death. CONCLUSIONS: Norwood LOS, PA stenoses, and nonelective stage 2 procedure, but not shunt type, are independently associated with longer LOS. Nonelective stage 2 procedure, moderate or greater AVV regurgitation, and need for AVV repair are among the risk factors for death.

Timing of neonatal cardiac surgery is not associated with perioperative outcomes.

OBJECTIVE: The optimal timing for neonatal cardiac surgery is unknown. We aimed to determine the relationship between age at surgery and perioperative outcomes, hypothesizing that earlier intervention would be associated with lower morbidity and mortality. METHODS: A retrospective review was performed of neonates who had undergone an arterial switch operation, stage 1 palliation for functional single ventricle, or systemic-to-pulmonary shunt for obstructed pulmonary blood flow from January 1, 2005, to December 31, 2010. The subjects with clinical indications for delayed surgery or prematurity were excluded. Age at surgery was evaluated as both a continuous and a categorical variable. The primary outcome was a composite endpoint of mortality or prolonged intensive care stay. RESULTS: Of 344 subjects, 286 (77 arterial switch operation, 124 stage 1 palliation, 85 systemic-to-pulmonary shunt) met the inclusion criteria. In each group, age at surgery was not associated with the primary composite endpoint. The patients who died after systemic-to-pulmonary shunt had a median age at surgery of 3 days versus 6 days for the survivors (P = .04). A similar, but nonsignificant, pattern was seen for patients undergoing arterial switch operations (4.5 vs 7 days; P = .09). Earlier surgery was not associated with a reduced duration of vasoactive support, mechanical ventilation, or intensive care unit length of stay in any group. Stage 1 palliation subjects in the upper age quartile (≥8 days) at surgery were less likely to require prolonged mechanical ventilation (P = .03). CONCLUSIONS: Younger age at intervention in the neonatal period was not associated with reduced morbidity or mortality in any procedural subgroup studied. In our cohort, earlier systemic-to-pulmonary shunt for obstructed pulmonary blood flow was associated with a greater likelihood of a poor outcome.

Design and rationale of safe pediatric euglycemia after cardiac surgery: a randomized controlled trial of tight glycemic control after pediatric cardiac surgery.

OBJECTIVES: To describe the design of a clinical trial testing the hypothesis that children randomized to tight glycemic control with intensive insulin therapy after cardiac surgery will have improved clinical outcomes compared to children randomized to conventional blood glucose management. DESIGN: Two-center, randomized controlled trial. SETTING: Cardiac ICUs at two large academic pediatric centers. PATIENTS: Children from birth to those aged 36 months recovering in the cardiac ICU after surgery with cardiopulmonary bypass. INTERVENTIONS: Subjects in the tight glycemic control (intervention) group receive an intravenous insulin infusion titrated to achieve normoglycemia (target blood glucose range of 80-110 mg/dL; 4.4-6.1 mmol/L). The intervention begins at admission to the cardiac ICU from the operating room and terminates when the patient is ready for discharge from the ICU. Continuous glucose monitoring is performed during insulin infusion to minimize the risks of hypoglycemia. The standard care group has no target blood glucose range. MEASUREMENTS AND MAIN RESULTS: The primary outcome is the development of any nosocomial infection (bloodstream, urinary tract, and surgical site infection or nosocomial pneumonia). Secondary outcomes include mortality, measures of cardiorespiratory function and recovery, laboratory indices of nutritional balance, immunologic, endocrinologic, and neurologic function, cardiac ICU and hospital length of stay, and neurodevelopmental outcome at 1 and 3 yrs of age. A total of 980 subjects will be enrolled (490 in each treatment arm) for sufficient power to show a 50% reduction in the prevalence of the primary outcome. CONCLUSIONS: Pediatric cardiac surgery patients may recognize great benefit from tight glycemic control in the postoperative period, particularly with regard to reduction of nosocomial infections. The Safe Pediatric Euglycemia after Cardiac Surgery trial is designed to provide an unbiased answer to the question of whether this therapy is indeed beneficial and to define the associated risks of therapy.

Risk factors for surgical site infection in pediatric cardiac surgery patients undergoing delayed sternal closure.

OBJECTIVES: To determine the incidence of surgical site infections (SSIs) in congenital heart surgery (CHS) patients undergoing delayed sternal closure (DSC) and to evaluate risk factors for SSI. METHODS: A nested case-control study was performed within a cohort of CHS patients undergoing DSC at our institution between 2005 and 2009. Cases met 2008 Centers for Disease Control and Prevention criteria for SSI; control subjects were matched based on year of surgery. Uni- and multivariate logistic regressions were performed to identify SSI risk factors. RESULTS: Of 375 patients who underwent DSC, 43 (11%) developed an SSI. The analysis included 172 patients (43 cases, 129 controls); 118 (69%) were neonates, 80 (47%) had undergone Norwood procedure, and 150 (87%) had DSC initiated in the operating room. Case and control subjects were similar based on pre- and intraoperative characteristics. Duration of mechanical ventilation, intensive care unit and hospital length of stay, and mortality were significantly greater in patients with an SSI. Multiple periods of DSC, longer duration of DSC, greater dependence on parenteral nutrition, and extracorporeal membrane oxygenation were significantly associated with SSI in univariate analyses. Multivariate analysis demonstrated that multiple periods of DSC (adjusted odds ratio, 5.9; 95% confidence interval, 1.7-20.1) and extracorporeal membrane oxygenation (adjusted odds ratio, 2.9; 95% confidence interval, 1.1-7.6) remained independent risk factors for SSI. CONCLUSIONS: For CHS patients undergoing DSC, extracorporeal membrane oxygenation and multiple periods of DSC are independent risk factors for SSI. New strategies for prevention and prophylaxis of SSI may be indicated for these high-risk patients who have worse outcomes and greater health care resource utilization.

Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial.

OBJECTIVE: For infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors. METHODS: Participants in the Single Ventricle Reconstruction trial who survived to discharge after the Norwood procedure were included (n = 426). Interstage mortality was defined as death postdischarge after the Norwood procedure and before the stage II procedure. Univariate analysis and multivariable logistic regression were performed adjusting for site. RESULTS: Overall interstage mortality was 50 of 426 (12%)-13 of 225 (6%) for RVPAS and 37 of 201 (18%) for MBTS (odds ratio [OR] for MBTS, 3.4; P < .001). When moderate to severe postoperative atrioventricular valve regurgitation (AVVR) was present, interstage mortality was similar between shunt types. Interstage mortality was independently associated with gestational age less than 37 weeks (OR, 3.9; P = .008), Hispanic ethnicity (OR, 2.6; P = .04), aortic atresia/mitral atresia (OR, 2.3; P = .03), greater number of post-Norwood complications (OR, 1.2; P = .006), census block poverty level (P = .003), and MBTS in subjects with no or mild postoperative AVVR (OR, 9.7; P < .001). CONCLUSIONS: Interstage mortality remains high at 12% and is increased with the MBTS compared with the RVPAS if postoperative AVVR is absent or mild. Preterm delivery, anatomic, and socioeconomic factors are also important. Avoiding preterm delivery when possible and close surveillance after Norwood hospitalization for infants with identified risk factors may reduce interstage mortality.