ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Factors associated with the need for inotropic support following pulmonary artery banding surgery for CHD.

OBJECTIVE: We aimed to identify factors independently associated with the need for inotropic support for low cardiac output or haemodynamic instability after pulmonary artery banding surgery for CHD. METHODS: We performed a retrospective chart review of all neonates and infants who underwent pulmonary banding between January 2016 and June 2019 at our institution. Bivariate and multivariable analyses were performed to identify factors independently associated with the use of post-operative inotropic support, defined as the initiation of inotropic infusion(s) for depressed myocardial function, hypotension, or compromised perfusion within 24 hours of pulmonary artery banding. RESULTS: We reviewed 61 patients. Median age at surgery was 10 days (25%,75%:7,30). Cardiac anatomy was biventricular in 38 patients (62%), hypoplastic right ventricle in 14 patients (23%), and hypoplastic left ventricle in 9 patients (15%). Inotropic support was implemented in 30 patients (49%). Baseline characteristics of patients who received inotropic support, including ventricular anatomy and pre-operative ventricular function, were not statistically different from the rest of the cohort. Patients who received inotropic support, however, were exposed to larger cumulative doses of ketamine intraoperatively - median 4.0 mg/kg (25%,75%:2.8,5.9) versus 1.8 mg/kg (25%,75%:0.9,4.5), p < 0.001. In a multivariable model, cumulative ketamine dose greater than 2.5mg/kg was associated with post-operative inotropic support (odds ratio 5.5; 95% confidence interval: 1.7,17.8), independent of total surgery time. CONCLUSIONS: Inotropic support was administered in approximately half of patients who underwent pulmonary artery banding and more commonly occurred in patients who received higher cumulative doses of ketamine intraoperatively, independent of the duration of surgery.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia.

BACKGROUND: We sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. METHODS: This retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. RESULTS: Of 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. CONCLUSIONS: For infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

BACKGROUND: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE). METHODS: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression models. RESULTS: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, stroke occurred in 16 (6%), and 23 (8%) died. The presence of 2 major coronary artery stenoses (adjusted odds ratio, 4.99; 95% CI, 1.16-21.39) and lower weight at first intervention (adjusted odds ratio, 1.52; 95% CI, 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n = 10). CONCLUSIONS: In a multicenter cohort, 1 in 5 neonates with PA-IVS experienced MACE after their initial intervention. Patients with 2 major coronary artery stenoses or lower weight at the time of the initial procedure were most likely to experience MACE and warrant vigilance during preintervention planning and postintervention management.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

BACKGROUND: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited. METHODS: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions. RESULTS: We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00). CONCLUSIONS: In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.

Cardiac Arrest in Children Following Cardiac Surgery: A Scoping Review of Contributing Factors.

Nearly half of children experiencing cardiac arrest following cardiac surgery do not survive hospital discharge and patients who survive often experience significant neurological impairment. Additionally, increased resource utilization following cardiac arrest translates into adverse logistical and financial consequences. Although some studies have identified patient characteristics that increase the risk of cardiac arrest after pediatric cardiac surgery, modifiable risk factors, which could provide a foundation for effective prevention strategies, have been elusive. This scoping review explores the current knowledge surrounding risk factors associated with cardiac arrest in children following cardiac surgery and provides recommendations for future research.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p < 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

Development and Validation of a Seizure Prediction Model in Neonates After Cardiac Surgery.

BACKGROUND: Electroencephalographic seizures (ESs) after neonatal cardiac surgery are often subclinical and have been associated with poor outcomes. An accurate ES prediction model could allow targeted continuous electroencephalographic monitoring (CEEG) for high-risk neonates. METHODS: ES prediction models were developed and validated in a multicenter prospective cohort where all postoperative neonates who underwent cardiopulmonary bypass (CPB) also underwent CEEG. RESULTS: ESs occurred in 7.4% of neonates (78 of 1053). Model predictors included gestational age, head circumference, single-ventricle defect, deep hypothermic circulatory arrest duration, cardiac arrest, nitric oxide, extracorporeal membrane oxygenation, and delayed sternal closure. The model performed well in the derivation cohort (c-statistic, 0.77; Hosmer-Lemeshow, P = .56), with a net benefit (NB) over monitoring all and none over a threshold probability of 2% in decision curve analysis (DCA). The model had good calibration in the validation cohort (Hosmer-Lemeshow, P = .60); however, discrimination was poor (c-statistic, 0.61), and in DCA there was no NB of the prediction model between the threshold probabilities of 8% and 18%. By using a cut point that emphasized negative predictive value in the derivation cohort, 32% (236 of 737) of neonates would not undergo CEEG, including 3.5% (2 of 58) of neonates with ESs (negative predictive value, 99%; sensitivity, 97%). CONCLUSIONS: In this large prospective cohort, a prediction model of ESs in neonates after CPB had good performance in the derivation cohort, with an NB in DCA. However, performance in the validation cohort was weak, with poor discrimination, poor calibration, and no NB in DCA. These findings support CEEG of all neonates after CPB.

Intermediate Outcomes After Repair of Anomalous Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: Multicenter studies on infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) are lacking. We report the intermediate-term outcomes after ALCAPA repair in a multicenter cohort and identify risk factors for reintervention or death after discharge. METHODS: We retrospectively reviewed infants under 1 year of age who underwent ALCAPA repair from January 2009 to March 2018 at 21 US centers. The primary composite outcome was freedom from reintervention or death after discharge. We used the Kaplan-Meier survival analysis to examine freedom from reintervention or death and the Cox proportional hazard analysis to identify risk factors for this composite outcome. RESULTS: One hundred seventy-seven infants underwent ALCAPA repair; 170 (97%) survived to hospital discharge without transplantation. Twenty-three patients were lost to follow-up. The median duration of follow-up in the remaining 147 patients was 3.8 years (25%, 75%: 1.9 years, 6.0 years). Echocardiographic data were available at ∼3 years after discharge in 98 patients. Left ventricular function was normal in 96 patients (98%), whereas 26 patients (27%) had greater than mild mitral valve regurgitation. Sixteen patients (11%) underwent 20 reinterventions with 1 late death. Patients undergoing the Takeuchi procedure or atypical repairs (hazard ratio, 8.0; 95% confidence interval, 2.1-30.0) or with moderate or greater mitral regurgitation on discharge echocardiogram (hazard ratio, 3.4; 95% confidence interval, 1.2-9.1) were at increased risk for reintervention. CONCLUSIONS: Intermediate-term outcomes after ALCAPA repair in infants are favorable. Persistent left ventricular dysfunction and reinterventions were uncommon, and mortality was rare. Patients who required atypical surgical repair or had moderate or greater mitral regurgitation at discharge warrant closer follow-up.

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described. To characterize this cohort, we compared these data to patients repaired before 1 year of age. Of 248 ALCAPA patients, 71 (29%) underwent repair ≥1 year of age. Among this subset, the median age at diagnosis was 8.3 years. Chronic arrhythmia occurred in 7%. Patients had good postoperative recovery of left ventricle (LV) dysfunction (90%) and LV dilation (75%), although a low incidence of recovery of mitral regurgitation (40%). Compared to infants, older patients were more likely to present with cardiac arrest (11% vs 1%) and less likely to have moderate or worse LV dysfunction or mitral regurgitation. Older patients had significantly less postoperative extracorporeal membrane oxygenation use, and shorter ICU and hospital stay. In the older cohort, operative mortality occurred in only 1 patient and no patient died after discharge (median follow-up 2.7 years). Survival of patients who presented with ALCAPA beyond infancy was excellent, although chronic mitral regurgitation and chronic arrhythmia were not uncommon. Patients who underwent ALCAPA repair ≥1 year of age were less likely to present with LV dysfunction but more likely to present with cardiac arrest than younger patients.

Effects of Vasopressin Infusion After Pediatric Cardiac Surgery: A Meta-analysis.

Vasopressin has been used to augment blood pressure; however, cardiovascular effects after cardiac surgery have not been well established. The primary objective of this study was to survey the current literature and quantify the pooled effect of vasopressin on hemodynamic parameters in children after pediatric cardiac surgery. A systematic review was conducted to identify studies characterizing the hemodynamic effects of vasopressin after pediatric cardiac surgery. Studies were assessed and those of satisfactory quality with pre- and post-vasopressin hemodynamics for each patient were included in the final analyses. 6 studies with 160 patients were included for endpoints during the first 2 h of infusions. Patients who received vasopressin infusion had greater mean, systolic, and diastolic blood pressures and lower heart rates at 2 h after initiation. 8 studies with 338 patients were included for the effects at 24 h. Patients who received vasopressin infusion had lower central venous pressures and decreased lactate concentrations 24 h after initiation. A subset analysis for children with functionally univentricular hearts found significant decrease in inotrope score and central venous pressure. A subset analysis for neonates found significant decrease in inotrope score and fluid balance. Vasopressin leads to decrease in heart rate and increase in blood pressure in the first 2 h of initiation. Later effects include decrease in inotrope score, central venous pressure, fluid balance, and in lactate within the first 24 h. Findings vary in neonates and in those with functionally univentricular hearts although beneficial effects are noted in both.

Analysis of factors associated with prolonged post-operative course after surgical repair of aortic coarctation.

OBJECTIVE: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation. METHODS: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay. RESULTS: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay. CONCLUSIONS: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

Upper body peripherally inserted central catheter in pediatric single ventricle patients.

BACKGROUND: There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement. This complication is more serious among patients with single ventricle physiology, as it might preclude them from undergoing further life-sustaining palliative surgery. AIM: To describe complications associated with the use of upper extremity percutaneous intravenous central catheters (PICCs) in children with single ventricle physiology. METHODS: A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure. Clinical data including ultrasonography, cardiac catheterization, echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels. Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter (CVC), and use of anticoagulation were recorded. RESULTS: Seventy-six patients underwent superior cavopulmonary anastomoses, of which 56 (73%) had an upper extremity PICC at some point prior to this procedure. Median duration of PICC usage was 24 d (25%, 75%: 12, 39). Seventeen patients (30%) with PICCs also had internal jugular or subclavian central venous catheters (CVCs) in place at some point prior to their superior cavopulmonary anastomoses, median duration 10 d (25%, 75%: 8, 14). Thrombus was detected in association with 2 of the 56 PICCs (4%) and 3 of the 17 CVCs (18%). All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients. No patients developed clinically significant venous stenosis. CONCLUSION: Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheter-associated thrombosis.

Emergency department utilisation and critical readmission in patients with Fontan circulation.

BACKGROUND: We aimed to conduct a multi-centre study characterising emergency department utilisation and critical readmissions experienced by children with Fontan circulation. METHODS: We conducted a retrospective review of children who underwent the Fontan operation at three institutions (i.e., centres A, B, and C) between 2009 and 2014, with follow-up through December 2015. Multi-variable analyses were performed to determine factors associated for emergency department utilisation within 1 year of surgery, emergency department utilisation at any time following surgery, or critical readmission (defined as admission to ICU, operating room, or cardiac catheterisation). RESULTS: We reviewed 297 patients, of which 147 patients (49%) had 607 emergency department encounters. Forty-six patients (15%) required 71 critical readmissions. Multi-variable analyses revealed centre C (p = 0.02) and post-operative hospitalisation ≥ 14 days (p = 0.03) to be significantly associated with emergency department utilisation within 1 year, whereas centre B (p < 0.001), post-operative hospitalisation ≥ 14 days (p = 0.002), and African-American/Black race (p = 0.04) were significantly associated with critical readmission. CONCLUSIONS: In this multi-centre study, nearly half of patients with Fontan circulation received emergency department care, often presenting with high disease acuity requiring readmission. Emergency department utilisation and need for critical readmission were independently influenced by the centre at which surgery was performed, prolonged post-operative hospitalisation, and racial background. These data could help guide quality improvement efforts aimed at reducing morbidity in this unique patient population.

Influence of Early Extubation Location on Outcomes Following Pediatric Cardiac Surgery.

OBJECTIVES: Early extubation following pediatric cardiac surgery is common, but debate exists whether location affects outcome, with some centers performing routine early extubations in the operating room (odds ratio) and others in the cardiac ICU. We aimed to define early extubation practice variation across hospitals and assess impact of location on hospital length-of-stay and other outcomes. DESIGN: Secondary analysis of the Pediatric Cardiac Critical Care Consortium registry. SETTING: Twenty-eight Pediatric Cardiac Critical Care Consortium hospitals. PATIENTS: Patients undergoing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1-3 operations between August 2014 and February 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We defined early extubation as extubation less than 6 hours after postoperative admission. Hospitals were categorized based on the proportion of their early extubation patients who underwent an odds ratio extubation. Categories included low- (< 50% of early extubation, n = 12), medium- (50%-90%, n = 8), or high- (> 90%, n = 8) frequency odds ratio early extubation centers. The primary outcome of interest was postoperative hospital length-of-stay. We analyzed 16,594 operations (9,143 early extubation, 55%). Rates of early extubation ranged from 16% to 100% across hospitals. Odds ratio early extubation rates varied from 16% to 99%. Patient characteristics were similar across hospital odds ratio early extubation categories. Early extubation rates paralleled the hospital odds ratio early extubation rates-77% patients underwent early extubation at high-frequency odds ratio extubation centers compared with 39% at low-frequency odds ratio extubation centers (p < 0.001). High- and low-frequency odds ratio early extubation hospitals had similar length-of-stay, cardiac arrest rates, and low mortality. However, high-frequency odds ratio early extubation hospitals used more noninvasive ventilation than low-frequency hospitals (15% vs. 9%; p < 0.01), but had fewer extubation failures (3.6% vs. 4.5%; p = 0.02). CONCLUSIONS: Considerable variability exists in early extubation practices after low- and moderate-complexity pediatric cardiac surgery. In this patient population, hospital length-of-stay did not differ significantly between centers with different early extubation strategies based on location or frequency.

Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-to-moderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable.

Right Ventricular Outflow Tract Reconstruction in Infant Truncus Arteriosus: A 37-year Experience.

BACKGROUND: Multiple conduits for right ventricular outflow tract reconstruction exist, although the ideal conduit that maximizes outcomes remains controversial. We evaluated long-term outcomes and compared conduits for right ventricular outflow tract reconstruction in children with truncus arteriosus. METHODS: Records of patients who underwent truncus arteriosus repair at our institution between 1981 and 2018 were retrospectively reviewed. Primary outcomes included survival and freedom from catheter reintervention or reoperation. Secondary analyses evaluated the effect of comorbidity, operation era, conduit type, and conduit size. RESULTS: One hundred patients met inclusion criteria. Median follow-up time was 15.6 years (interquartile range, 5.3-22.2). Actuarial survival at 30 days, 5 years, 10 years, and 15 years was 85%, 72%, 72%, and 68%, respectively. Early mortality was associated with concomitant interrupted aortic arch (hazard ratio, 5.4; 95% confidence interval, 1.7-17.4; P = .005). Median time to surgical reoperation was 4.6 years (interquartile range, 2.9-6.8; n = 58). Right ventricle to pulmonary artery continuity was established with an aortic homograft (n = 14), pulmonary homograft (n = 41), or bovine jugular vein conduit (n = 36) in most cases. Multivariate analysis revealed longer freedom from reoperation with the bovine jugular vein conduit compared with the aortic homograft (hazard ratio, 3.1; 95% confidence interval, 1.3-7.7; P = .02) with no difference compared with the pulmonary homograft. Larger conduit size was associated with longer freedom from reoperation (hazard ratio, 0.7; 95% confidence interval, 0.6-0.9; P < .001). CONCLUSIONS: The bovine jugular vein conduit is a favorable conduit for right ventricular outflow tract reconstruction in patients with truncus arteriosus. Concomitant interrupted aortic arch is a risk factor for early mortality.

Minimization of Surgical Site Infections in Patients With Delayed Sternal Closure After Pediatric Cardiac Surgery.

BACKGROUND: Delayed sternal closure (DSC) following pediatric cardiac surgery is commonly implemented at many centers. Infectious complications occur in 18.7% of these patients based on recent multicenter data. We aimed to describe our experience with DSC, hypothesizing that our practices surrounding the implementation and maintenance of the open sternum during DSC minimize the risk of infectious complications. METHODS: We reviewed patients less than 365 days who underwent DSC between 2012 and 2016 at our institution. Infectious complications as defined by the Society of Thoracic Surgeons Congenital Heart Surgery Database were recorded. Patients with and without infectious complications were compared using Wilcoxon rank sum tests or Fisher exact tests as appropriate. RESULTS: We identified 165 patients less than 365 days old who underwent DSC, 135 (82%) of whom had their skin closed over their open sternum. Median duration of open sternum was 3 days (range: 1-32 days). Infectious complications occurred in 15 (9.1%) patients-13 developed clinical sepsis with positive blood cultures, one patient developed ventilator-associated pneumonia, and one patient developed wound infection (0.6%). No cases of mediastinitis occurred. No statistical differences in characteristics between patients with and without infectious complications could be identified. CONCLUSION: Infectious complications after DSC at our institution were notably less than reported in recent literature, primarily due to minimization of surgical site infections. Practices described in the article, including closing skin over the open sternum whenever possible, could potentially aid other institutions aiming to reduce infectious complications associated with DSC.

Near-infrared spectroscopy for prediction of extubation success after neonatal cardiac surgery.

INTRODUCTION: Reliable predictors of extubation readiness are needed and may reduce morbidity related to extubation failure. We aimed to examine the relationship between changes in pre-extubation near-infrared spectroscopy measurements from baseline and extubation outcomes after neonatal cardiac surgery. MATERIALS AND METHODS: In this retrospective cross-sectional multi-centre study, a secondary analysis of prospectively collected data from neonates who underwent cardiac surgery at seven tertiary-care children's hospitals in 2015 was performed. Extubation failure was defined as need for re-intubation within 72 hours of the first planned extubation attempt. Near-infrared spectroscopy measurements obtained before surgery and before extubation in patients who failed extubation were compared to those of patients who extubated successfully using t-tests. RESULTS: Near-infrared spectroscopy measurements were available for 159 neonates, including 52 with single ventricle physiology. Median age at surgery was 6 days (range: 1-29 days). A total of 15 patients (9.4 %) failed extubation. Baseline cerebral and renal near-infrared spectroscopy measurements were not statistically different between those who were successfully extubated and those who failed, but pre-extubation cerebral and renal values were significantly higher in neonates who extubated successfully. An increase from baseline to time of extubation values in cerebral oximetry saturation by ≥ 5 % had a positive predictive value for extubation success of 98.6 % (95%CI: 91.1-99.8 %). CONCLUSION: Pre-extubation cerebral near-infrared spectroscopy measurements, when compared to baseline, were significantly associated with extubation outcomes. These findings demonstrate the potential of this tool as a valuable adjunct in assessing extubation readiness after paediatric cardiac surgery and warrant further evaluation in a larger prospective study.

Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis.

OBJECTIVE: We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) in the immediate postoperative period in a contemporary multicenter cohort. METHODS: We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded. MACE was defined as the need for postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. Risk factors for MACE were identified using multivariable logistic regression analysis and reported as odds ratios (ORs) with 95% confidence intervals (CIs). RESULTS: We reviewed 216 patients. MACE occurred in 44 patients (20%) and did not vary significantly over time. Twenty-two patients (10%) received postoperative extracorporeal membrane oxygenation, 26 (12%) received cardiopulmonary resuscitation, and 15 (7%) suffered operative mortality. With multivariable logistic regression analysis (which included adjustment for center effect), factors independently associated with MACE were failure to diagnose truncus arteriosus before discharge from the nursery (OR, 3.1; 95% CI, 1.3-7.4), cardiopulmonary bypass duration >150 minutes (OR, 3.5; 95% CI, 1.5-8.5), and right ventricle-to-pulmonary artery conduit diameter >50 mm/m2 (OR, 4.7; 95% CI, 2.0-11.1). CONCLUSIONS: In a contemporary multicenter analysis, 20% of children who underwent repair of truncus arteriosus experienced MACE. Early diagnosis, shorter duration of cardiopulmonary bypass, and use of smaller diameter right ventricle-to-pulmonary artery conduits represent potentially modifiable factors that could decrease morbidity and mortality in this fragile patient population.