Risk of Hypovitaminosis and Vitamin C Deficiency in Pediatric Patients Undergoing Cardiopulmonary Bypass.

Vitamin C levels are known rapidly decrease in adult critical illness. Vitamin C scavenges free radicals, provides critical protection of the endothelial barrier, and improves endothelial responsiveness to catecholamines. Children with congenital heart disease and undergoing cardiac surgery might be at increased risk for low circulating vitamin C levels. A prospective single-center observational study investigated perioperative changes in vitamin C levels in critically ill Children who underwent congenital heart surgery using CPB. Vitamin C serum levels were collected preoperatively and postoperatively (upon admission to the ICU, 24 and 72 h). Linear mixed-effect model was used to estimate mean circulating concentration of vitamin C and to estimate changes in concentration over time. Primary outcome was change in circulating levels of vitamin C before and after CPB. Secondary outcomes were hospital length of stay (LOS), acute kidney injury (AKI), and illness severity. Forty-one patients with a median age of 4.5 [interquartile range (IQR) 2.6-65.6] months at the time of surgery were consented and enrolled. Median CPB duration was 130 [90-175] minutes, and hospital LOS was 9.1 [5.2-19] days. Mean vitamin C levels (µmol/L) before CPB, at PICU admission, 24 h, and 72 h were 82.0 (95% CI 73.4-90.7), 53.4 (95% CI 44.6,62.0), 55.1 (95% CI 46.3,63.8), and 59.2 (95% CI 50.3,68.1), respectively. Upon postoperative admission to the PICU, vitamin C levels decreased by 28.7 (95% CI 20.6-36.8; p < 0.001) µmol/L, whereas levels at 24 and 72 h recovered and did not differ substantially from concentrations reported upon PICU admission (p > 0.15). Changes in vitamin C concentration were not associated with CPB time, STAT mortality category, age, or PIM3. Three patients had post-CPB hypovitaminosis C or vitamin C deficiency. Reduction in vitamin C levels was not associated with hospital LOS (p = 0.673). A 25 µmol/L decrease in vitamin C levels upon PICU admission was associated with developing AKI (aOR = 3.65; 95% CI 1.01-18.0, p = 0.049). Pediatric patients undergoing cardiac surgery with CPB showed decreased vitamin C levels during the immediate postoperative period. Effects of hypovitaminosis C and vitamin C deficiency in this population remain unclear.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Systems of Care Factors Should Be Considered in Regionalization of Congenital Cardiac Surgery.

BACKGROUND: Regionalization of care has been proposed to optimize outcomes in congenital cardiac surgery (CCS). We hypothesized that hospital infrastructure and systems of care factors could also be considered in regionalization efforts. METHODS: Observed-to-expected (O/E) mortality ratio and hospital volumes were obtained between 2015 and 2018 from public reporting data. Using a resource dependence framework, we examined factors obtained from American Hospital Association, Children's Hospital Association, and hospital websites. Linear regression models were estimated with volume only, then with hospital factors, stratified by procedural complexity. Robust regression models were reestimated to assess the impact of outliers. RESULTS: We found wide variation in the volume of congenital cardiac surgeries performed (89-3920) and in the surgical outcomes (O/E ratio range, 0.3-3.1). Six outlier hospitals performed few high-complexity cases with high mortality. Univariate analysis including all cases indicated that higher volume predicted lower O/E ratio (ß = -0.02; SE = 0.008; P = .011). However, this effect was driven by the most complex cases. Models stratified by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category show that volume is a significant predictor only in category 5 cases (ß = -1.707; SE = 0.663; P = .012). Robust univariate regression accounting for outliers found no effect of volume on O/E ratio (ß = 0.005; SE = 0.002; P = .975). Elimination of outliers through robust multivariate regression decreased the volume-outcome relationship and found a modest relationship between health plan ownership and outcomes. CONCLUSIONS: Systems of care factors should be considered in addition to volume in designing regionalization in CCS. Patient-level data sets will better define these factors.

Nicardipine Is a Safe, Efficacious, and Cost-Effective Antihypertensive for Neonates and Young Infants Undergoing Cardiac Surgery.

OBJECTIVE: The use of nicardipine in congenital cardiac surgery has been guarded given the calcium sensitivity of immature myocardium and paucity of clinical data. Reports of nicardipine use have excluded neonates with single ventricles. The goal of this study was to compare the use of nicardipine and sodium nitroprusside for postoperative blood pressure control in young patients recovering from cardiac surgery. METHODS: All neonates (<30 days) and young infants (31-180 days) who received either sodium nitroprusside or nicardipine as first-line therapy for blood pressure control were retrospectively reviewed. Some patients had multiple index operations and each index operation was counted separately regarding treatment with sodium nitroprusside or nicardipine. RESULTS: A total of 59 patients underwent 70 procedures (24 as neonates and 46 as infants). Nicardipine was administered as initial therapy following 33 procedures (n = 28 patients), and sodium nitroprusside was administered as initial therapy following 37 index procedures (n = 31 patients). The duration of treatment was longer (P = .025) when sodium nitroprusside was the initial treatment. Five (15%) patients that received nicardipine required a second blood pressure management agent, and seven (19%) patients that received sodium nitroprusside required a second agent (P = .66). No adverse events related to titratable antihypertensive therapy were recorded in any treatment group. The use of nicardipine resulted in significant medication cost reduction. Based on average wholesale price, patient costs for sodium nitroprusside use were $182,952 ($5,544/pt), while costs for nicardipine were only $24,960 ($780/pt). CONCLUSIONS: Nicardipine can be safely used as a first-line antihypertensive in infants. The use of nicardipine as initial antihypertensive therapy rather than sodium nitroprusside can lead to a significant reduction in medication costs without jeopardizing clinical outcomes.

Is Porcine Small Intestinal Submucosal Extracellular Matrix (ECM) a Suitable Material for Right Ventricular Outflow Tract Reconstruction in Association With Pulmonary Valve Replacement?

Pulmonary valve replacement (PVR) with right ventricular outflow tract (RVOT) reconstruction is a common congenital cardiac operation. Porcine submucosal intestinal-derived extracellular matrix (ECM) patches have been used for RVOT reconstruction. We present 2 adult patients with Tetralogy of Fallot who underwent PVR with RVOT reconstruction utilizing ECM. Both cases required reoperation due to patch dehiscence causing a large paravalvular leak. One patient also had a pseudoaneurysm associated with ECM dehiscence. There may be a propensity for ECM dehiscence in this application and, based on these cases, we recommend avoidance of ECM in RVOT reconstruction with PVR. PVR patients repaired with ECM should be monitored for this complication.

The Use of STS Public Reporting to Guide Referrals in Congenital Heart Surgery: Results of a Survey.

BACKGROUND: The Society of Thoracic Surgeons (STS) public reporting in congenital heart surgery has received considerable attention; however, it is unclear how pediatric cardiac providers use these data to guide surgical referrals. METHODS: We surveyed members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet members regarding use of STS public reporting. RESULTS: There were 155 respondents (90% cardiologist, 7% surgeons) from approximately 800 solicitations (∼19% response rate). While most (83%) felt that STS public reporting is important, 60% are unsure of its accuracy and only 37% find it useful in practice. Most (71%) believe STS public reporting leads to risk aversion. Overall, 92% answered that STS public reporting rarely or never overrides other factors determining referrals. Compared with smaller centers (<300 cases/year), providers in larger centers were more likely to report that STS public reporting data never overrides other factors determining referrals (54% vs 32%, P = .03). Providers using STS public reporting to guide referrals (14% overall) trust the system's accuracy (P = .03) and believe it presents useful outcomes (P < .01). There was no correlation between use of STS public reporting to guide referrals and practice size, type, location, time in practice, surgical center affiliation, or center volume. CONCLUSIONS: Providers believe that public reporting of outcomes is important; however, most do not use the data to guide surgical referrals. Understanding these limitations of the current STS public reporting may enable change and increased usefulness for providers.

The Use of Alveolar Dead Space Fraction to Predict Postoperative Outcomes after Pediatric Cardiac Surgery: A Retrospective Study.

Patients with congenital heart disease (CHD) that have surgical repair with cardiopulmonary bypass (CPB) reflect a unique population with multiple pulmonary and systemic factors that may contribute to increased alveolar dead space and low cardiac output syndrome. This study aimed to assess and compare changes in the alveolar dead space fraction (AVDSf) in the immediate postoperative period with outcomes in children with CHD who underwent repair on CPB. A single-center retrospective review study of critically ill children with CHD, younger than 18 years of age admitted to the Pediatric Intensive Care Unit (PICU) after undergoing surgical repair on CPB and received invasive mechanical ventilation for at least 24 h. One hundred and two patients were included in the study. Over the first 24 h, mean AVDSf was significantly higher in patients who had longer hospital length of stay (LOS) (> 21 days) p = 0.02, and longer duration of invasive mechanical ventilation (DMV) (> 170 h) p = 0.01. Cross-sectional analyses at 23-24 h revealed that AVDSf > 0.25 predicts mortality and DMV (p = 0.03 and P = 0.02 respectively); however, it did not predict prolonged hospital LOS. For every 0.1 increase in the AVDSf, the odds of mortality, DMV, and hospital LOS increased by 4.9 [95% CI = 1.45-16.60, p = 0.002], 2.06 [95% CI = 1.14-3.71, p = 0.01], and 1.43[95% CI = 0.84-2.45, p = 0.184], respectively. The area under the ROC curve at 23-24 h for AVDSf was 0.868 to predict mortality as an outcome. AVDSf > 0.25 at 23-24 h postoperatively was an independent predictor of mortality with sensitivity and specificity of 83% and 80%, respectively and was superior to other commonly used surrogates of cardiac output. In the immediate postoperative period of pediatric patients with CHD, high AVDSf is associated with longer hospital length of stay and duration of invasive mechanical ventilation. Increased AVDSf values at 23-24 h postoperatively is associated with mortality in patients with CHD exposed to CPB.

Case report: a step-wise management of concurrent presentation of congenital single lung and aberrant right subclavian artery in an infant girl.

INTRODUCTION: Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA. CASE PRESENTATION: An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms. CONCLUSIONS: Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.

Prophylactic Peritoneal Drainage is Associated with Improved Fluid Output after Congenital Heart Surgery.

Infants undergoing congenital heart surgery (CHS) with cardiopulmonary bypass (CPB) are at risk of acute kidney injury (AKI) and fluid overload. We hypothesized that placement of a passive peritoneal drain (PPD) can improve postoperative fluid output in such infants. We analyzed 115 consecutive patients, age birth to 60 days, admitted to the PICU after CHS with CPB between 2012 and 2018. Patients who needed postoperative ECMO were excluded. Linear and logistic regression models compared postoperative fluid balances, diuretics administration, AKI, vasoactive-inotropic scores (VIS), time intubated, and length of stay after adjusting for pre/operative predictors including STAT category, bypass time, age, weight, and open chest status. PPD patients had higher STAT category (p = 0.001), longer CPB times (p = 0.001), and higher VIS on POD 1-3 (p ≤ 0.005 daily). PPD patients also had higher AKI rates (p = 0.01) that did not reach significance in multivariable modeling. There were no postoperative deaths. Postoperative hours of intubation, hospital length of stay, and POD 1-5 fluid intake did not differ between groups. Over POD 1-5, PPD use accounted for 48.8 mL/kg increased fluid output (95% CI [2.2, 95.4], p = 0.043) and 3.41 mg/kg less furosemide administered (95% CI [1.69, 5.14], p < 0.001). No PPD complications were observed. Although PPD placement did not affect end-outcomes, it was used in higher acuity patients. PPD placement is associated with improved fluid output despite lower diuretic administration and may be a useful postoperative fluid management adjunct in some complex CHS patients.

Three-dimensional printing in congenital heart disease.

Three-dimensional printing (3Dp) has a broad array of medical applications and has been applied extensively in congenital heart disease given the variety and complexity of lesions encountered. 3Dps are unique when compared to other imaging modalities in that they are theoretically equally interpretable by radiologists, cardiologists and surgeons. The literature regarding 3Dp in congenital heart disease is recent and burgeoning. This review, categorized by application, should provide the reader with a comprehensive, albeit not complete, overview of 3Dp in congenital heart disease during the last decade.

Beyond 30 Days: Analysis of Unplanned Readmissions During the First Year Following Congenital Heart Surgery.

OBJECTIVE: We investigated the incidence and etiologies for unplanned hospital readmissions during the first year following congenital heart surgery (CHS) at our institution and the potential association of readmissions with longer term survival. METHODS: We retrospectively reviewed 263 patients undergoing CHS at our institution from August 2011 to June 2015. Scheduled readmissions were excluded. RESULTS: Seventy patients accrued a total of 120 readmissions (1.7 readmission/patient) within one year after surgery. The first readmission for 57% of the patients was within 30 days postdischarge. Twenty-two patients were first readmitted between 31 and 90 days postdischarge. Eight patients were first readmitted between 90 days and 1 year postdischarge. Median time-to-first readmission was 21 days. Median hospital length of stay at readmission was two days. Causes of 30-day readmissions included viral illness (25%), wound infections (15%), and cardiac causes (15%). Readmissions between 30 and 90 days included viral illness (27%), gastrointestinal (27%), and cardiac causes (9%). Age, STAT category, length of surgery, intubation, intensive care unit, and hospital stay were risk factors associated with readmissions based on logistic regression. Distance to hospital had a significant effect on readmissions (P < .001). Patients with higher family income were less likely to be readmitted (P < .001). There was no difference in survival between readmitted and non-readmitted patients (P = .68). CONCLUSIONS: The first 90 days is a high-risk period for unplanned hospital readmissions after CHS. Complicated postoperative course, higher surgical complexity, and lower socioeconomic status are risk factors for unplanned readmissions the first 90 days after surgery. Efforts to improve the incidence or readmission after CHS should extend to the first 3 months after surgery and target these high-risk patient populations.

Technical Performance Score Predicts Perioperative Outcomes in Complex Congenital Heart Surgery Performed in a Small-to-Medium-Volume Program.

As the quality of surgical outcomes depend on many factors, the development of validated tools to assess the different aspects of complex multidisciplinary teams' performance is crucial. The Technical Performance Score (TPS) has only been validated to correlate with outcomes in large-volume surgical programs. Here we assess the utility of TPS in correlation to perioperative outcomes for complex congenital heart surgeries (CHS) performed in a small-to-medium-volume program. 673 patients underwent CHS from 4/2012 to 12/2017 at our institution. Of those, 122 were STAT 4 and STAT 5. TPS was determined for each STAT 4 and STAT 5 operation using discharge echocardiogram: 1 = optimal, 2 = adequate, 3 = inadequate. Patient outcomes were compared including mortality, length of stay, ventilation times, and adverse events. 69 patients (57%) were neonates, 32 (26%) were infants, 17 (14%) were children, 4 (3%) were adults. TPS class 1 was assigned to 85 (70%) operations, TPS class 2 was assigned to 25 (20%) operations, and TPS class 3 was assigned to 12 (10%) operations. TPS was associated with re-intubation, ICU length of stay, postoperative length of stay, and mortality. TPS did not correlate with unplanned 30-day readmissions, need for reoperation, and inotropic score. Technical performance score was associated with perioperative outcomes and is a useful tool to assess the adequacy of repair for high complexity CHS in a small-to-medium-volume surgical program. TPS should be a part of program review in congenital heart programs of all sizes to identify strategies that may reduce postoperative morbidity and potentially improve long-term outcomes.

A unique indication for the senning procedure: Atrioventricular discordance with ventriculoarterial concordance.

OBJECTIVE: We describe experience treating a patient with atrioventricular (AV) discordance with ventriculoarterial (VA) concordance, ventricular septal defect (VSD), and situs inversus. This is a rare congenital lesion in which closing the VSD would septate D-transposition physiology and performing an arterial switch and VSD closure would produce congenitally corrected transposition of the great arteries physiology. As such, it is the only lesion for which an isolated atrial switch (and VSD closure) remains the preferred correction. CASE: A term baby girl born with AV discordance/VA concordance, a large VSD, and situs inversus totalis was palliated with atrial septostomy on day of life 3 to allow for optimal mixing and pulmonary artery banding during the 6th week of life to control symptoms of pulmonary overcirculation and protect the pulmonary vasculature. At 8 months of age, she underwent complete repair with a Senning atrial switch procedure, VSD closure, and pulmonary artery debanding with pulmonary arterioplasty. RESULTS: The patient underwent corrective surgery with patch closure of the VSD, and the Senning atrial switch procedure resulting in a total anatomic correction. The patient was discharged on postoperative day 6 and is clinically doing well 12 months later. Follow-up transthoracic echocardiogram shows no pulmonary venous baffle obstruction, mild systemic venous baffle obstruction, and moderate pulmonary stenosis/pulmonary insufficiency. CONCLUSION: The isolated atrial switch is rarely employed as its outcomes are inferior to the arterial switch operation in the setting of the dextro-transposition of the great arteries. However, it remains the procedure of choice for AV discordance with VA concordance as it leads to physiologically corrected biventricular circulation.