Child Opportunity Index and Pediatric Extracorporeal Membrane Oxygenation Outcomes; the Role of Diagnostic Category.

OBJECTIVES: To study the impact of social determinants of health (SDoH) on pediatric extracorporeal membrane oxygenation (ECMO) outcomes. DESIGN, SETTING, AND PATIENTS: Retrospective study of children (< 18 yr) supported on ECMO (October 1, 2015 to March 1, 2021) using Pediatric Health Information System (44 U.S. children's hospitals). Patients were divided into five diagnostic categories: neonatal cardiac, pediatric cardiac, neonatal respiratory, pediatric respiratory, and sepsis. SDoH included the Child Opportunity Index (COI; higher indicates social advantage), race, ethnicity, payer, and U.S. region. Children without COI were excluded. Diagnostic category-specific clinical variables related to baseline health and illness severity were collected. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children supported on ECMO experienced a 33% in-hospital mortality (2863/8710). Overall, children with lower COI, "other" race, Hispanic ethnicity, public insurance and from South or West regions had greater mortality. Associations between SDoH and ECMO outcomes differed between diagnostic cohorts. Bivariate analyses found that only pediatric cardiac patients had an association between COI or race and mortality. Multivariable logistic regression analyses examined relationships between SDoH, clinical variables and mortality within diagnostic categories. Pediatric cardiac patients had 5% increased odds of death (95% CI, 1.01-1.09) for every 10-point decrement in COI, while Hispanic ethnicity was associated with higher survival (adjusted odds ratio [aOR] 0.72 [0.57-0.89]). Children with heart disease from the highest COI quintile had less cardiac-surgical complexity and earlier cannulation. Independent associations with mortality were observed in sepsis for Black race (aOR 1.62 [1.06-2.47]) and other payer in pediatric respiratory patients (aOR 1.94 [1.23-3.06]). CONCLUSIONS: SDoH are statistically associated with pediatric ECMO outcomes; however, associations differ between diagnostic categories. Influence of COI was observed only in cardiac patients while payer, race, and ethnicity results varied. Further research should investigate differences between diagnostic cohorts and age groups to understand drivers of inequitable outcomes.

Perioperative Hyper-coagulation and Thrombosis: Cost Analysis After Congenital Heart Surgery.

Thrombosis, a major adverse event of congenital heart surgery, has been associated with poor outcomes. We hypothesized that in CHD patients undergoing cardiac surgery, increased perioperative use of pro-coagulant products may be associated with postoperative thrombosis in the setting of hyperfibrinogenemia, leading to greater hospital and blood product costs. Single-center retrospective study. Data from Boston Children's Hospital's electronic health record database was used in this study. All patients undergoing congenital heart surgery between 2015 and 2018 with postoperative fibrinogen levels above 400 mg/dl were reviewed. Of 334 patients with high plasma fibrinogen levels, 28 (8.4%) developed postoperative thrombosis (median age: one year, 59% male). In our cohort, 25 (7%) demonstrated evidence of baseline hypercoagulability by one or more panel test results. Thrombosis was associated with greater hospital and blood product costs, longer ventilation times, and longer hospital and ICU length of stays. Preoperative hypercoagulable state (odds ratio: 2.58, 95% CI [1.07, 9.99], p = 0.002), postoperative red blood cell transfusion (odds ratio: 1.007, 95% CI [1.000, 1.015], p = 0.04), and single ventricle physiology (univariate odds ratio: 2.94, 95% CI [1.09, 7.89], p = 0.03) were predictors of postoperative thrombosis. Preoperative hypercoagulable state and intraoperative platelet transfusion were predictors of hospital cost. Thrombosis was associated with worse in-hospital outcomes and higher costs. Preoperative hypercoagulable state and postoperative red blood cell transfusion were significant predictors of thrombosis. Risk prediction models that can guide thrombosis prevention are needed to improve outcomes of patients undergoing congenital heart surgery.

Postoperative Troponin Levels in Children Undergoing Open Heart Surgery With and Without Coronary Intervention.

We aimed to characterize the ranges, temporal trends, influencing factors, and prognostic significance of postoperative troponin levels after congenital heart surgery. This single-center retrospective study included patients from 2006 to 2021 who had ≥ 1 postoperative troponin-T measurement collected within 96 h of congenital heart surgery (CHS). Patients were grouped as Anomalous Aortic Origin of the Coronary Artery-"AAOCA repair," or congenital heart surgery with "Other Coronary Interventions" other than AAOCA repair, or "No Coronary Intervention." In each group, information on concomitant surgery requiring one or more of the following-atriotomy, ventriculotomy, right ventricular muscle bundle resection, and/or septal myectomy-was collected. Clinical correlates of troponin values were analyzed in three postoperative windows: < 8, 8-24, and 24-48 h. The highest median [range] troponin levels (ng/mL) for the samples were 0.34 [0.06, 1.32] at < 8 h for "AAOCA repair," 1.35 [0.14, 12.0] at < 8 h for those undergoing CHS with "Other Coronary Interventions," and 0.87 [0.06, 25.1] at 8-24 h for those undergoing CHS with "No Coronary Interventions." Atriotomy was associated with higher median troponin levels in the AAOCA group at < 8 h (0.40 [0.31, 0.77] vs. 0.29 [0.17, 0.54], P = 0.043) and in the Other Coronary Intervention group at 8-24 h (1.67 [1.04, 2.63] vs. 0.40 [0.19, 1.32], P = 0.002). Patients experiencing major postoperative complications (vs. those who did not) had higher troponin levels in the AAOCA group as early as 8-24 h (0.36 [0.24, 0.57] vs. 0.21 [0.14, 0.33], P = 0.03). Similar findings were noted in the Coronary Intervention (2.20 [1.34, 3.90] vs. 1.11 [0.51, 2.90], P = 0.028) and No Coronary Intervention (2.2 [1.49, 15.1] vs. 0.74 [0.40, 2.34], P = 0.027) groups but earlier at < 8 h. In the AAOCA group, 2/18 (11%) troponin outliers experienced cardiac arrest in comparison to 0/80 (0%) non-outliers (P = 0.032). In the Other Coronary Intervention group, troponin outliers had longer median times to ICU discharge (10 vs. 4 days) and hospital discharge (21 vs. 10 days) (both P < 0.001). Postoperative troponin levels depend on a multitude of factors and may have prognostic value in patients undergoing congenital heart surgery with coronary interventions.

Risk Factors for Adverse Outcomes in Term Infants with CHD and Definitive Necrotising Enterocolitis.

OBJECTIVES: To define the incidence of definitive necrotising enterocolitis in term infants with CHD and identify risk factors for morbidity/mortality. METHODS: We performed a 20-year (2000-2020) single-institution retrospective cohort study of term infants with CHD admitted to the Boston Children's Hospital cardiac ICU with necrotising enterocolitis (Bell's stage ≥ II). The primary outcome was a composite of in-hospital mortality and post-necrotising enterocolitis morbidity (need for extracorporeal membrane oxygenation, multisystem organ failure based on the paediatric sequential organ failure assessment score, and/or need for acute gastrointestinal intervention). Predictors included patient characteristics, cardiac diagnosis/interventions, feeding regimen, and severity measures. RESULTS: Of 3933 term infants with CHD, 2.1% (n = 82) developed necrotising enterocolitis, with 67% diagnosed post-cardiac intervention. Thirty (37%) met criteria for the primary outcome. In-hospital mortality occurred in 14 infants (17%), of which nine (11%) deaths were attributable to necrotising enterocolitis. Independent predictors of the primary outcome included moderate to severe systolic ventricular dysfunction (odds ratio 13.4,confidence intervals 1.13-159) and central line infections pre-necrotising enterocolitis diagnosis (odds ratio 17.7, confidence intervals 3.21-97.0) and mechanical ventilation post-necrotising enterocolitis diagnosis (odds ratio 13.5, confidence intervals 3.34-54.4). Single ventricle, ductal dependency, and feeding related factors were not independently associated with the primary outcome. CONCLUSIONS: The incidence of necrotising enterocolitis was 2.1% in term infants with CHD. Adverse outcomes occurred in greater than 30% of patients. Presence of systolic dysfunction and central line infections prior to diagnosis and need for mechanical ventilation after diagnosis of necrotising enterocolitis can inform risk triage and prognostic counseling for families.

Echocardiographic image collection and evaluation in infants with CHD: lessons learned from the imaging core lab for the Residual Lesion Score study.

Many factors affect patient outcome after congenital heart surgery, including the complexity of the heart disease, pre-operative status, patient specific factors (prematurity, nutritional status and/or presence of comorbid conditions or genetic syndromes), and post-operative residual lesions. The Residual Lesion Score is a novel tool for assessing whether specific residual cardiac lesions after surgery have a measurable impact on outcome. The goal is to understand which residual lesions can be tolerated and which should be addressed prior to leaving the operating room. The Residual Lesion Score study is a large multicentre prospective study designed to evaluate the association of Residual Lesion Score to outcomes in infants undergoing surgery for CHD. This Pediatric Heart Network and National Heart, Lung, and Blood Institute-funded study prospectively enrolled 1,149 infants undergoing 5 different congenital cardiac surgical repairs at 17 surgical centres. Given the contribution of echocardiographic measurements in assigning the Residual Lesion Score, the Residual Lesion Score study made use of a centralised core lab in addition to site review of all data. The data collection plan was designed with the added goal of collecting image quality information in a way that would permit us to improve our understanding of the reproducibility, variability, and feasibility of the echocardiographic measurements being made. There were significant challenges along the way, including the coordination, de-identification, storage, and interpretation of very large quantities of imaging data. This necessitated the development of new infrastructure and technology, as well as use of novel statistical methods. The study was successfully completed, but the size and complexity of the population being studied and the data being extracted required more technologic and human resources than expected which impacted the length and cost of conducting the study. This paper outlines the process of designing and executing this complex protocol, some of the barriers to implementation and lessons to be considered in the design of future studies.

Contemporary Socioeconomic and Childhood Opportunity Disparities in Congenital Heart Surgery.

BACKGROUND: While singular measures of socioeconomic status have been associated with outcomes after surgery for congenital heart disease, the multifaceted pathways through which a child's environment impacts similar outcomes remain incompletely characterized. We sought to evaluate the association between childhood opportunity level and adverse outcomes after congenital heart surgery. METHODS: Data from patients undergoing congenital cardiac surgery from January 2011 to January 2020 at a quaternary referral center were retrospectively reviewed. Outcomes of interest included predischarge (early) mortality or transplant, postoperative hospital length-of-stay, inpatient cost of hospitalization, postdischarge (late) mortality or transplant, and late unplanned reintervention. The primary predictor was a US census tract-based, nationally-normed composite metric of contemporary child neighborhood opportunity comprising 29 indicators across 3 domains (education, health and environment, and socioeconomic), categorized as very low, low, moderate, high, and very high. Associations between childhood opportunity level and outcomes were evaluated using logistic regression (early mortality), generalized linear (length-of-stay and cost), Cox proportional hazards (late mortality), or competing risk (late reintervention) models, adjusting for baseline patient-related factors, case complexity, and residual lesion severity. RESULTS: Of 6133 patients meeting entry criteria, the median age was 2.0 years (interquartile range, 3.6 months-8.3 years). There were 124 (2.0%) early deaths or transplants, the median postoperative length-of-stay was 7 days (interquartile range, 5-13 days), and the median inpatient cost was $76 000 (interquartile range, $50 000-130 000). No significant association between childhood opportunity level and early mortality or transplant was observed (P=0.21). On multivariable analysis, children with very low and low opportunity had significantly longer length-of-stay and incurred higher costs compared with those with very high opportunity (all P<0.05). Of 6009 transplant-free survivors of hospital discharge, there were 175 (2.9%) late deaths or transplants, and 1008 (16.8%) reinterventions at up to 10.5 years of follow-up. Patients with very low opportunity had a significantly greater adjusted risk of late death or transplant (hazard ratio, 1.7 [95% CI, 1.1-2.6]; P=0.030) and reintervention (subdistribution hazard ratio, 1.9 [95% CI, 1.5-2.3]; P<0.001), versus those with very high opportunity. CONCLUSIONS: Childhood opportunity level is independently associated with adverse outcomes after congenital heart surgery. Children from resource-limited settings thus constitute an especially high-risk cohort that warrants closer surveillance and tailored interventions.

Implications of Transfusion in Adults with Congenital Heart Disease Undergoing Cardiac Surgery.

The number of adults with congenital heart disease (CHD) requiring cardiovascular (CV) surgery is increasing rapidly in today's era. We hypothesized that exposure to perioperative blood products is associated with worse outcomes in adults. All adults (≥ 18 years old) undergoing CV surgery with Cardio-Pulmonary Bypass (CPB) between 2015 and 2020 were reviewed retrospectively. Associations between transfusion and outcomes were studied by univariable logistic regression and Wilcoxon rank sum tests. Cox/ logistic regression was used to assess (a) postoperative ventilation time and length of stay, and (b) major complications, respectively. Of 323 patients, 170 (53%) received blood products perioperatively. The median age was 27 (interquartile range [IQR]: 22-36) years, there were 181 (46%) males, and 16 (5%) patients had single ventricle anatomy. Patients receiving products experienced more complications (OR: 6.6, 95% CI: [2.9, 14.7], p < 0.001) specifically, cardiac arrest (OR: 8.8, 95% CI: [1.1, 71.9], p = 0.04). Transfusion was associated with greater frequency of thrombosis ((OR: 7.8, 95% CI: [1.8, 34.7], p = 0.01)), longer ventilation time (HR: 3.0, 95% CI: [2.4, 3.9], p < 0.001), and longer hospital length of stay (HR: 2.7, 95% CI: [2.1, 3.4], p < 0.001). Longer CPB time (OR: 1.0, 95% CI: [1.0, 1.1], p < 0.001) and prior cardiac surgery (OR: 1.6, 95% CI: [1.3, 2.1], p < 0.001) were independent predictors of perioperative blood product transfusion. Adults who received perioperative blood products experienced more complications and worse in-hospital outcomes. Future research on optimizing blood product transfusion based on risk prediction is needed to optimize outcomes in adults with CHD.

Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy.

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) typically presents in infancy; however, there are cases of patients who survive the infant period and present later in life. We aimed to characterize patients with late ALCAPA diagnoses and to assess perioperative and functional outcomes. A retrospective chart review of patients who underwent ALCAPA repair between 1996 and 2020 at Boston Children's Hospital was performed. This cohort was divided into early ALCAPA (< 1 year) and late ALCAPA (≥ 1 year) groups. Perioperative data were collected. Longitudinal functional assessments were made by echocardiography, exercise stress test, and cardiac magnetic resonance imaging. The median age of the late ALCAPA group was 7.6 years with 25% (6/24) of patients over 18 years. The late ALCAPA group was more likely to present as an incidental finding (63%) and required less preoperative intervention compared to the early group. On preoperative echocardiogram, the late ALCAPA group had less moderate or severe mitral regurgitation (16.7% vs 62%, p < 0.001) or left ventricular dysfunction (16.7% vs 89%, p < 0.001) compared to the early group. Reoperation was uncommon, and both groups demonstrated almost complete resolution of mitral regurgitation and left ventricular dysfunction over time. There are important differences between late and early ALCAPA subtypes. Revascularization results in excellent outcomes in both early and late groups, but long-term surveillance of ALCAPA patients is warranted as they may have functional deficits after repair.

Impact of Residual Lesion Severity on Neurodevelopmental Outcomes Following Congenital Heart Surgery in Infancy and Childhood.

Children with congenital heart disease are at increased risk of neurodevelopmental delay throughout their lifespan. This risk is exacerbated following congenital heart surgery (CHS) in infancy. However, there are few modifiable risk factors for postoperative neurodevelopmental delay. In this study, we assessed the Residual Lesion Score (RLS), a quality assessment metric that evaluates residual lesion severity following CHS, as a predictor of neurodevelopmental delay. This was a single-center, retrospective review of patients who underwent CHS from 01/2011 to 03/2021 and post-discharge neurodevelopmental evaluation from 12 to 42 months of age using the Bayley Scales of Infant Development, 3rd Edition (BSID-III). RLS was assigned per published criteria: RLS 1, no residua; RLS 2, minor residua; and RLS 3, major residua or pre-discharge reintervention. Associations between RLS and BSID-III scores, as well as trends in neurodevelopmental outcomes over time, were evaluated. Of 517 patients with median age at neurodevelopmental testing of 20.0 (IQR 18.0-22.7) months, 304 (58.8%), 146 (28.2%), and 67 (13.0%) were RLS 1, 2, and 3, respectively. RLS 3 patients had significantly lower scaled scores in the cognitive, receptive, and expressive communication, and fine and gross motor domains, compared with RLS 1 patients. Multivariable models accounted for 21.5%-31.5% of the variation in the scaled scores, with RLS explaining 1.4-7.3% of the variation. In a subgroup analysis, RLS 3 patients demonstrated relatively fewer gains in cognitive, expressive communication, and gross motor scores over time (all p < 0.05). In conclusion, RLS 3 patients are at increased risk for neurodevelopmental delay, warranting closer follow-up and greater developmental support for cognitive, language, and motor skills soon after surgery.

Post-operative vomiting and enhanced recovery after congenital cardiac surgery.

BACKGROUND: Post-operative nausea and vomiting is frequent after congenital cardiac surgery. AIMS: We sought to determine factors associated to severe post-operative vomiting after congenital cardiac surgery and the effect on post-operative outcomes. METHODS: Patients > 30 days of age who underwent elective cardiac surgical repair as part of an enhanced recovery after congenital cardiac surgery programme were retrospectively reviewed. Patient characteristics and perioperative factors were compared by univariate analysis for patients with severe post-operative vomiting, defined as three events or more, and for patients with no-or-mild post-operative vomiting. All variables with a p-value < 0.1 were included in a multivariable model, and major post-operative outcomes were compared using regression analysis. RESULTS: From 1 October, 2018 to 30 September, 2019, 430 consecutive patients were included. The median age was 4.8 years (interquartile range 1.2-12.6). Twenty-one per cent of patients (91/430) experienced severe post-operative vomiting. Total intraoperative opioids > 5.0 mg/kg of oral morphine equivalent (adjusted odds ratio 1.72) and post-operative inotropes infusion(s) (adjusted odds ratio 1.64) were identified as independent predictors of severe post-operative vomiting after surgery. Patients suffering from severe post-operative vomiting had increased pulmonary complications (adjusted odds ratio 5.18) and longer post-operative hospitalisation (adjusted coefficient, 0.89). CONCLUSIONS: Greater cumulative intraoperative opioids are associated with severe post-operative vomiting after congenital cardiac surgery. Multimodal pain strategies targeting the reduction of intraoperative opioids should be considered during congenital cardiac surgery to enhance recovery after surgery.

Clinical implications of acute shunt thrombosis in paediatric patients with systemic-to-pulmonary shunt re-interventions.

PURPOSE: Systemic-to-pulmonary shunts are used as a source of pulmonary blood flow in palliated Congenital Heart Disease in neonates and young infants. Shunt thrombosis, often requiring shunt interventions during index hospitalisation, is associated with poor outcomes. We hypothesised that extensive use of perioperative pro-coagulant products may be associated with shunt thrombosis. METHODS: Children (≤18 years) undergoing systemic-to-pulmonary shunts with in-hospital shunt reinterventions between 2016 and 2020 were reviewed retrospectively. Perioperative associations to shunt thrombosis were examined by univariate logistic regression and Wilcoxon rank sum tests as appropriate. Cox and log transformed linear regression were used to analyse postoperative ventilation duration, length of stay, and cost. RESULTS: Of 71 patients requiring in-hospital shunt intervention after systemic-to-pulmonary shunts, 10 (14%) had acute shunt thrombosis, and among them five (50%) died. The median age was four (interquartile range: 0-15) months. There were 40 (56%) males, 41 (58%) had single ventricle anatomy, and 29 (40%) were on preoperative anticoagulants. Patients with acute shunt thrombosis received greater volume of platelets (p = 0.04), cryoprecipitate (p = 0.02), and plasma (p = 0.04) postoperatively in the ICU; experienced more complications (p = 0.01) including re-exploration for bleeding (p = 0.008) and death (p = 0.02), had longer hospital length of stays (p = 0.004), greater frequency of other arterial/venous thrombosis (p = 0.02), and greater hospital costs (p = 0.002). CONCLUSIONS: Patients who develop acute shunt thrombosis receive more blood products perioperatively and experience worse hospital outcomes and higher hospital costs. Future research on prevention/early detection of shunt thrombosis is needed to improve outcomes in infants after systemic-to-pulmonary shunt surgery.

Pre-Glenn aorto-pulmonary collaterals in single-ventricle patients.

BACKGROUND: In single-ventricle patients undergoing staged-bidirectional Glenn, 36-59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention. METHODS: Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes. RESULTS: From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17-11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33-3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08-1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37-3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32-2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21-5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08-1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75-0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65-0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63-0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months). CONCLUSIONS: Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.

Impact of pre-bypass ultrafiltration on prime values and clinical outcomes in neonatal and infant cardiopulmonary bypass.

BACKGROUND: A standard blood prime for cardiopulmonary bypass (CPB) in congenital cardiac surgery may possess non-physiologic values for electrolytes, glucose, and lactate. Pre-bypass Ultrafiltration (PBUF) can make these values more physiologic and standardized prior to bypass initiation. We aimed to determine if using PBUF on blood primes including packed red blood cells and thawed plasma would make prime values more predictable and physiologic. Additionally, we aimed to evaluate whether the addition of PBUF had an impact on outcome measures. METHODS: Retrospective review of consecutive patients ≤ 1 year of age undergoing an index cardiac operation on CPB between 8/2017 and 9/2021. As PBUF was performed at the perfusionists' discretion, a natural grouping of patients that received PBUF vs. those that did not occur. Differences in electrolytes, glucose, and lactate were compared at specific time points using Fisher's exact test for categorical variables and the Wilcoxon rank sum test for continuous variables. Clinical outcomes were also assessed. RESULTS: In both cohorts, the median age at surgery was 3 months and 47% of patients were female; 308/704 (44%) of the PBUF group and 163/414 (39%) of the standard prime group had at least one preoperative risk factor. The proportion of PBUF circuits which demonstrated more physiologic values for glucose (318 [45%]), sodium (434, [62%]), potassium (688 [98%]), lactate (612 [87%]) and osmolality (595 [92%]) was significantly higher when compared to standard prime circuit levels for glucose (8 [2%]), sodium (13 [3%], potassium (150 [36%]), lactate (56 [13%]) and osmolality (23 [6%]) prior to CPB initiation. There were no differences in clinical outcomes or rates of major adverse events between the two cohorts. CONCLUSIONS: PBUF creates standardized and more physiologic values for electrolytes, glucose, and lactate before the initiation of bypass without significant impacts on in-hospital outcomes.

Digital solution for follow-up in congenital cardiac surgery.

BACKGROUND: In this era of public scrutiny, there is an ongoing need for innovative methods for patient follow-up. OBJECTIVES: As part of a quality initiative, we developed an automated post-operative follow-up system for patients following discharge after cardiac surgery at Boston Children's Hospital. METHODS: Discharge Communication (DisCo) is a web-based system developed at Boston Children's Hospital. An automated text and e-mail with a link to a health status survey are sent at 30 days and 1 year post-discharge in English/Spanish. If there is no response, surveys are completed via phone calls to the patient/patient's physician or chart review. Responses are stored in the DisCo database and the patient's medical record. Patients who underwent cardiac surgery and survived to hospital discharge from October, 2016 received the surveys. RESULTS: Overall, 3345 30-day and 2563 1-year surveys were sent between October, 2016 and June, 2020. Of 3345 30-day surveys, there were 3191 responses (95%). Of 2563 1-year surveys, there were 1807 responses (71%). Most patients/families responded directly to the link at 30 days (65% for paediatrics/75% for adults) and at 1 year (72% for paediatrics/78% for adults). Multi-variable logistic regression revealed that higher complexity of cardiac lesion, presence of major non-cardiac anomalies and presence of major residua were associated with readmission and catheter/surgical reinterventions. Non-cardiac anomalies were associated with increased need for services for learning, development or behaviour. CONCLUSIONS: DisCo provides a successful web-based health status assessment of patients following congenital cardiac surgery. It helps to identify high-risk patients who need closer follow-up.

Development of the Residual Lesion Score for congenital heart surgery: the RAND Delphi methodology.

BACKGROUND AND OBJECTIVE: The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology. METHODS: A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the "final" Residual Lesion Score. RESULTS: The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the "final" Residual Lesion Score. CONCLUSION: Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network's Residual Lesion Score study.

Comparison of Thromboelastography Devices TEG®6S Point of Care Device vs. TEG®5000 in Pediatric Patients Undergoing Cardiac Surgery.

Thromboelastography (TEG) can predict bleeding in pediatric patients undergoing cardiac surgery. We hypothesized that results obtained from TEG®5000 correlate with the new point-of-care TEG®6S system and that TEG®6S rewarming maximum amplitude (MA) is associated with surrogate endpoints for perioperative bleeding in pediatric patients who underwent complex cardiac surgery. We describe a retrospective study of pediatric (≤18 years) patients who underwent complex cardiac surgery on cardiopulmonary bypass. Citrate whole-blood samples were used to compared TEG®5000 vs.TEG®6S and TEG®6S-FLEV (with fibrinogen measurement) vs. Clauss-fibrinogen methods. TEG®6S parameters obtained during rewarming were compared to the surrogate endpoints for perioperative bleeding using linear regression analysis. Among 100 patients, 225 TEG®5000 vs.TEG®6S comparisons and 54 TEG®6S-FLEV were analyzed. Good correlation was observed for all parameters comparing TEG®5000 to TEG®6S and TEG®6S-FLEV to the Clauss-fibrinogen method (Pearson r ≥ .7). Similar to rewarming TEG®5000 MA, rewarming TEG®6S MA was the only parameter independently associated with risk for perioperative bleeding (median [interquartile range {IQR}] in bleeding vs. nonbleeding patients: 35 [29, 48] vs. 37 [32, 55]; p = .02). A platelet transfusion calculator was developed based on TEG®6S results by determining the relationship between platelet transfusion volume (mL/kg) and percent change in MA using linear regression analysis. TEG®6S is a good alternative point-of-care method to analyze a patient's coagulation profile and it is comparable to TEG®5000 in pediatric patients undergoing cardiac surgery on cardiopulmonary bypass. Lower TEG®6S MA during rewarming is associated with increased risk for perioperative bleeding. TEG analysis during rewarming may be useful in customizing platelet transfusion therapy by reducing the risk of bleeding while minimizing excessive blood product transfusions.

Management of Complex Left Ventricular Outflow Tract Obstruction: A Comparison of Konno and Modified Konno Techniques.

Management of complex left ventricular outflow tract obstruction (LVOTO) can be achieved with a Konno or Modified Konno procedure to enlarge the LVOT. We hypothesized that patients who undergo a Modified Konno procedure would have a higher rate of LVOT re-intervention compared to the Konno procedure. Patients who underwent a Konno or Modified Konno procedure for LVOTO at a single tertiary care center between 1990 and 2014 were retrospectively reviewed. The primary outcome was LVOT re-intervention post-discharge from index Konno or Modified Konno procedure. Cox regression and Kaplan-Meier estimates were used for time-to-event analysis of LVOT re-interventions, any unplanned re-interventions, and transplant-free survival. The study included 122 patients: 51 (41.8%) in the Konno group and 71 (58.2%) in the Modified Konno group. Median age at surgery was 8.2 (IQR 3-16) years in the Konno group and 3.9 (IQR 1.5-11) years in the Modified Konno group. Multiple left heart lesions were less prevalent in Modified Konno patients. There were 36 (29.5%) patients with LVOT re-interventions: 8 (16%) in the Konno group and 28 (39.4%) in the Modified Konno group (p = 0.01). Transplant-free survival at five years was 87.2% for the Konno group and 93.5% for the Modified Konno group. A higher rate of LVOT re-intervention was found in the Modified Konno group although the Konno and Modified Konno techniques were applied to different patient populations. This finding suggests that careful preoperative decision-making can direct therapy appropriately and that fundamental diagnosis affects procedure choice.

Better preoperative exercise function is associated with shorter hospital stay after paediatric pulmonary valve replacement or conduit revision.

Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored. METHODS: Single centre retrospective cohort study of patients aged 8-18 years who underwent surgical pulmonary valve replacement. The primary predictor was preoperative percent predicted peak oxygen consumption, and primary outcome was total hospital length of stay. Clinical, imaging, and cardiopulmonary exercise test data were reviewed and compared to total hospital length of stay. Cox proportional hazards regression was used to examine the association between total hospital length of stay and percent predicted peak oxygen consumption. RESULTS: Three-hundred and seventy patients undergoing pulmonary valve replacement/conduit change between 2003 and 2017 at Boston Children's Hospital were identified. Ninety had preoperative cardiopulmonary exercise tests within 6 months of surgery. Exclusion for inadequate exercise data (n = 3) and imaging data (n = 1) left 86 patients for review. Patients with percent predicted peak oxygen consumption ≥ 70% (n = 46, 53%) had shorter total hospital length of stay (4.4 days) than the 40 with percent predicted peak oxygen consumption <70% (5.4 days, p = 0.007). Median percent predicted peak oxygen consumption increased over sequential surgical eras (p < 0.001), but total hospital length of stay did not correlate with surgical era, preoperative left ventricular function, or preoperative right ventricular dilation. CONCLUSION: Children undergoing surgical pulmonary valve replacement with better preoperative exercise capacity had shorter total hospital length of stay. Exercise capacity is a potentially modifiable factor prior to and after pulmonary valve replacement. Until more patients systematically undergo cardiopulmonary exercise tests, the full impact of optimisation of exercise capacity will not be known.

Hyperlactataemia as a predictor of adverse outcomes post-cardiac surgery in neonates with congenital heart disease.

OBJECTIVE: To evaluate the discriminative ability of hyperlactataemia for early morbidity and mortality in neonates with CHD following cardiac surgery. METHODS: Retrospective, observational study of neonates who underwent cardiac surgery on cardiopulmonary bypass at a tertiary care children's hospital from June 2015 to June 2019. The primary predictor was lactate. The primary composite outcome was defined as ≥1 of the following: cardiac arrest or extracorporeal membrane oxygenation within 72 hours or 30-day mortality post-operatively. The secondary outcome was the presence of major residual lesions, according to the Technical Performance Score. RESULTS: Of 432 neonates, 28 (6.5%) sustained the composite outcome. On univariate analysis, peak lactate within 48 hours, increase in lactate from ICU admission through 12 hours, and single ventricle physiology were significantly associated with the composite outcome. The peak lactate occurred at a median of 2.9 hours (interquartile range: 1, 35) before the event. Through multi-variable analysis, a multi-variable risk algorithm was created. Predicted probabilities demonstrated an increasing risk based on single ventricle status and delta lactate, ranging from 1.8% (95% CI: 0.9, 3.9) to 52.4% (95% CI: 32.4, 71.7). The model had good discriminative ability for the composite outcome on receiver operating characteristic analysis (area under the curve = 0.79; 95% CI: 0.75, 0.89). Moreover, a peak lactate of 7.3 mmol/l or greater was significantly associated with the presence of a major residual lesion (odds ratios: 5.16, 95% CI: 3.01, 8.87). CONCLUSIONS: We present a simple, two-variable model, including delta lactate in the immediate post-operative period and single ventricle status, to prognosticate the risk of early morbidity and mortality in neonates undergoing cardiac surgery for potential intervention.

Registry-based trials: a potential model for cost savings?

BACKGROUND/AIMS: Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials. METHODS: We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design. RESULTS: Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field. CONCLUSIONS: Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.