Inpatient Resource Utilization for Hypoplastic Left Heart Syndrome from Birth Through Fontan.

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.

Validation of Automated Data Extraction From the Electronic Medical Record to Provide a Pediatric Risk Assessment Score.

BACKGROUND: Although the rate of pediatric postoperative mortality is low, the development and validation of perioperative risk assessment models have allowed for the stratification of those at highest risk, including the Pediatric Risk Assessment (PRAm) score. The clinical application of such tools requires manual data entry, which may be inaccurate or incomplete, compromise efficiency, and increase physicians' clerical obligations. We aimed to create an electronically derived, automated PRAm score and to evaluate its agreement with the original American College of Surgery National Surgical Quality Improvement Program (ACS NSQIP)-derived and validated score. METHODS: We performed a retrospective observational study of children <18 years who underwent noncardiac surgery from 2017 through 2021 at Boston Children's Hospital (BCH). An automated PRAm score was developed via electronic derivation of International Classification of Disease (ICD) -9 and -10 codes. The primary outcome was agreement and correlation among PRAm scores obtained via automation, NSQIP data, and manual physician entry from the same BCH cohort. The secondary outcome was discriminatory ability of the 3 PRAm versions. Fleiss Kappa, Spearman correlation (rho), and intraclass correlation coefficient (ICC) and receiver operating characteristic (ROC) curve analyses with area under the curve (AUC) were applied accordingly. RESULTS: Of the 6014 patients with NSQIP and automated PRAm scores (manual scores: n = 5267), the rate of 30-day mortality was 0.18% (n = 11). Agreement and correlation were greater between the NSQIP and automated scores (rho = 0.78; 95% confidence interval [CI], 0.76-0.79; P <.001; ICC = 0.80; 95% CI, 0.79-0.81; Fleiss kappa = 0.66; 95% CI, 0.65-0.67) versus the NSQIP and manual scores (rho = 0.73; 95% CI, 0.71-0.74; P < .001; ICC = 0.78; 95% CI, 0.77-0.79; Fleiss kappa = 0.56; 95% CI, 0.54-0.57). ROC analysis with AUC showed the manual score to have the greatest discrimination (AUC = 0.976; 95% CI, 0.959,0.993) compared to the NSQIP (AUC = 0.904; 95% CI, 0.792-0.999) and automated (AUC = 0.880; 95% CI, 0.769-0.999) scores. CONCLUSIONS: Development of an electronically derived, automated PRAm score that maintains good discrimination for 30-day mortality in neonates, infants, and children after noncardiac surgery is feasible. The automated PRAm score may reduce the preoperative clerical workload and provide an efficient and accurate means by which to risk stratify neonatal and pediatric surgical patients with the goal of improving clinical outcomes and resource utilization.

Cardiac arrest and cardiopulmonary resuscitation in pediatric patients with cardiac disease: a narrative review.

Children with cardiac disease are at a higher risk of cardiac arrest as compared to healthy children. Delivering adequate cardiopulmonary resuscitation (CPR) can be challenging due to anatomic characteristics, risk profiles, and physiologies. We aimed to review the physiological aspects of resuscitation in different cardiac physiologies, summarize the current recommendations, provide un update of current literature, and highlight knowledge gaps to guide research efforts. We specifically reviewed current knowledge on resuscitation strategies for high-risk categories of patients including patients with single-ventricle physiology, right-sided lesions, right ventricle restrictive physiology, left-sided lesions, myocarditis, cardiomyopathy, pulmonary arterial hypertension, and arrhythmias. Cardiac arrest occurs in about 1% of hospitalized children with cardiac disease, and in 5% of those admitted to an intensive care unit. Mortality after cardiac arrest in this population remains high, ranging from 30 to 65%. The neurologic outcome varies widely among studies, with a favorable neurologic outcome at discharge observed in 64%-95% of the survivors. Risk factors for cardiac arrest and associated mortality include younger age, lower weight, prematurity, genetic syndrome, single-ventricle physiology, arrhythmias, pulmonary arterial hypertension, comorbidities, mechanical ventilation preceding cardiac arrest, surgical complexity, higher vasoactive-inotropic score, and factors related to resources and institutional characteristics. Recent data suggest that Extracorporeal membrane oxygenation CPR (ECPR) may be a valid strategy in centers with expertise. Overall, knowledge on resuscitation strategies based on physiology remains limited, with a crucial need for further research in this field. Collaborative and interprofessional studies are highly needed to improve care and outcomes for this high-risk population. What is Known: • Children with cardiac disease are at high risk of cardiac arrest, and cardiopulmonary resuscitation may be challenging due to unique characteristics and different physiologies. • Mortality after cardiac arrest remains high and neurologic outcomes suboptimal. What is New: • We reviewed the unique resuscitation challenges, current knowledge, and recommendations for different cardiac physiologies. • We highlighted knowledge gaps to guide research efforts aimed to improve care and outcomes in this high-risk population.

Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain.

BACKGROUND: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH. METHODS: Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram. RESULTS: Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was - 14.9 ± 5.6% at baseline and improved to - 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was - 19.5 ± 5.4% at baseline and improved to - 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge. CONCLUSION: PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter.

The Role of Chronic Conditions in Outcomes following Noncardiac Surgery in Children with Congenital Heart Disease.

OBJECTIVES: To compare outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery by presence of chronic conditions and identify associated risk factors. STUDY DESIGN: Retrospective analysis of 14 031 children with CHD who underwent noncardiac surgery in the 2016 Healthcare Cost and Utilization Project Kid's Inpatient Database. Multivariable regression was used to assess patient and hospital factors associated with in-hospital mortality and length of stay (LOS). RESULTS: Overall, 94% had at least 1 chronic condition. The in-hospital mortality rate was 5.6%. Neonates with CHD only had the highest mortality (15.6%); otherwise, children with CHD and at least 1 chronic condition had higher mortality than patients with CHD only (infant 3.93%, child 1.22%, adolescent 1.04% vs 2.34%, 0%, and 0%). Neonates (OR, 15.5; 95% CI, 7.1-34.1 vs adolescent), number of chronic conditions (OR, 1.34; 95% CI, 1.27-1.42), chronic conditions type (circulatory system; OR 2.46; 95% CI, 2.04-2.98), and low socioeconomic status (OR, 1.36; 95% CI, 1.05-1.77) were associated with increased mortality. The median LOS was 20 days (IQR, 5-66). Those with CHD and at least 1 chronic condition had a greater LOS (21 days; IQR, 5-68) than those with CHD only (9 days; IQR, 3-46). Neonates (adjusted coefficient, 44.3; 95% CI, 40.3-48.3 vs adolescent), Black race (adjusted coefficient, 4.78; 95% CI, 2.27-7.3), chronic condition indicator number (adjusted coefficient, 5.17; 95% CI, 4.56-5.78), and subtype (adjusted coefficient, 23.6; 95% CI, 20.4-26.7) were associated with a prolonged LOS. CONCLUSIONS: Most children with CHD who undergo noncardiac surgery have at least 1 chronic condition. Age, chronic conditions type and number, low socioeconomic status, and Black race impart increased risks of in-hospital mortality and prolonged LOS. Further research is needed to evaluate the impact of specific chronic conditions and determine barriers to equitable care.

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.

Comprehensive Risk Assessment of Morbidity in Pediatric Patients Undergoing Noncardiac Surgery: An Institutional Experience.

BACKGROUND: Utilizing the intrinsic surgical risk (ISR) and the patient's chronic and acute conditions, this study aims to develop and validate a comprehensive predictive model of perioperative morbidity in children undergoing noncardiac surgery. METHODS: Following institutional review board (IRB) approval at a tertiary care children's hospital, data for all noncardiac surgical encounters for a derivation dataset from July 2017 to December 2018 including 16,724 cases and for a validation dataset from January 2019 to December 2019 including 9043 cases were collected retrospectively. The primary outcome was a composite morbidity score defined by unplanned transfer to an intensive care unit (ICU), acute respiratory failure requiring intubation, postoperative need for noninvasive or invasive positive pressure ventilation, or cardiac arrest. Internal model validation was performed using 1000 bootstrap resamples, and external validation was performed using the 2019 validation cohort. RESULTS: A total of 1519 surgical cases (9.1%) experienced the defined composite morbidity. Using multivariable logistic regression, the Risk Assessment of Morbidity in Pediatric Surgery (RAMPS) score was developed with very good predictive ability in the derivation cohort (area under the curve [AUC] = 0.805; 95% confidence interval [CI], 0.795-0.816), very good internal validity using 1000 bootstrap resamples (bias-corrected Nagelkerke R = 0.21 and Brier score = 0.07), and good external validity (AUC = 0.783; 95% CI, 0.770-0.797). The included variables are age <5 years, critically ill, chronic condition indicator (CCI) ≥3, significant CCI ≥2, and ISR quartile ≥3. The RAMPS score ranges from 0 to 10, with the risk of composite morbidity ranging from 1.8% to 42.7%. CONCLUSIONS: The RAMPS score provides the ability to identify a high-risk cohort of pediatric patients using a 5-component tool, and it demonstrated good internal and external validity and generalizability. It also provides an opportunity to improve perioperative planning with the intent of improving both individual-patient outcomes and the appropriate allocation of health care resources.

Updates in Pediatric Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation is an increasingly used mode of life support for patients with cardiac and/or respiratory failure refractory to conventional therapy. This review provides a synopsis of the evolution of extracorporeal life support in neonates, infants, and children and offers a framework for areas in need of research. Specific aspects addressed are the changing epidemiology; technologic advancements in extracorporeal membrane oxygenation circuitry; the current status and future direction of anticoagulation management; sedative and analgesic strategies; and outcomes, with special attention to the lessons learned from neonatal survivors.

Prospective External Validation of the Pediatric Risk Assessment Score in Predicting Perioperative Mortality in Children Undergoing Noncardiac Surgery.

BACKGROUND: Early identification of children at high risk for perioperative mortality could lead to improved outcomes; however, there is a lack of well-validated risk prediction tools. The Pediatric Risk Assessment (PRAm) score is a new model to prognosticate perioperative risk of mortality in pediatric patients undergoing noncardiac surgery. It was derived from the American College of Surgeons (ACS) National Surgical Quality Improvement Program (NSQIP) Pediatric database. In this study, we aimed to externally validate the PRAm score at 1 large institution. METHODS: A PRAm score was prospectively assigned by the primary anesthesia team to children ≤18 years of age undergoing noncardiac surgery between July 2017 and July 2018 at a tertiary care pediatric hospital. The primary outcome was the PRAm score's ability to predict 30-day mortality. The area under the receiver operating characteristic (ROC) curve was utilized to determine discriminative ability. Sensitivity and specificity at varying cutoffs were considered. Youden J index and the gray zone approach were applied to determine the optimal PRAm cutoff for predicting 30-day mortality. RESULTS: Among the 13,530 cases included in the external validation cohort, the incidence of 30-day mortality was 0.21% (29/13,530). The PRAm score was found to predict 30-day mortality with an area under the curve (AUC) of 0.956 (95% confidence interval [CI], 0.938-0.974; P < .001). Youden J index determined the optimal PRAm score threshold to be ≥5 with a sensitivity of 86% and a specificity of 91%. The gray zone identified an inconclusive risk of mortality in 6.93% (938/13,530) of patients who had PRAm scores of 4 or 5 (sensitivity or specificity <90%, respectively), therefore refining the optimal cutoff point to be a PRAm score of ≥6. The incidence of mortality for patients with an American Society of Anesthesiologists Physical Status (ASA PS) ≤3 (0.06%, 8/13,530) increased 8-fold for those with an ASA PS of ≤3 and a PRAm score of ≥6. CONCLUSIONS: The PRAm score is a simple and objective tool that has excellent ability to predict perioperative risk of mortality in pediatric patients undergoing noncardiac surgery and can be easily used by clinicians. The application of the PRAm score could have important implications on the safety and quality of care delivered to infants and children and on the resource utilization in the pediatric health care system.

Arch watch: current approaches and opportunities for improvement.

Coarctation of the aorta (CoA) is a ductus arteriosus (DA)-dependent form of congenital heart disease (CHD) characterized by narrowing in the region of the aortic isthmus. CoA is a challenging diagnosis to make prenatally and is the critical cardiac lesion most likely to go undetected on the pulse oximetry-based newborn critical CHD screen. When undetected CoA causes obstruction to blood flow, life-threatening cardiovascular collapse may result, with a high burden of morbidity and mortality. Hemodynamic monitoring practices during DA closure (known as an "arch watch") vary across institutions and existing tools are often insensitive to developing arch obstruction. Novel measures of tissue oxygenation and oxygen deprivation may improve sensitivity and specificity for identifying evolving hemodynamic compromise in the newborn with CoA. We explore the benefits and limitations of existing and new tools to monitor the physiological changes of the aorta as the DA closes in infants at risk of CoA.

Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

Background Surgical systemic-to-pulmonary artery shunts have been the standard approach to establish stable pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow. More recently, transcatheter ductal stents have been performed as an alternative, less invasive intervention. We aimed to characterize trends in the utilization of surgical shunts versus ductal stents and compare associated outcomes. Methods and Results Using data from the Pediatric Health Information System, we retrospectively analyzed neonates with congenital heart disease with ductal-dependent pulmonary blood flow who underwent surgical shunt or ductal stent placement between January 2016 and December 2021. Patients were identified by International Classification of Diseases, Tenth Revision (ICD-10) diagnosis and procedure codes. The primary outcome was length of hospital stay. Secondary outcomes were reintervention risk and adjusted hospital costs. Of 936 patients included, 65.2% underwent a surgical shunt over the 6-year period. The proportion who underwent ductal stenting increased from 19% to 53.4% from 2016 to 2021. The median adjusted difference in postintervention length of hospital stay was 11 days greater for the surgical shunt cohort (95% CI, 7.2-14.8; P<0.001). The adjusted reintervention risks within 3 (odds ratio [OR], 3.37 [95% CI, 1.91-5.95], P<0.001) and 6 months (OR, 2.43 [95% CI, 1.62-3.64], P<0.001) were significantly greater in the ductal stent group. Median adjusted index hospital costs were $198 300 ($11 6400-$340 000) versus $120 400 ($81 800-$192 400) for the surgical shunt and ductal stent cohorts, respectively (P<0.001). Conclusions Ductal stenting has become an increasingly utilized palliative approach to secure pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow in the United States. Ductal stenting is associated with decreased length of hospital stay and reduced overall cost for the index hospitalization but with a greater reintervention risk than surgical shunting.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.