Socioeconomic Impact on Outcomes During the First Year of Life of Patients with Single Ventricle Heart Disease: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

Socioeconomic status (SES) affects a range of health outcomes but has not been extensively explored in the single ventricle population. We investigate the impact of community-level deprivation on morbidity and mortality for infants with single ventricle heart disease in the first year of life. Retrospective cohort analysis of infants enrolled in the National Pediatric Cardiology Improvement Collaborative who underwent staged single ventricle palliation examining mortality and length of stay (LOS) using a community-level deprivation index (DI). 974 patients met inclusion criteria. Overall mortality was 20.5%, with 15.7% of deaths occurring between the first and second palliations. After adjusting for clinical risk factors, the DI was associated with death (log relative hazard [Formula: see text] = 8.92, p = 0.030) and death or transplant (log relative hazard [Formula: see text] = 8.62, p = 0.035) in a non-linear fashion, impacting those near the mean DI. Deprivation was associated with LOS following the first surgical palliation (S1P) (p = 0.031) and overall hospitalization during the first year of life (p = 0.018). For every 0.1 increase in the DI, LOS following S1P increased by 3.35 days (95% confidence interval 0.31-6.38) and total hospitalized days by 5.08 days (95% CI 0.88-9.27). Community deprivation is associated with mortality and LOS for patients with single ventricle congenital heart disease. While patients near the mean DI had a higher hazard of one year mortality compared to those at the extremes of the DI, LOS and DI were linearly associated, demonstrating the complex nature of SES factors.

Factors Associated with Inability to Discharge After Stage 1 Palliation for Single Ventricle Heart Disease: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Database.

Patient-level characteristics associated with survival for single ventricle heart disease following initial staged palliation have been described. However, the impact of peri-operative events on hospital discharge has not been examined. To characterize patient-level characteristics and peri-operative events that were associated with inability to be discharged after Stage 1 palliation (S1P). Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Dataset including patients who underwent a S1P procedure between 2016 and 2019 (Norwood or Hybrid Stage 1 procedure). We examined patient-level characteristics and peri-operative events as possible predictors of inability to discharge after S1P. We constructed multivariate logistic regression models examining post-S1P discharge and in-hospital mortality, adjusting for covariates. 843 patients underwent a S1P and 717 (85%) patients were discharged home or remained inpatient until Stage 2 for social but not medical concerns. Moderate or greater pre-operative atrioventricular valve regurgitation (odds ratio (OR) 4.6, 95% confidence interval (CI) 1.8-12), presence of high-risk pre-operative adverse events (OR 1.5, 95%CI 1.0-2.3), peri-operative events: temporary dialysis (OR 5.4, 95%CI 1.5-18.9), cardiac catheterization or cardiac surgery (OR 2.9, 95%CI 1.8-4.6), sepsis (OR 2.7, 95%CI 1.2-6.2), junctional tachycardia (OR 2.6, 95%CI 1.0-6.3), necrotizing enterocolitis (OR 2.6, 95%CI 1.3-5.2), ECMO (OR 2.5, 95%CI 1.4-4.3), neurological injury (OR 2.1, 95%CI 1.1-4.1), and re-intubation (OR 1.8, 95%CI 1.1-2.9) were associated with inability to discharge after Stage 1. Cardiac anatomical factors, pre-operative adverse events, post-operative re-intubation, post-operative ECMO, infectious complications, and unplanned catheter or surgical re-interventions were associated with inability to discharge after S1P. These findings suggest that quality improvement efforts aimed at reducing these peri-operative events may improve Stage 1 survival and likelihood of discharge.

Beta-blocker use after complete repair of tetralogy of Fallot: an analysis of a national database.

INTRODUCTION: In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics. METHODS: Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities. RESULTS: A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality. CONCLUSIONS: Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.

Debate - Replacement of the Mitral Valve Under One Year of Age: Mechanical Valves Should Be Used.

This article reviews the literature, focusing on publications from the third millennium and the results of mitral valve replacement in children younger than 1 year of age. Special consideration has been given to neonatal and infant valve replacement to provide insights into valve choice and technique. Mitral valve replacement is an important topic because it carries the highest mortality and poorer long-term prognosis than any other valve replacement in children.

Spectrum Medical Quantum or Terumo CDI 500: Which Device Measures Hemoglobin and Oxygen Saturation Most Accurately When Compared to a Benchtop Blood Analyzer?

To examine the accuracy between analyzers, the Terumo CDI 500 and the Spectrum Medical Quantum were compared to each other and to the ABL90 FLEX benchtop blood analyzer. Patients were retrospectively identified who underwent cardiac surgery requiring cardiopulmonary bypass between August 1, 2018 and November 1, 2019. Hemoglobin and venous saturation (SvO2) values from all three analyzers were collected. Measurements from the Quantum and the CDI 500 were averaged over 1 minute to provide a single value for the minute for the given device. Blood analysis on the ABL90 benchtop device was performed at a minimum of every hour during congenital cardiopulmonary bypass (CPB). There were 519 patients included in the analysis. Data points numbering 69,404 and 70,598 were analyzed when comparing the CDI 500 to the Quantum for hemoglobin and SvO2, respectively. Comparison of hemoglobin and SvO2 for the CDI 500 and Quantum versus ABL90 used 2283 and 1414 data points respectively, in each group. The CDI 500 and Quantum reported hemoglobin within 1 g/dL of the ABL90 86.9% and 87.5% of the time, respectively. The CDI 500 and Quantum reported SvO2 within 3% of the ABL90 61.0% and 57.9% of the time, respectively. The mean difference between the CDI 500 and Quantum hemoglobin and SvO2 measurements equaled .194 g/dL (p < .001) and .861% (p < .001), respectively and were both significantly different from zero. All device comparisons were statistically significantly different when compared to zero difference, likely due to the large data set as the magnitudes of these differences are all quite small and may not be clinically significant. However, while the reader should judge for themselves based upon their specific practice, in our opinion, the 95% Limit of Agreement was too large for either the CDI 500 or Quantum hemoglobin and SvO2 values to be substituted for ABL90 values. As recommended by the manufacturers, the CDI 500 and Quantum should only be used as a trending device.

Neonatal Cardiopulmonary Bypass Circuit Blood Prime Quality Analysis.

Blood-primed cardiopulmonary bypass circuits are frequently necessary to achieve safe support during pediatric open-heart surgery. Literature is lacking regarding suitable prime constituents or methods for achieving a physiologically appropriate blood-primed circuit. We examined the chemistry and hematology of neonatal blood-primed circuits from the conclusion of the priming procedure until the initiation of bypass. Base deficit/excess, pH, pO2, pCO2, HCO3, glucose, sodium, potassium, calcium, hematocrit, lactate, and osmolality were analyzed. Any deviation over time from the original prime value was compared for significance. Statistically significant changes were found between T0 and all time points for all parameters, except for pH and pO2 out to 1 hour. Among all parameters, various rates of change were observed. Although most changes in the parameters were found to be statistically significant, those changes may not be clinically significant based on clinician interpretation. Attention to the prime quality beyond the immediate post-priming period may be beneficial. Should the time period between validation of the prime quality and initiation of bypass be extended, it may be advisable to reevaluate the prime quality.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

How Small Is Too Small? Decision-Making and Management of the Small Aortic Root in the Setting of Interrupted Aortic Arch.

Interrupted aortic arch is commonly associated with a posterior malalignment ventricular septal defect (VSD) and left ventricular outflow tract (LVOT) hypoplasia. Standard repair is carried out in the neonatal period and includes re-establishing arc continuity and VSD closure. Reintervention on the LVOT for obstruction is a common and an ongoing source of morbidity and mortality. A variety of preoperative echocardiographic measurements have been identified to identify patients at risk for developing LVOT obstruction but an aortic valve annulus dimension (mm) < patient's weight (kg) + 1 mm is a reasonable threshold to identify a patient at increased risk for future LVOT reintervention. Prophylactic direct approaches to prevent future LVOT obstruction include myectomy/myotomy and left-sided placement of the VSD patch but do not reliably prevent late LVOT obstruction. Patients amendable to a biventricular repair but with important LVOT hypoplasia are probably best served with a Yasui operation, either as a primary operation or staged with a Norwood procedure. In the case of complex redo operations, a Ross-Konno provides another valuable option for a durable repair. Though smaller preoperative LVOT structures predict the need for reoperation, careful preoperative planning may minimize the need for LVOT reintervention and improve long-term survival.

Timing of Fontan Completion in Children with Functionally Univentricular Hearts and Isomerism: The Impact of Age, Weight, and Pre-Fontan Arterial Oxygen Saturation.

Isomerism, or heterotaxy, impacts morbidity and mortality after various stages of univentricular palliation. Timing of Fontan completing in these patients based on preoperative factors has not been investigated previously. The aim of this study was to determine the impact of preoperative factors on various outcomes including length of hospital stay and duration of chest tubes. A cross-sectional study was conducted. Patients with isomerism having undergone Fontan at the Children's Hospital of Wisconsin between 1998 and 2014 were identified. Preoperative, operative, and postoperative data were collected on these patients. Linear regression analysis was conducted to determine preoperative characteristics associated with various postoperative outcomes. Receiver operator curve analysis was also performed to determine the sensitivity and specificity of age and pre-Fontan arterial oxygen saturation in predicting increased length of hospitalization and increased duration of chest tubes. Younger age and lower pre-Fontan arterial oxygen saturation were associated with increased length of hospitalization while younger age, lower pre-Fontan arterial oxygen saturation, interrupted inferior caval vein, and worse pre-Fontan atrioventricular valve regurgitation were associated with increased length of chest tubes. Age, arterial oxygen saturation, pulmonary arteriovenous malformations, interruption of the inferior caval vein, and atrioventricular valve regurgitation should all be taken into consideration when timing Fontan completion in patients with isomerism. Arterial oxygen saturations between 82 and 84% with an approximate age of 3 years appear to be a time at which it is reasonable to consider Fontan in patients with isomerism.

Is there an optimal organ acceptance rate for pediatric heart transplantation: "A sweet spot"?

Despite a limited supply of donors, potential donor hearts are often declined for subjective concerns regarding organ quality. This analysis will investigate the relationship between donor heart AR and patient outcome at pediatric transplant centers. The UNOS database was used to identify all match runs for pediatric candidates (age < 18 years) from 2008 through March 2015 in which a heart offer was ultimately placed. Centers which received ≥10 offers/y were included (10 634 offers, 38 centers). Transplant centers were stratified based on their AR: low (<20%, n = 13), medium (20%-40%, n = 16), or high (>40%, n = 9). Low AR centers experienced worse negative WL outcome compared with medium (P = .022) and high (P = .004) AR centers. Low AR centers had similar post-transplant graft survival to medium (P = .311) or high (P = .393) AR centers; however, medium AR centers had better post-transplant graft survival than high AR centers (P = .037). E-F survival from listing regardless of transplant was worse for low AR centers compared with medium (P < .001) or high (P = .001) AR centers. Low AR centers experience worse WL outcomes without improvement in post-transplant outcomes. High AR centers experience higher post-transplant graft failure than medium AR centers. AR of 20%-40% appears to have optimal WL and post-transplant outcomes.

Identifiable Risk Factors and Miscalculations During Listing for Pediatric Heart Transplantation.

The objective of this study is to describe identifiable risk factors, complications, and pitfalls while listing pediatric patients for heart transplantation, which is the standard of care for end-stage heart disease in children. Since the introduction of cyclosporine in the 1980s, the management in pediatric heart transplantation has shown consistent improvement, mainly because of technological advances and the integration of multidisciplinary teams in the field. However, the complexity of this patient population makes medical providers vulnerable to complications as a result of undesirable mistakes. Transplant survival is impacted negatively when mistakes from health-care providers compound the high-risk status of the patient. The identification of multiple risk factors and undesirable miscalculations may help transplant teams make decisions before allocating organs, intervene or minimize morbidity, and provide the best quality of life to recipients.

The Norwood Stage 1 procedure - conduct of perfusion: 2017 Survey results from NPC-QIC member institutions.

Hypoplastic left heart syndrome (HLHS) is a rare and severe congenital cardiac defect. Approximately 1000 infants are born with HLHS in the United States every year. Healthcare collaboratives over the last decade have focused on sharing patient experiences and techniques in an effort to improve outcomes. In 2010, cardiologists and patient families joined together to improve the care of HLHS patients by forming the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Sixty-six of the approximately 110 institutions caring for patients with HLHS in the United States and Canada are now members of NPC-QIC. In 2017, cardiovascular perfusionists joined the collaborative as another specialty involved in the care of HLHS patients. Perfusionists and cardiac surgeons developed the collaborative's first conduct of perfusion survey for the Norwood Stage 1 procedure, specifically targeting the provision of cardiopulmonary bypass for patients with HLHS. This manuscript discusses the results of this survey, unveiling a significant variance in the conduct of perfusion for this patient population.

Congenital Heart Surgery Subspecialty Certification: How Is It Working? The American Board of Thoracic Surgery Perspective.

Congenital heart surgery is among the most challenging fields in all of medicine. The unique skills and knowledge base required prompted the American Board of Thoracic Surgery (ABTS) to establish the Subspecialty Certificate in Congenital Heart Surgery (CHS). Standards for operative experience and fellowship training programs were established by the ABTS and the Accreditation Council for Graduate Medical Education. Since 2009, 188 Subspecialty Certificates in CHS have been granted, including 38 earned through the fellowship training pathway. Today, the overwhelming majority of congenital heart surgeons in the United States hold a Subspecialty Certificate in CHS. Recent survey data suggests those individuals who have completed Accreditation Council for Graduate Medical Education fellowship training in CHS were satisfied with the operative experience they received during training and the vast majority are practicing CHS. While surgical training and education will always be a work in progress, in the 7 years since its inception, the ABTS Subspecialty Certificate in CHS has achieved the goal of quality control and standardization of CHS training.

Predicting the Need for Neoaortic Arch Intervention in Infants with Hypoplastic Left Heart Syndrome Through the Glenn Procedure.

Neoaortic recoarctation is present in up to over one-third of patients having undergone the Norwood procedure for hypoplastic left heart syndrome. Some of these patients will require reintervention by catheterization or surgery through the time of the Glenn procedure. Echocardiography and catheterization are often utilized in this period to assess hemodynamics although no specific criteria have been identified to predict whether and when neoaortic arch reintervention will be needed. We sought to identify predictors, including but not limited to echocardiographic and catheterization gradients, to predict such intervention. A retrospective analysis was conducted including patients with hypoplastic left heart syndrome. Patients with significantly comorbid lesions such as isomerism, anomalous pulmonary venous connections, and significant atrioventricular valve insufficiency were excluded as were patients without interstage echocardiographic and catheterization data. Receiver operator curve analysis was performed to establish peak-value gradients by echocardiography and catheterization that were predictive of neoaortic reintervention from the time of the Norwood through the time of the Glenn. These values were then entered into a multivariate regression with several other factors to determine what factors were predictive of need for such intervention. Bland-Altman analysis was conducted to compare echocardiographic and catheterization gradients. A peak echocardiographic gradient of 26 mmHg (100 % sensitivity, 85 % specificity) and a peak-to-peak catheterization gradient of 8.5 mm Hg (83 % sensitivity, 86 % specificity) were found to be predictive of need for neoaortic arch reintervention after multivariate analysis. Echocardiographic and catheterization gradients were found to have poor correlation with one another. A peak gradient of 26 mmHg or greater by echocardiography and a peak-to-peak gradient of 8.5 mmHg or greater by catheterization after the Norwood but prior to the Glenn are predictive of need for neoaortic reintervention through the time of the Glenn hospitalization.

The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p < 0.001) and MV changed from -2.88 to -1.41 (p < 0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1 year survival is high, but the need for RI and the presence of residual lesions are common.

Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

Accuracy of the Spectrum Medical M4 and Terumo CDI 500 compared to the Radiometer ABL90 FLEX benchtop blood analyzer.

The benchtop blood analyzer is the gold standard for blood oxygen saturation (SO2) and hemoglobin (Hb) analysis. However, the benchtop analyzer only provides values at a given point in time. In the field of cardiovascular perfusion and the practice of cardiopulmonary bypass (CPB), continuous measurement of SvO2 and hemoglobin values have become commonplace. Two devices currently available which monitor these values are the Terumo CDI 500 and Spectrum Medical M4. A retrospective study was conducted to examine the accuracy of the M4 technology and the CDI 500 as they compare to each other and the ABL90 FLEX, a benchtop blood gas analyzer. The data revealed the magnitude of mean differences were small, even when significant. However, the 95% Limits of Agreement were too large for either device to allow substitution of the CDI 500 and M4 hemoglobin or SvO2 values for ABL90 values. As recommended by the manufacturers, the CDI 500 and M4 should only be used as a trending device.

Impact of MYH6 variants in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a clinically and anatomically severe form of congenital heart disease (CHD). Although prior studies suggest that HLHS has a complex genetic inheritance, its etiology remains largely unknown. The goal of this study was to characterize a risk gene in HLHS and its effect on HLHS etiology and outcome. We performed next-generation sequencing on a multigenerational family with a high prevalence of CHD/HLHS, identifying a rare variant in the α-myosin heavy chain (MYH6) gene. A case-control study of 190 unrelated HLHS subjects was then performed and compared with the 1000 Genomes Project. Damaging MYH6 variants, including novel, missense, in-frame deletion, premature stop, de novo, and compound heterozygous variants, were significantly enriched in HLHS cases (P < 1 × 10-5). Clinical outcomes analysis showed reduced transplant-free survival in HLHS subjects with damaging MYH6 variants (P < 1 × 10-2). Transcriptome and protein expression analyses with cardiac tissue revealed differential expression of cardiac contractility genes, notably upregulation of the ß-myosin heavy chain (MYH7) gene in subjects with MYH6 variants (P < 1 × 10-3). We subsequently used patient-specific induced pluripotent stem cells (iPSCs) to model HLHS in vitro. Early stages of in vitro cardiomyogenesis in iPSCs derived from two unrelated HLHS families mimicked the increased expression of MYH7 observed in vivo (P < 1 × 10-2), while revealing defective cardiomyogenic differentiation. Rare, damaging variants in MYH6 are enriched in HLHS, affect molecular expression of contractility genes, and are predictive of poor outcome. These findings indicate that the etiology of MYH6-associated HLHS can be informed using iPSCs and suggest utility in future clinical applications.

Risk Factors for Abnormal Developmental Trajectories in Young Children With Congenital Heart Disease.

BACKGROUND: Children with congenital heart disease are at risk for developmental delay. This study sought to identify early risk factors for abnormal developmental trajectories in children with congenital heart disease. METHODS AND RESULTS: Children with congenital heart disease at high risk for developmental delay, without known genetic abnormality, and with ≥3 assessments by the use of the Bayley Scales of Infant and Toddler Development, Third Edition, were studied. Logistic regression was used to assess the impact of patient and clinical factors on cognitive, language, and motor score trajectories; classified as: average or improved if all scores were ≥85 (<1 standard deviation below the mean) or increased to ≥85 and never decreased; or abnormal if all scores were <85, fell to <85 and never improved, or fluctuated above and below 85. Data on 131 children with 527 Bayley Scales of Infant and Toddler Development, Third Edition assessments were analyzed. Subject age was 5.5 to 37.4 months. Overall, 56% had cognitive, language, and motor development in the average range. Delays occurred in single domains in 23%. Multiple domains were delayed in 21%. More cardiac surgeries, longer hospital stay, poorer linear growth, and tube feeding were associated with worse outcomes in all domains (P<0.05). In the multivariable model, the need for tube feeding was a risk factor for having an abnormal developmental trajectory (odds ratio, 5.1-7.9). Minority race and lack of private insurance had significant relationships with individual domains. CONCLUSIONS: Longitudinal developmental surveillance identified early factors that can help quantify the risk of developmental delay over time. Strategies to improve modifiable factors and early therapeutic intervention can be targeted to children at highest risk.

Advances in neonatal cardiac surgery: recent advances, the low-hanging fruit, what is on the horizon and the next moonshot.

PURPOSE OF REVIEW: To review the recent advances in neonatal cardiac surgery and identify future directions of this subspecialty of congenital heart surgery. RECENT FINDINGS: Recent advances in neonatal cardiac surgery have included the right ventricular to pulmonary artery conduit, the hybrid procedure and antegrade cerebral perfusion. In addition to refining the indications and techniques for these strategies, we can advance the field through collaborative learning between neonatal heart surgery centers. Collaborative learning is the low-hanging fruit that we can all use to advance the care of this patient population. On the horizon are a variety of strategies that will improve our management of this complex patient population. Among these improvements are better anticoagulation strategies targeting the unique hemostatic system of the neonate. The next transformative change, the next moonshot, may well be regenerative therapy that unlocks the body's ability to regenerate specific tissue such as valves and heart muscle to correct congenital defects and repair damaged muscle. SUMMARY: The current state of the specialty of neonatal cardiac surgery will be reviewed, and the possible future directions based on their ease and likelihood: specifically, the low-hanging fruit, what is on the horizon and the next moonshot.