Thoracic duct drainage patterns in heterotaxy.

BACKGROUND: Disordered lymphatic drainage is common in congenital heart diseases (CHD), but thoracic duct (TD) drainage patterns in heterotaxy have not been described in detail. This study sought to describe terminal TD sidedness in heterotaxy and its associations with other anatomic variables. METHODS: This was a retrospective, single-center study of patients with heterotaxy who underwent cardiovascular magnetic resonance imaging at a single center between July 1, 2019 and May 15, 2023. Patients with (1) asplenia (right isomerism), (2) polysplenia (left isomerism) and (3) pulmonary/abdominal situs inversus (PASI) plus CHD were included. Terminal TD sidedness was described as left-sided, right-sided, or bilateral. RESULTS: Of 115 eligible patients, the terminal TD was visualized in 56 (49 %). The terminal TD was left-sided in 25 patients, right-sided in 29, and bilateral in two. On univariate analysis, terminal TD sidedness was associated with atrial situs (p = 0.006), abdominal situs (p = 0.042), type of heterotaxy (p = 0.036), the presence of pulmonary obstruction (p = 0.041), superior vena cava sidedness (p = 0.005), and arch sidedness (p < 0.001). On multivariable analysis, only superior vena cava and aortic arch sidedness were independently associated with terminal TD sidedness. CONCLUSIONS: Terminal TD sidedness is highly variable in patients with heterotaxy. Superior vena cava and arch sidedness are independently associated with terminal TD sidedness. Type of heterotaxy was not independently associated with terminal TD sidedness. This data improves the understanding of anatomic variation in patients with heterotaxy and may be useful for planning for lymphatic interventions.

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.

Association of County-Level Availability of Pediatricians With Emergency Department Visits.

OBJECTIVES: The relationship between pediatrician availability and emergency department (ED) attendance is uncertain. We determined whether children in counties with more pediatricians had fewer ED visits. METHODS: We conducted a cross-sectional study of all ED visits among children younger than 18 years from 6 states. We obtained ED visit incidences by county and assessed the relationship to pediatrician density (pediatricians per 1000 children). Possible confounders included state, presence of an urgent care facility in the county, urban-rural status, and quartile of county-level characteristics: English-speaking, Internet access, White race, socioeconomic status, and public insurance. We estimated county-level changes in incidence by pediatrician density adjusting for state and separately for all possible confounders. RESULTS: Each additional pediatrician per 1000 children was associated with a 13.7% (95% confidence interval, -19.6% to -7.5%) decrease in ED visits in the state-adjusted model. In the full model, there was no association (-1.4%, 95% confidence interval, -7.2% to 4.8%). The presence of an urgent care, higher socioeconomic status score, urban status, and higher proportions of White race and nonpublic insurance were each associated with decreased ED visit rates. CONCLUSIONS: Pediatrician density is not associated with decreased ED visits after adjusting for other county demographic factors. Increasing an area's availability of pediatricians may not affect ED attendance.

Trajectories in Neurodevelopmental, Health-Related Quality of Life, and Functional Status Outcomes by Socioeconomic Status and Maternal Education in Children with Single Ventricle Heart Disease.

Trajectories of neurodevelopment and quality of life were analyzed in children with hypoplastic left heart syndrome according to socioeconomic status (SES) and maternal education. Lower SES and less maternal education were associated with greater early delays in communication and problem-solving and progressive delays in problem-solving and fine motor skills over time.

Prevalence and Predictors of Cholesterol Screening, Awareness, and Statin Treatment Among US Adults With Familial Hypercholesterolemia or Other Forms of Severe Dyslipidemia (1999-2014).

BACKGROUND: Familial hypercholesterolemia (FH) and other extreme elevations in low-density lipoprotein cholesterol significantly increase the risk of atherosclerotic cardiovascular disease; however, recent data suggest that prescription rates for statins remain low in these patients. National rates of screening, awareness, and treatment with statins among individuals with FH or severe dyslipidemia are unknown. METHODS: Data from the 1999 to 2014 National Health and Nutrition Examination Survey were used to estimate prevalence rates of self-reported screening, awareness, and statin therapy among US adults (n=42 471 weighted to represent 212 million US adults) with FH (defined using the Dutch Lipid Clinic criteria) and with severe dyslipidemia (defined as low-density lipoprotein cholesterol levels ≥190 mg/dL). Logistic regression was used to identify sociodemographic and clinical correlates of hypercholesterolemia awareness and statin therapy. RESULTS: The estimated US prevalence of definite/probable FH was 0.47% (standard error, 0.03%) and of severe dyslipidemia was 6.6% (standard error, 0.2%). The frequency of cholesterol screening and awareness was high (>80%) among adults with definite/probable FH or severe dyslipidemia; however, statin use was uniformly low (52.3% [standard error, 8.2%] of adults with definite/probable FH and 37.6% [standard error, 1.2%] of adults with severe dyslipidemia). Only 30.3% of patients with definite/probable FH on statins were taking a high-intensity statin. The prevalence of statin use in adults with severe dyslipidemia increased over time (from 29.4% to 47.7%) but not faster than trends in the general population (from 5.7% to 17.6%). Older age, health insurance status, having a usual source of care, diabetes mellitus, hypertension, and having a personal history of early atherosclerotic cardiovascular disease were associated with higher statin use. CONCLUSIONS: Despite the high prevalence of cholesterol screening and awareness, only ≈50% of adults with FH are on statin therapy, with even fewer prescribed a high-intensity statin; young and uninsured patients are at the highest risk for lack of screening and for undertreatment. This study highlights an imperative to improve the frequency of cholesterol screening and statin prescription rates to better identify and treat this high-risk population. Additional studies are needed to better understand how to close these gaps in screening and treatment.

Life Expectancy after Myocardial Infarction, According to Hospital Performance.

BACKGROUND: Thirty-day risk-standardized mortality rates after acute myocardial infarction are commonly used to evaluate and compare hospital performance. However, it is not known whether differences among hospitals in the early survival of patients with acute myocardial infarction are associated with differences in long-term survival. METHODS: We analyzed data from the Cooperative Cardiovascular Project, a study of Medicare beneficiaries who were hospitalized for acute myocardial infarction between 1994 and 1996 and who had 17 years of follow-up. We grouped hospitals into five strata that were based on case-mix severity. Within each case-mix stratum, we compared life expectancy among patients admitted to high-performing hospitals with life expectancy among patients admitted to low-performing hospitals. Hospital performance was defined by quintiles of 30-day risk-standardized mortality rates. Cox proportional-hazards models were used to calculate life expectancy. RESULTS: The study sample included 119,735 patients with acute myocardial infarction who were admitted to 1824 hospitals. Within each case-mix stratum, survival curves of the patients admitted to hospitals in each risk-standardized mortality rate quintile separated within the first 30 days and then remained parallel over 17 years of follow-up. Estimated life expectancy declined as hospital risk-standardized mortality rate quintile increased. On average, patients treated at high-performing hospitals lived between 0.74 and 1.14 years longer, depending on hospital case mix, than patients treated at low-performing hospitals. When 30-day survivors were examined separately, there was no significant difference in unadjusted or adjusted life expectancy across hospital risk-standardized mortality rate quintiles. CONCLUSIONS: In this study, patients admitted to high-performing hospitals after acute myocardial infarction had longer life expectancies than patients treated in low-performing hospitals. This survival benefit occurred in the first 30 days and persisted over the long term. (Funded by the National Heart, Lung, and Blood Institute and the National Institute of General Medical Sciences Medical Scientist Training Program.).

Impact of Socioeconomic Status on Outcomes of Patients with Kawasaki Disease.

OBJECTIVE: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. STUDY DESIGN: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 2017. Neighborhood SES was measured using a US census-based score derived from 6 measures related to income, education, and occupation. Linear and logistic regression were used to examine the association of SES with number of days of fever at time of treatment, LOS, and CAA. RESULTS: Patients in the lowest SES quartile were treated later than patients with greater SES (7 [IQR 5, 9] vs 6 [IQR 5, 8] days, P = .01). Patients in the lowest SES quartile were more likely to be treated after 10 days of illness, with an OR 1.9 (95% CI 1.3-2.8). In multivariable analysis, SES remained an independent predictor of the number of days of fever at time of treatment (P = .01). Patients in the lowest SES quartile had longer LOS than patients with greater SES (3 [IQR 2, 5] vs 3 [IQR 2, 4], P = .007). In subgroup analysis of white children, those in the lowest SES quartile vs quartiles 2-4 were more likely to develop large/giant CAA 17 (12%) vs 30 (6%), P = .03. CONCLUSIONS: Lower SES is associated with delayed treatment, prolonged LOS, and increased risk of large/giant CAA. Novel approaches to diagnosis and education are needed for children living in low-SES neighborhoods.

Timing and Causes of Common Pediatric Readmissions.

OBJECTIVE: To evaluate and compare readmission causes and timing within the first 30 days after hospitalization for 3 acute and 3 chronic common pediatric conditions. STUDY DESIGN: Data from the 2013 to 2014 Nationwide Readmissions Database were used to examine the daily percentage of readmissions occurring on days 1-30 and the leading causes of readmission after hospitalization for 3 acute (appendicitis, bronchiolitis/croup, and gastroenteritis) and 3 chronic (asthma, epilepsy, and sickle cell) conditions for patients aged 1-17 years (n = 2 753 488). Data were analyzed using Cox proportional hazards regression. RESULTS: The 30-day readmission rates ranged from 2.6% (SE, 0.1) after hospitalizations for appendectomy to 19.1% (SE, 0.5) after hospitalizations for sickle cell anemia. More than 50% of 30-day readmissions after acute conditions occurred within 15 days after discharge, whereas readmissions after chronic conditions occurred more uniformly throughout the 30 days after discharge. Higher numbers of patient comorbidities were associated with increased risk of readmission at days 1-7, 8-15, and 16-30 after discharge for all conditions examined. Most 30-day readmissions after chronic conditions were for the same diagnosis or closely related conditions as the index admission (67% for asthma, 65% for seizure disorder, and 82% for sickle cell anemia) in contrast with 50% or fewer readmissions after acute conditions (46% for appendectomy, 47% for bronchiolitis/croup, and 19% for gastroenteritis). CONCLUSIONS: The timing and causes of pediatric readmissions vary greatly across pediatric conditions. To be effective, strategies for reducing readmissions need to account for the index diagnosis to better target the highest risk period and causes for readmission.

Consistency of Physician Data Across Health Insurer Directories.

This study assesses the consistency of information across publicly available physician directories from 5 large national health insurers.

Race, Socioeconomic Status, and Life Expectancy After Acute Myocardial Infarction.

BACKGROUND: Previous studies have been unable to disentangle the negative associations of black race and low socioeconomic status (SES) with long-term outcomes of patients after acute myocardial infarction (AMI). Such information could assist in efforts to address both racial and socioeconomic disparities. METHODS AND RESULTS: We used data from the Cooperative Cardiovascular Project, a prospective cohort study of Medicare beneficiaries hospitalized with AMI with 17 years of follow-up, to evaluate the relationship between race, area-level SES (measured by zip code-level median household income), and life expectancy after AMI. Life expectancy was estimated by using Cox proportional hazards regression with extrapolation using exponential models. Of the 141 095 patients with AMI, 6.3% were black and 6.8% resided in low-SES areas; 26% of black patients lived in low-SES areas in comparison with 5.7% of white patients. Post-myocardial infarction life expectancy estimates were shorter for black patients than for white patients across all socioeconomic levels in patients ≤ 75 years of age. After adjustment for patient and treatment characteristics, the association between race and life expectancy persisted but was attenuated. Younger black patients (<68 years) had shorter life expectancies than white patients, whereas older black patients had longer life expectancies. The largest white-black gap in life expectancy occurred in patients residing in high- and medium-SES areas (P=0.02 interaction). CONCLUSIONS: Black and white patients residing in low-SES areas have similar life expectancies after AMI, which are lower than those living in higher-SES areas. Racial disparities were most prominent among patients living in high-SES areas.

Sex differences in perceived stress and early recovery in young and middle-aged patients with acute myocardial infarction.

BACKGROUND: Younger age and female sex are both associated with greater mental stress in the general population, but limited data exist on the status of perceived stress in young and middle-aged patients presenting with acute myocardial infarction. METHODS AND RESULTS: We examined sex difference in stress, contributing factors to this difference, and whether this difference helps explain sex-based disparities in 1-month recovery using data from 3572 patients with acute myocardial infarction (2397 women and 1175 men) 18 to 55 years of age. The average score of the 14-item Perceived Stress Scale at baseline was 23.4 for men and 27.0 for women (P<0.001). Higher stress in women was explained largely by sex differences in comorbidities, physical and mental health status, intrafamily conflict, caregiving demands, and financial hardship. After adjustment for demographic and clinical characteristics, women had worse recovery than men at 1 month after acute myocardial infarction, with mean differences in improvement score between women and men ranging from -0.04 for EuroQol utility index to -3.96 for angina-related quality of life (P<0.05 for all). Further adjustment for baseline stress reduced these sex-based differences in recovery to -0.03 to -3.63, which, however, remained statistically significant (P<0.05 for all). High stress at baseline was associated with significantly worse recovery in angina-specific and overall quality of life, as well as mental health status. The effect of baseline stress on recovery did not vary between men and women. CONCLUSIONS: Among young and middle-aged patients, higher stress at baseline is associated with worse recovery in multiple health outcomes after acute myocardial infarction. Women perceive greater psychological stress than men at baseline, which partially explains women's worse recovery.

Underweight, Markers of Cachexia, and Mortality in Acute Myocardial Infarction: A Prospective Cohort Study of Elderly Medicare Beneficiaries.

BACKGROUND: Underweight patients are at higher risk of death after acute myocardial infarction (AMI) than normal weight patients; however, it is unclear whether this relationship is explained by confounding due to cachexia or other factors associated with low body mass index (BMI). This study aimed to answer two questions: (1) does comprehensive risk adjustment for comorbid illness and frailty measures explain the higher mortality after AMI in underweight patients, and (2) is the relationship between underweight and mortality also observed in patients with AMI who are otherwise without significant chronic illness and are presumably free of cachexia? METHODS AND FINDINGS: We analyzed data from the Cooperative Cardiovascular Project, a cohort-based study of Medicare beneficiaries hospitalized for AMI between January 1994 and February 1996 with 17 y of follow-up and detailed clinical information to compare short- and long-term mortality in underweight and normal weight patients (n = 57,574). We used Cox proportional hazards regression to investigate the association of low BMI with 30-d, 1-y, 5-y, and 17-y mortality after AMI while adjusting for patient comorbidities, frailty measures, and laboratory markers of nutritional status. We also repeated the analyses in a subset of patients without significant comorbidity or frailty. Of the 57,574 patients with AMI included in this cohort, 5,678 (9.8%) were underweight and 51,896 (90.2%) were normal weight at baseline. Underweight patients were older, on average, than normal weight patients and had a higher prevalence of most comorbidities and measures of frailty. Crude mortality was significantly higher for underweight patients than normal weight patients at 30 d (25.2% versus 16.4%, p < 0.001), 1 y (51.3% versus 33.8%, p < 0.001), 5 y (79.2% versus 59.4%, p < 0.001), and 17 y (98.3% versus 94.0%, p < 0.001). After adjustment, underweight patients had a 13% higher risk of 30-d death and a 26% higher risk of 17-y death than normal weight patients (30-d hazard ratio [HR] 1.13, 95% CI 1.07-1.20; 17-y HR 1.26, 95% CI 1.23-1.30). Survival curves for underweight and normal weight patients separated early and remained separate over 17 y, suggesting that underweight patients remained at a significant survival disadvantage over time. Similar findings were observed among the subset of patients without comorbidity at baseline. Underweight patients without comorbidity had a 30-d adjusted mortality similar to that of normal weight patients but a 21% higher risk of death over the long term (30-d HR 1.08, 95% CI 0.93-1.26; 17-y HR 1.21, 95% CI 1.14-1.29). The adverse effects of low BMI were greatest in patients with very low BMIs. The major limitation of this study was the use of surrogate markers of frailty and comorbid conditions to identify patients at highest risk for cachexia rather than clear diagnostic criteria for cachexia. CONCLUSIONS: Underweight BMI is an important risk factor for mortality after AMI, independent of confounding by comorbidities, frailty measures, and laboratory markers of nutritional status. Strategies to promote weight gain in underweight patients after AMI are worthy of testing.

Excess weight and life expectancy after acute myocardial infarction: The obesity paradox reexamined.

BACKGROUND: An "obesity paradox" has been described in patients with acute myocardial infarction (AMI), whereby obese and overweight patients have a lower risk of short-term mortality after AMI than normal-weight patients. However, the long-term association of obesity with mortality after AMI remains unknown. METHODS: We used data from the Cooperative Cardiovascular Project, a prospective medical record study of Medicare beneficiaries hospitalized with AMI with 17 years of follow-up (N = 124,981), to evaluate the association of higher body mass index (BMI) with short- and long-term survival after AMI. Cox proportional hazards models were used to estimate life expectancy after AMI and the years of potential life lost or gained attributable to excess weight. RESULTS: Approximately 41.5% of patients were classified as normal weight; 38.7%, as overweight; 14.3%, as obese; and 5.5%, as morbidly obese. Normal-weight patients had the highest crude mortality at all follow-up time points, whereas obese patients had the lowest. Adjustment for patient and treatment characteristics reduced this difference, but the survival benefit persisted in overweight and obese patients at all time points. Morbidly obese and normal-weight patients had a comparable risk of death at 17 years. Life expectancy estimates were generally lowest for morbidly obese patients and highest for overweight patients. Differences in life expectancy between BMI groups were most pronounced in younger patients. After adjustment, overweight and obesity were associated with greater life years at all ages; however, morbid obesity was only associated with better survival in patients ≥75 years of age at the time of AMI. CONCLUSIONS: Overweight and obesity are associated with improved short- and long-term survival after AMI, which results in moderate gains in life expectancy relative to normal-weight patients. These findings suggest that higher BMI confers a protective advantage over the entire remaining lifespan in older patients with AMI.

Sex differences in long-term mortality after myocardial infarction: a systematic review.

BACKGROUND: Studies of sex differences in long-term mortality after acute myocardial infarction have reported mixed results. A systematic review is needed to characterize what is known about sex differences in long-term outcomes and to define gaps in knowledge. METHODS AND RESULTS: We searched the Medline database from 1966 to December 2012 to identify all studies that provided sex-based comparisons of mortality after acute myocardial infarction. Only studies with at least 5 years of follow-up were reviewed. Of the 1877 identified abstracts, 52 studies met the inclusion criteria, of which 39 were included in this review. Most studies included fewer than one-third women. There was significant heterogeneity across studies in patient populations, methodology, and risk adjustment, which produced substantial variability in risk estimates. In general, most studies reported higher unadjusted mortality for women compared with men at both 5 and 10 years after acute myocardial infarction; however, many of the differences in mortality became attenuated after adjustment for age. Multivariable models varied between studies; however, most reported a further reduction in sex differences after adjustment for covariates other than age. Few studies examined sex-by-age interactions; however, several studies reported interactions between sex and treatment whereby women have similar mortality risk as men after revascularization. CONCLUSIONS: Sex differences in long-term mortality after acute myocardial infarction are largely explained by differences in age, comorbidities, and treatment use between women and men. Future research should aim to clarify how these differences in risk factors and presentation contribute to the sex gap in mortality.

The Evolving Role of Genetic Evaluation in the Prenatal Diagnosis and Management of Congenital Heart Disease.

Congenital heart disease (CHD) is increasingly diagnosed prenatally and the ability to screen and diagnose the genetic factors involved in CHD have greatly improved. The presence of a genetic abnormality in the setting of prenatally diagnosed CHD impacts prenatal counseling and ensures that families and providers have as much information as possible surrounding perinatal management and what to expect in the future. This review will discuss the genetic evaluation that can occur prior to birth, what different genetic testing methods are available, and what to think about in the setting of various CHD diagnoses.

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.

Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. Results: Among 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P < .001), and had longer in-hospital length of stay (20 vs 12 days, P = .009). Among patients with moderate to severe atrioventricular valve regurgitation, single-ventricle palliation is associated with a greater risk of unplanned reintervention compared with patients undergoing biventricular repair (hazard ratio, 2.13; CI, 1.10-4.12; P = .025). Conclusions: There was no significant difference in early or late outcomes in single-ventricle versus biventricular repair strategies in heterotaxy. In the subgroup of patients with moderate/severe atrioventricular valve regurgitation, patients who underwent single-ventricle palliation were 2.5 times more likely to need a late reintervention compared with those undergoing biventricular repair.

Risk Factors for Death or Transplant After Stage 2 Palliation for Single Ventricle Heart Disease.

Background: For infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur. Objectives: This study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday. Methods: Retrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P. Multivariable logistic regression and classification and regression trees were performed to identify risk factors for mortality and transplantation referral after S2P. Results: Of the 1,455 patients in the cohort who survived to S2P, 5.2% died and 2.3% were referred for transplant. Overall event rates at 30 and 100 days after S2P were 2% and 5%, respectively. Independent risk factors for mortality and transplantation referral included the presence of a known genetic syndrome, shunt type at stage 1 procedure (S1P), tricuspid valve repair at S1P, longer time to extubation and reintubation after S1P, ≥ moderate tricuspid regurgitation prior to S2P, younger age at S2P, and the risk groups identified in the classification and regression tree analysis (extracorporeal membrane oxygenation after S1P and longer S2P cardiopulmonary bypass time without extracorporeal membrane oxygenation). Conclusions: Mortality and transplantation referral rates after S2P to 1 year of age remain high ∼7%. Many of the identified risk factors after S2P are similar to those established for interstage factors around the S1P, whereas others may be unique to the period after S2P.

Prenatal care coordination, racial and socioeconomic inequities, and pre- and post-operative outcomes in hypoplastic left heart syndrome.

OBJECTIVE: We sought to identify associations between prenatal care coordination (PNC) and outcomes in hypoplastic left heart syndrome (HLHS). STUDY DESIGN: We hypothesized that suboptimal PNC is associated with worse pre-operative status. HLHS patients from 2016 through 2019 were identified using a multicenter registry. Optimal PNC was defined as (1) a completed interdisciplinary conference and (2) closed-loop communication with the obstetric team. Associations between PNC and outcomes were identified. RESULTS: Of 1441 patients, 1242 (86%) had prenatal diagnosis. Among those with a prenatal diagnosis, PNC was achieved in only 845 (68%). Suboptimal PNC was associated with adverse events (50% vs 40%, p < 0.001), inotrope need (19% vs 13%, p = 0.007), mechanical ventilation (22% vs 16%, p = 0.016), and parenteral feeding (60% vs 46%, p < 0.001). African-American race and non-commercial insurance were associated with a lower likelihood of optimal PNC (p = 0.006 and p < 0.001, respectively). CONCLUSION: Improving PNC and overcoming racial and socioeconomic barriers are important targets to improve HLHS perinatal care.

Annual Variation in 30-Day Risk-Adjusted Readmission Rates in U.S. Children's Hospitals.

OBJECTIVE: Reducing pediatric readmissions has become a national priority; however, the use of readmission rates as a quality metric remains controversial. The goal of this study was to examine short-term stability and long-term changes in hospital readmission rates. METHODS: Data from the Pediatric Health Information System were used to compare annual 30-day risk-adjusted readmission rates (RARRs) in 47 US children's hospitals from 2016 to 2017 (short-term) and 2016 to 2019 (long-term). Pearson correlation coefficients and weighted Cohen's Kappa statistics were used to measure correlation and agreement across years for hospital-level RARRs and performance quartiles. RESULTS: Median (IQR) 30-day RARRs remained stable from 7.7% (7.0-8.3) in 2016 to 7.6% (7.0-8.1) in 2019. Individual hospital RARRs in 2016 were strongly correlated with the same hospital's 2017 rate (R2 = 0.89 [95% confidence interval (CI) 0.80-0.94]) and moderately correlated with those in 2019 (R2 = 0.49 [95%CI 0.23-0.68]). Short-term RARRs (2016 vs 2017) were more highly correlated for medical conditions than surgical conditions, but correlations between long-term medical and surgical RARRs (2016 vs 2019) were similar. Agreement between RARRs was higher when comparing short-term changes (0.73 [95%CI 0.59-0.86]) than long-term changes (0.45 [95%CI 0.27-0.63]). From 2016 to 2019, RARRs increased by ≥1% in 7 (15%) hospitals and decreased by ≥1% in 6 (13%) hospitals. Only 7 (15%) hospitals experienced reductions in RARRs over the short and long-term. CONCLUSIONS: Hospital-level performance on RARRs remained stable with high agreement over the short-term suggesting stability of readmission measures. There was little evidence of sustained improvement in hospital-level performance over multiple years.