Widening our Focus: Characterizing Socioeconomic and Racial Disparities in Congenital Heart Disease.

BACKGROUND: Socioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome. METHODS: The Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions. RESULTS: We identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02). CONCLUSIONS: Interactions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives.

Early Impact of the COVID-19 Pandemic on Congenital Heart Surgery Programs Across the World: Assessment by a Global Multi-Societal Consortium.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

Pediatric Heart Donor Assessment Tool (PH-DAT): A novel donor risk scoring system to predict 1-year mortality in pediatric heart transplantation.

BACKGROUND: In this study we sought to quantify hazards associated with various donor factors into a cumulative risk scoring system (the Pediatric Heart Donor Assessment Tool, or PH-DAT) to predict 1-year mortality after pediatric heart transplantation (PHT). METHODS: PHT data with complete donor information (5,732) were randomly divided into a derivation cohort and a validation cohort (3:1). From the derivation cohort, donor-specific variables associated with 1-year mortality (exploratory p-value < 0.2) were incorporated into a multivariate logistic regression model. Scores were assigned to independent predictors (p < 0.05) based on relative odds ratios (ORs). RESULTS: The final model had an acceptable predictive value (c-statistic = 0.62). The significant 5 variables (ischemic time, stroke as the cause of death, donor-to-recipient height ratio, donor left ventricular ejection fraction, glomerular filtration rate) were used for the scoring system. The validation cohort demonstrated a strong correlation between the observed and expected rates of 1-year mortality (r = 0.87). The risk of 1-year mortality increases by 11% (OR 1.11 [1.08 to 1.14]; p < 0.001) in the derivation cohort and 9% (OR 1.09 [1.04 to 1.14]; p = 0.001) in the validation cohort with an increase of 1-point in score. Mortality risk increased 5 times from the lowest to the highest donor score in this cohort. Based on this model, a donor score range of 10 to 28 predicted 1-year recipient mortality of 11% to 31%. CONCLUSION: This novel pediatric-specific, donor risk scoring system appears capable of predicting post-transplant mortality. Although the PH-DAT may benefit organ allocation and assessment of recipient risk while controlling for donor risk, prospective validation of this model is warranted.

Transposition of the great arteries--outcomes and time interval of early neonatal repair.

BACKGROUND: This study evaluates the relationship of morbidity and resource utilization with the timing of early neonatal repair of transposition of the great arteries and intact ventricular septum (d-TGA/IVS). METHODS: All patients with d-TGA/IVS who underwent arterial switch in the first 14 days of life, between January 2000 and May 2011, were reviewed. Patients undergoing repair at ≤ 4 days of age were categorized as group I, 5 to 7 days as group II, and 8 to 14 days as group III. Outcomes included mortality, morbidity, and resource utilization. RESULTS: Hospital survival was 69 (98.6%) of 70. The length of stay (LOS) and total charges were lowest in group I--15.5 days compared to group II--18.0 days and group III--23.5 days (P = .005); group I--US$128,219 compared to group II--US$141,729 and group III--US$217,427 (P = .0006). Using regression analysis to account for potentially confounding effects of multiple variables and treating time as a continuous variable demonstrated that age at surgery was significantly associated with total LOS (P = .029), hospital charges (P = .029) and intensive care unit charges (P = .002). Younger age at repair was not associated with worse outcomes for any measure of morbidity. CONCLUSIONS: Earlier repair of d-TGA/IVS was associated with decreased resource utilization and no detriment to clinical outcomes. Further analysis based on a larger cohort of patients is needed to verify these results that have important implications for improving the value of care.

Noninvasive assessment of liver fibrosis in adult patients following the Fontan procedure.

OBJECTIVE: Recent data indicate that patients after the Fontan procedure are at risk for significant liver dysfunction; however, the prevalence and extent of liver disease in the Fontan population remains unknown. Furthermore, limited data exist in regard to screening for liver disease in adult Fontan patients. We sought to determine the prevalence of liver disease in adult patients following the Fontan procedure using computed tomography (CT) and serum biomarkers of liver fibrosis. DESIGN: Adult Fontan patients underwent screening for liver disease as part of their annual evaluation. Screening consisted of laboratory evaluation and dual-phase liver CT scan. Laboratory evaluation included analysis of liver function, viral hepatitis serologies, and FibroSURE panel (LabCorp), a test that analyzes the results of serum biomarkers to provide a quantitative surrogate marker for liver fibrosis. RESULTS: Sixteen patients, mean age 30.3 (range 20-41) years, were enrolled in the study. Mean length of follow-up from time of Fontan palliation was 20.5 (range 11-33) years. No patients had serologic evidence of viral hepatitis or synthetic liver dysfunction. Twelve patients (75%) had abnormal FibroSURE scores, seven (44%) had elevated FibroSURE scores predictive of Metavir fibrosis stage F2 or greater on liver biopsy, and one (6%) had a FibroSURE score predictive of cirrhosis on biopsy. All 16 patients had abnormal radiologic liver findings identified on CT, including heterogeneous enhancement in 11 (69%), varices in six (38%), and liver nodules in five patients (31%). Length of time since Fontan surgery correlated significantly with an elevated FibroSURE score (P = .05) and having more CT scan abnormalities (P = .04). CONCLUSIONS: Liver fibrosis detected by serum biomarkers and dual phase CT scan is common in adult patients following the Fontan procedure. Further studies are needed to determine the long-term clinical significance of these findings.

Thromboelastography in the assessment of bleeding following surgery for congenital heart disease.

BACKGROUND: Perioperative bleeding is common in pediatric cardiac surgery patients. Traditional laboratory tests do not adequately characterize coagulation derangements in patients with bleeding. We sought to establish preoperative thromboelastography parameters in children prior to cardiopulmonary bypass, to compare thromboelastography assessment with standard coagulation parameters postoperatively, and to assess thromboelastography in children with significant hemorrhage. METHODS: Sixty patients requiring cardiopulmonary bypass were enrolled in a prospective observational study of perioperative thromboelastography. Thromboelastography measures were obtained preoperatively, intraoperatively after protamine administration, upon admit to the intensive care unit, and when patients were treated for bleeding. Thromboelastography measures were not used for clinical care. Postoperative thromboelastography measurements were compared with the standard coagulation parameters. Intraoperative thromboelastography, postoperative thromboelastography, and clinical outcomes were compared among patients who did and did not have significant postoperative bleeding. RESULTS: Preoperative thromboelastography parameters were similar to other published normal values for pediatric patients. Transfusion recommendations based on thromboelastography measurements were significantly different from those based on the standard coagulation testing. Thromboelastography measures after initial protamine administration were significantly different in patients with postoperative bleeding. This difference was not present upon arrival to the intensive care unit. Patients with significant bleeding tended to cease bleeding when clinical interventions were in agreement with recommendations based on thromboelastography. CONCLUSIONS: Pediatric patients with significant postoperative bleeding after surgery are more likely to have abnormal thromboelastography early after cessation of cardiopulmonary bypass. Thromboelastography illustrates derangements in the coagulation system and may aid in the treatment of postoperative bleeding.

Noninvasive assessment of cardiac output.

Improved outcome from shock depends on early detection and correction of circulatory abnormalities. Global cardiac output and oxygen delivery must be adequate and distributed appropriately to meet metabolic demands to prevent the development of multiple organ system dysfunction, prolonged morbidity, and death. Circulatory assessment using standard monitors gives incomplete and sometimes misleading information. This article focuses on the available and emerging technologies that emphasize assessment of blood flow and regional tissue oxygenation.