Faculty Bibliography

BACKGROUND:Congenital heart defects are the most common and resource-intensive birth defects. As children with congenital heart defects increasingly survive beyond early childhood, it is imperative to understand longitudinal disease burden. OBJECTIVES:The purpose of this study was to examine chronic outpatient prescription medication use and expenditures for New York State pediatric Medicaid enrollees, comparing children who undergo cardiac surgery (cardiac enrollees) and the general pediatric population. METHODS:This was a retrospective cohort study of all Medicaid enrollees age <18 years using the New York State Congenital Heart Surgery Collaborative for Longitudinal Outcomes and Utilization of Resources database (2006-2019). Primary outcomes were total chronic medications per person-year, enrollees per 100 person-years using ≥1 and ≥3 medications, and medication expenditures per person-year. We described and compared outcomes between cardiac enrollees and the general pediatric population. Among cardiac enrollees, multivariable regression examined associations between outcomes and clinical characteristics. RESULTS:We included 5,459 unique children (32,131 person-years) who underwent cardiac surgery and 4.5 million children (22 million person-years) who did not. More than 4 in 10 children who underwent cardiac surgery used ≥1 chronic medication compared with approximately 1 in 10 children who did not have cardiac surgery. Medication expenditures were 10 times higher per person-year for cardiac compared with noncardiac enrollees. Among cardiac enrollees, disease severity was associated with chronic medication use; use was highest among infants; however, nearly one-half of adolescents used ≥1 chronic medication. CONCLUSIONS:Children who undergo cardiac surgery experience high medication burden that persists throughout childhood. Understanding chronic medication use can inform clinicians (both pediatricians and subspecialists) and policymakers, and ultimately the value of care for this medically complex population.

BACKGROUND:Understanding the longitudinal burden of health care expenditures and utilization after pediatric cardiac surgery is needed to counsel families, improve care, and reduce outcome inequities. OBJECTIVES:The purpose of this study was to describe and identify predictors of health care expenditures and utilization for Medicaid-insured pediatric cardiac surgical patients. METHODS:All Medicaid enrolled children age <18 years undergoing cardiac surgery in the New York State CHS-COLOUR database, from 2006 to 2019, were followed in Medicaid claims data through 2019. A matched cohort of children without cardiac surgical disease was identified as comparators. Expenditures and inpatient, primary care, subspecialist, and emergency department utilization were modeled using log-linear and Poisson regression models to assess associations between patient characteristics and outcomes. RESULTS:In 5,241 New York Medicaid-enrolled children, longitudinal health care expenditures and utilization for cardiac surgical patients exceeded noncardiac surgical comparators (cardiac surgical children: $15,500 ± $62,000 per month in year 1 and $1,600 ± $9,100 per month in year 5 vs noncardiac surgical children: $700 ± $6,600 per month in year 1 and $300 ± $2,200 per month in year 5). Children after cardiac surgery spent 52.9 days in hospitals and doctors' offices in the first postoperative year and 90.5 days over 5 years. Being Hispanic, compared with non-Hispanic White, was associated with having more emergency department visits, inpatient admissions, and subspecialist visits in years 2 to 5, but fewer primary care visits and greater 5-year mortality. CONCLUSIONS:Children after cardiac surgery have significant longitudinal health care needs, even among those with less severe cardiac disease. Health care utilization differed by race/ethnicity, although mechanisms driving disparities should be investigated further.

Myxomas are rare tumors in neonates and tend to have a different presentation compared to adults. We present an infant with a left atrial myxoma presenting with episodic tachycardia who underwent successful surgical excision. In addition, we performed a review of the literature, identifying 17 cases of neonatal myxomas. Unlike adults, neonatal myxomas are more common in males and occur more often on the right side of the heart. Constitutional symptoms such as fever or embolism are rare among neonates. Most patients have favorable outcomes following surgical excision, refuting earlier claims that neonatal myxomas are associated with poor outcomes.

We present the case of a 4-month-old, former 23-week premature baby who underwent patent ductus arteriosus device closure in the cardiac catheterisation lab with an Amplatzer Piccoloâ„¢ device at 12 weeks of life. This was complicated by late migration of the device into the aorta resulting in severe obstruction and requiring surgical intervention.

Surgical management of single ventricle with interrupted inferior vena cava and azygos continuation typically requires a Kawashima procedure with subsequent completion of Fontan. However, this group is at risk of development of pulmonary arteriovenous malformations. Evidence suggests preservation of hepatic venous flow into the pulmonary circulation can potentially delay this development. We hereby describe a method of preserving antegrade pulmonary blood flow during the Kawashima procedure in the setting of prior right ventricular outflow tract stents.

BACKGROUND:As the cardiac community strives to improve outcomes, accurate methods of risk stratification are imperative. Since adoption of International Classification of Disease-10th Revision (ICD-10) in 2015, there is no published method for congenital heart surgery risk stratification for administrative data. OBJECTIVES/OBJECTIVE:This study sought to develop an empirically derived, publicly available Risk Stratification for Congenital Heart Surgery (RACHS-2) tool for ICD-10 administrative data. METHODS:The RACHS-2 stratification system was iteratively and empirically refined in a training dataset of Pediatric Health Information Systems claims to optimize sensitivity and specificity compared with corresponding locally held Society of Thoracic Surgeons-Congenital Heart Surgery (STS-CHS) clinical registry data. The tool was validated in a second administrative data source: New York State Medicaid claims. Logistic regression was used to compare the ability of RACHS-2 in administrative data to predict operative mortality vs STAT Mortality Categories in registry data. RESULTS:The RACHS-2 system captured 99.6% of total congenital heart surgery registry cases, with 1.0% false positives. RACHS-2 predicted operative mortality in both training and validation administrative datasets similarly to STAT Mortality Categories in registry data. C-statistics for models for operative mortality in training and validation administrative datasets-adjusted for RACHS-2-were 0.76 and 0.84 (95% CI: 0.72-0.80 and 0.80-0.89); C-statistics for models for operative mortality-adjusted for STAT Mortality Categories-in corresponding clinical registry data were 0.75 and 0.84 (95% CI: 0.71-0.79 and 0.79-0.89). CONCLUSIONS:RACHS-2 is a risk stratification system for pediatric cardiac surgery for ICD-10 administrative data, validated in 2 administrative-registry-linked datasets. Statistical code is publicly available upon request.