Faculty Bibliography

The use of prostaglandin infusion to maintain patency of the ductus arteriosus in patients with critical coarctation of the aorta (CoA) to support systemic circulation is the standard of care. However, pulmonary overcirculation resulting from a patent ductus arteriosus in patients with critical CoA is not well described in the literature. We report two cases of critical CoA that required invasive measures to control pulmonary blood flow before surgical repair of the CoA. Both patients had signs of decreased oxygen delivery, hyperlactatemia, and systemic to pulmonary flow via the ductus arteriosus. One patient required surgical pulmonary artery banding and the second patient underwent pulmonary flow restrictor device placement for the control of pulmonary blood flow. A rapid improvement in oxygen delivery and normalization of lactate levels were observed after control of pulmonary overcirculation. Both patients underwent successful surgical repair of the coarctation A and were discharged home.

Extracorporeal life support (ECLS) therapy is increasingly being used to support children with refractory cardiorespiratory failure, but its use is occasionally associated with complications.1 Neonatal aortic dissection in association with ECLS is rare and the clinical sequelae of aortic dissection in neonates are poorly understood. We report a case of extensive type B aortic dissection in a neonate receiving ECLS therapy for refractory cardiogenic shock secondary to tachycardia-induced cardiomyopathy and Wolf Parkinson White (WPW) syndrome. The patient was noted to have aortic dissection along with multiple abdominal organ ischemic injury a day after ECLS arterial cannula position adjustment. The patient was rapidly decannulated from ECLS and the aortic dissection was managed conservatively with good outcome. We discuss our approach and rationale behind conservative management of this rarely reported complication associated with ECLS therapy and discuss available literature.

BACKGROUND:Despite documented associations between social determinants of health and outcomes post-congenital heart surgery, clinical risk models typically exclude these factors. OBJECTIVES/OBJECTIVE:The study sought to characterize associations between social determinants and operative and longitudinal mortality as well as assess impacts on risk model performance. METHODS:Demographic and clinical data were obtained for all congenital heart surgeries (2006-2021) from locally held Congenital Heart Surgery Collaborative for Longitudinal Outcomes and Utilization of Resources Society of Thoracic Surgeons Congenital Heart Surgery Database data. Neighborhood-level American Community Survey and composite sociodemographic measures were linked by zip code. Model prediction, discrimination, and impact on quality assessment were assessed before and after inclusion of social determinants in models based on the 2020 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model. RESULTS:Of 14,173 total index operations across New York State, 12,321 cases, representing 10,271 patients at 8 centers, had zip codes for linkage. A total of 327 (2.7%) patients died in the hospital or before 30 days, and 314 children died by December 31, 2021 (total n = 641; 6.2%). Multiple measures of social determinants of health explained as much or more variability in operative and longitudinal mortality than clinical comorbidities or prior cardiac surgery. Inclusion of social determinants minimally improved models' predictive performance (operative: 0.834-0.844; longitudinal 0.808-0.811), but significantly improved model discrimination; 10.0% more survivors and 4.8% more mortalities were appropriately risk classified with inclusion. Wide variation in reclassification was observed by site, resulting in changes in the center performance classification category for 2 of 8 centers. CONCLUSIONS:Although indiscriminate inclusion of social determinants in clinical risk modeling can conceal inequities, thoughtful consideration can help centers understand their performance across populations and guide efforts to improve health equity.

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.

Canine chronic ulcerative stomatitis (CCUS) is a spontaneously occurring, painful, and often debilitating condition of the oral cavity, with a suspected immune-mediated component. The response to pharmacological treatment is generally poor, thus the need to identify more effective medical therapies for this condition. This article describes a prospective clinical trial that was designed to evaluate the efficiency of a combination of cyclosporine and metronidazole in managing CCUS. The hypothesis was that a combination of cyclosporine and metronidazole would effectively minimize clinical signs associated with CCUS. Ten client-owned dogs with a biopsy-confirmed diagnosis consistent with CCUS were prescribed cyclosporine (5 mg/kg) for 1 week, followed by the addition of metronidazole (15-20 mg/kg), both administered orally once daily. The cyclosporine dosage interval was lengthened over time. Dogs were observed for a 6-month period and evaluated using a 32-point Canine Ulcerative Stomatitis Disease Activity Index (CUSDAI). Regular cyclosporine therapeutic drug monitoring was also conducted by the measurement of whole blood cyclosporine levels and the pharmacodynamic assessment of the T-cell expression of IL-2. The results demonstrated that a combination of cyclosporine and metronidazole was effective in minimizing the clinical signs of CCUS and in reducing CUSDAI scores. Neither blood cyclosporine levels nor the T-cell expression of IL-2 predicted improvement in clinical signs and CUSDAI scores, although there was a correlation between blood drug concentrations and the suppression of T-cell IL-2 expression. The evaluation of clinical signs and CUSDAI scores appears to be the most effective means of assessing response to therapy, and therapeutic drug level monitoring does not appear to be routinely indicated.

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.