Regional Differences in Cost and Length of Stay in Neonates with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) is a highly resource-intensive diagnosis. Geographic variation in cost and length of stay (LOS) in HLHS is not well described. Neonates diagnosed with HLHS between 2000 and 2012 were identified using the Kids' Inpatient Database. Hospitalizations were stratified into two groups: (1) birth and (2) secondary. United States regional differences in hospital charges and LOS were compared using adjusted linear regression. Of 2431 birth hospitalizations, 449 neonates (18.5%) died while inpatient and mortality rates differed by region (p = 0.02). After birth, 40.5% (n = 985) of neonates were transferred; transfers were most common in the Midwest (p < 0.0001). Adjusted average LOS was shortest in the West and longest in the South (26.1 days; 95% CI 24.0, 35.1 vs. 34.9 days; 95% CI 31.8, 38.1). Average adjusted charges were lowest in the Northeast ($324,600; 95% CI $271,400, $377,900) and highest in the West ($400,500; 95% CI $346,700, $454,300, p = 0.05). Among 1895 secondary hospitalizations, 24.9% of neonates died as inpatients, and the average adjusted LOS was shortest in the West (26.8 days; 95% CI 23.9, 29.7) and longest in the South (38.5 days; 95% CI 34.4, 42.4). Average adjusted charges were lowest in the Northeast ($326,900; 95% CI $270,700, $383,100) and highest in the South ($505,900; 95% CI $450,200, $561,500, p < 0.0001). Significant geographic variations in mortality, LOS, and hospital charges exist in care of US HLHS neonates. Reducing variation in care should remain a priority in national quality efforts in congenital heart disease.

Feasibility of a healthcare system-based tetralogy of Fallot patient registry.

BACKGROUND: Patient-reported outcomes and epidemiological studies in adults with tetralogy of Fallot are lacking. Recruitment and longitudinal follow-up investigation across institutions is particularly challenging. Objectives of this study were to assess the feasibility of recruiting adult patients with tetralogy of Fallot for a patient-reported outcomes study, describe challenges for recruitment, and create an interactive, online tetralogy of Fallot registry. METHODS: Adult patients living with tetralogy of Fallot, aged 18-58 years, at the University of North Carolina were identified using diagnosis code query. A survey was designed to collect demographics, symptoms, history, and birth mother information. Recruitment was attempted by phone (Part I, n=20) or by email (Part II, n=20). Data analysis included thematic grouping of recruitment challenges and descriptive statistics. Feasibility threshold was 75% for recruitment and for data fields completed per patient. RESULTS: In Part I, 60% (12/20) were successfully contacted and eight (40%) were enrolled. Demographics and birth mother information were obtained for all enrolled patients. In Part II, 70% (14/20) were successfully contacted; 30% (6/20) enrolled and completed all data fields linked to REDCap database; the median time for survey completion was 8 minutes. Half of the patients had cardiac operations/procedures performed at more than one hospital. Automatic electronic data entry from the online survey was uncomplicated. CONCLUSIONS: Although recruitment (54%) fell below our feasibility threshold, enrolled individuals were willing to complete phone or online surveys. Incorrect contact information, privacy concerns, and patient-reported time constraints were challenges for recruitment. Creating an online survey and linked database is technically feasible and efficient for patient-reported outcomes research.

Effect of Concomitant Birth Defects and Genetic Anomalies on Infant Mortality in Tetralogy of Fallot.

BACKGROUND: A substantial proportion of infants born with tetralogy of Fallot (TOF) die in infancy. A better understanding of the heterogeneity associated with TOF, including extracardiac malformations and chromosomal anomalies is vital to stratifying risk and optimizing outcomes during infancy. METHODS: Using the North Carolina Birth Defects Monitoring Program, infants diagnosed with TOF and born between 2003 and 2012 were included. Kaplan-Meier survival curves were used to estimate cumulative 1-year mortality, stratified by the presence of concomitant birth defects (BDs) and chromosomal anomalies. Multivariable logistic regression was used to estimate the direct effect of each concomitant BD, after adjusting for all others. RESULTS: A total of 496 infants with TOF were included, and 15% (n = 76) died. The number of concomitant BD systems was significantly associated with the risk of death at 1-year, p < 0.0001. Specifically, the risk of mortality was 8% among infants with TOF with or without additional cardiac defects, 16% among infants with TOF and 1 extracardiac BD system, 19% among infants with 2 extracardiac BD systems, and 39% among infants with ≥ 3 extracardiac BD systems. After adjustment, concomitant eye and gastrointestinal defects were significantly associated increased with 1-year mortality, odds ratio 2.83 (95% confidence interval, 1.08-7.32) and odds ratio 4.43 (95% confidence interval, 1.57, 12.45), respectively. Infants with trisomy 13 or trisomy 18 were also significantly more likely to die, p < 0.0001. CONCLUSION: Both concomitant BDs and genetic anomalies increase the risk of mortality among infants with TOF. Future studies are needed to identify the underlying genetic and socioeconomic risk factors for high-risk TOF infants. Birth Defects Research 109:1154-1165, 2017. © 2017 Wiley Periodicals, Inc.