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Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require significant operations shortly after birth. Few studies have examined the impact of these combined lesions. We sought to examine the incidence of CHD in patients with MMC, and to evaluate length of stay (LOS), hospital charges, and mortality. Using the Texas Inpatient Public Use Data File, ~ 6.9 million newborn records between 1/1999 and 12/2016 were examined. Hospitalizations were classified as MMC without CHD (n = 3054), CHD without MMC (n = 72,266), and MMC with CHD (n = 171). The birth prevalence of CHD with MMC was 0.3/10,000 live hospital births, with 5% of patients with MMC having CHD, and 0.2% of those with CHD having MMC. There was increased LOS in patients with both MMC and CHD (median 15 days, IQR 5–31), compared to CHD without MMC (median 6 days, IQR 2–20) and MMC without CHD (median 8 days, IQR 1–14) and higher total hospital charges (median $95,007, IQR $26,731–$222,660) compared to CHD without MMC (median $27,726, $6463–$118,370) and MMC without CHD (median $40,066, IQR $5744–$97,490). Mortality was significantly higher in patients with MMC and CHD (22.2% compared to 3.1% in MMC without CHD and 4.1% in CHD without MMC). Significance remained when limiting for patients without genetic conditions or additional major birth defects. MMC with CHD in the newborn compared to either CHD or MMC alone is associated with longer LOS, higher charges, and increased mortality. [ABSTRACT FROM AUTHOR]

Epidemiologic research in pediatric heart failure (HF) is limited, focused on hospitalized children identified from administrative databases. Challenges in HF case ascertainment and lack of outpatient data constrain the estimates of HF incidence. Aims: to estimate a regional incidence of pediatric HF and describe cohort characteristics. In an observational retrospective cohort, children with HF were identified from the Electronic Data Warehouse (EDW) of a tertiary referral center 01/2010-12/2020. Inclusion criteria: Utah address; 31 days - 19 years old at receipt of a HF ICD code. Patients were included if assigned 2 or more HF ICD codes or assigned a code in an inpatient encounter and treated with a HF-specific medication; methods previously described. EDW data were linked with the Society of Thoracic Surgeons National Database. Patients undergoing VSD or AVSD repair, PDA closure were eligible only if HF ICD codes were assigned post-procedure. 763 patients were included. Chart review of 37% (282/763) demonstrated 80% diagnostic accuracy in identifying HF patients. Based on census data, the incidence of HF was 7.0 per 100,000 patient-years (95% CI 6.5, 7.5). Nearly 20% (152) of the cohort was diagnosed with HF while outpatient and 25% (38/152) were not hospitalized during follow-up. Congenital heart disease (CHD) was most prevalent (464; 61%). Patients had a median of 3 comorbidities at the time of HF diagnosis (IQR 2,4). Median time to hospitalization/rehospitalization following diagnosis was 2.4 months (IQR 1.2,7.2), similar across all subgroups: CHD, cardiomyopathy (CM), CHD+CM, other diagnoses (p= 0.57). Mechanical support was required by 106 (14%) and heart transplant (tx) by 46 (6%) patients. Median time to tx was shortest among CM patients (p<0.001). There were 140 deaths (18%), most taking place during the index HF admission. Younger age at diagnosis, increased number of comorbidities, simple biventricular CHD vs CM were associated with risk of hospitalization (p=0.029, <0.001, 0.035 respectively). Diagnoses of hypertrophic cardiomyopathy or complex biventricular CHD were associated with risk of death or tx (p< 0.028, 0.22 respectively). This contemporary study describes the estimated regional incidence of pediatric HF using granular single-center data. Risk of mortality and rehospitalization are confirmed to be high in this population. [ABSTRACT FROM AUTHOR]