Prenatally diagnosed congenital heart disease: the cost of maternal care.

OBJECTIVE: Little is known regarding the effects of a prenatal diagnosis of congenital heart disease (CHD) on the cost of antenatal and delivery care. We sought to compare the maternal costs of care in pregnancies where the fetus or child was diagnosed prenatally vs. postnatally. METHODS: Costs of maternal care were determined for pregnancies in which the fetus or child was diagnosed with CHD between 1997 and 2012 in the state of Utah. Cases of CHD were identified via a statewide birth defect surveillance program which included data on the timing of diagnosis, maternal demographic and clinical data, and linked to statewide inpatient maternal hospital discharge records. Antenatal testing costs were determined using Medicaid fee estimates and total facility costs were determined for all hospitalizations including delivery. The association of timing of diagnosis of CHD with costs was analyzed using univariable and multivariable models. RESULTS: Of 2128 pregnancies included in the study, 36% had a fetus prenatally diagnosed with CHD. The prenatal diagnosis group was more likely to have a termination or stillbirth and were younger at delivery (gestational age 37.3 vs 38.0 weeks, p < .001). Labor induction and cesarean delivery rates were similar between groups. Antenatal testing and delivery hospitalization costs were higher in the prenatal diagnosis group: $5819 vs $4041 (p < .001) and $10,509 vs $7802 (p < .001), respectively. Patients in the prenatal diagnosis group had longer lengths of hospital stays (3.5 vs 2.4 d, p > .001). After controlling for significant differences between the groups, including lesion severity, the prenatal diagnosis remained directly associated with antenatal testing costs (+$1472), maternal hospitalization costs (+$2713), and maternal hospital length of stay (+1.0 d). CONCLUSION: A prenatal diagnosis of fetal CHD was associated with increased prenatal costs, hospitalization costs, and hospital length of stay for affected pregnant patients.

Implications of Left Ventricular Dysfunction at Presentation for Infants with Coarctation of the Aorta.

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants < 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) < 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p < 0.001), and more ventilator days (3.5 vs. 1, p < 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, > 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

Factors That Contribute to Cost Differences Based on ICU of Admission in Neonates Undergoing Congenital Heart Surgery: A Novel Decomposition Analysis.

OBJECTIVES: We leveraged decomposition analysis, commonly used in labor economics, to understand determinants of cost differences related to location of admission in children undergoing neonatal congenital heart surgery. DESIGN: A retrospective cohort study. SETTING: Pediatric Health Information Systems database. PATIENTS: Neonates (<30 d old) undergoing their index congenital heart surgery between 2004 and 2013. MEASUREMENTS AND MAIN RESULTS: A decomposition analysis with bootstrapping determined characteristic (explainable by differing covariate levels) and structural effects (if covariates are held constant) related to cost differences. Covariates included center volume, age at admission, prematurity, sex, race, genetic or major noncardiac abnormality, Risk Adjustment for Congenital Heart Surgery-1 score, payor, admission year, cardiac arrest, infection, and delayed sternal closure.Of 19,984 infants included (10,491 [52%] to cardiac ICU/PICU and 9,493 [48%] to neonatal ICU), admission to the neonatal ICU had overall higher average costs ($24,959 ± $3,260; p < 0.001) versus cardiac ICU/PICU admission. Characteristic effects accounted for higher costs in the neonatal ICU ($28,958 ± $2,044; p < 0.001). Differing levels of prematurity, genetic syndromes, hospital volume, age at admission, and infection contributed to higher neonatal ICU costs, with infection rate providing the most significant contribution ($13,581; p < 0.001). Aggregate structural effects were not associated with cost differences for those admitted to the neonatal ICU versus cardiac ICU/PICU (p = 0.1). Individually, prematurity and age at admission were associated with higher costs due to structural effects for infants admitted to the neonatal ICU versus cardiac ICU/PICU. CONCLUSIONS: The difference in cost between neonatal ICU and cardiac ICU/PICU admissions is largely driven by differing prevalence of risk factors between these units. Infection rate was a modifiable factor that accounted for the largest difference in costs between admitting units.

Early Childhood Inpatient Costs of Critical Congenital Heart Disease.

OBJECTIVE: To assess longitudinal estimates of inpatient costs through early childhood in patients with critical congenital heart defects (CCHDs), for whom reliable estimates are scarce, using a population-based cohort of clinically validated CCHD cases. STUDY DESIGN: Longitudinal retrospective cohort of infants with CCHDs live born from 1997 to 2012 in Utah. Cases identified from birth defect registry data were linked to inpatient discharge abstracts and vital records to track inpatient days and costs through age 10 years. Costs were adjusted for inflation and discounted by 3% per year to generate present value estimates. Multivariable models identified infant and maternal factors potentially associated with higher resource utilization and were used to calculate adjusted costs by defect type. RESULTS: The final statewide cohort included 1439 CCHD cases among 803 509 livebirths (1.8/1000). The average cost per affected child through age 10 years was $136 682 with a median of $74 924 because of a small number of extremely high cost children; costs were highest for pulmonary atresia with ventricular septal defect and hypoplastic left heart syndrome. Inpatient costs increased by 1.6% per year during the study period. A single birth year cohort (~50 000 births/year) had estimated expenditures of $11 902 899 through age 10 years. Extrapolating to the US population, inpatient costs for a single birth year cohort through age 10 years were ~$1 billion. CONCLUSIONS: Inpatient costs for CCHDs throughout childhood are high and rising. These revised estimates will contribute to comparative effectiveness research aimed at improving the value of care on a patient and population level.

Electrocardiograms in Healthy North American Children in the Digital Age.

BACKGROUND: Interpretation of pediatric ECGs is limited by lack of accurate sex- and race-specific normal reference values obtained with modern technology for all ages. We sought to obtain contemporary digital ECG measurements in healthy children from North America, to evaluate the effects of sex and race, and to compare our results to commonly used published datasets. METHODS: Digital ECGs (12-lead) were retrospectively collected for children ≤18 years old with normal echocardiograms at 19 centers in the Pediatric Heart Network. Patients were classified into 36 groups: 6 age, 2 sex, and 3 race (white, black, and other/mixed) categories. Standard intervals and amplitudes were measured; mean±SD and 2nd/98th percentiles were determined by age group, sex, and race. For each parameter, multivariable analysis, stratified by age, was conducted using sex and race as predictors. Parameters were compared with 2 large pediatric ECG data sets. RESULTS: Among ECGs from 2400 children, significant differences were found by sex and race categories. The corrected QT interval in lead II was greater for girls compared with boys for age groups ≥3 years (P≤0.03) and for whites compared with blacks for age groups ≥12 years (P<0.05). The R wave amplitude in V6 was greater for boys compared with girls for age groups ≥12 years (P<0.001), for blacks compared with white or other race categories for age groups ≥3 years (P≤0.006), and greater compared with a commonly used public data set for age groups ≥12 years (P<0.0001). CONCLUSIONS: In this large, diverse cohort of healthy children, most ECG intervals and amplitudes varied by sex and race. These differences have important implications for interpreting pediatric ECGs in the modern era when used for diagnosis or screening, including thresholds for left ventricular hypertrophy.

Assessment of Growth 6 Years after the Norwood Procedure.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00115934.

Surgical volume, hospital quality, and hospitalization cost in congenital heart surgery in the United States.

Hospital volume has been associated with improved outcomes in congenital cardiac surgery. However, the relationship between hospital volume and hospitalization cost remains unclear. This study examines the relationship between hospital surgical volume and hospitalization costs, while accounting for measures of quality, in children undergoing congenital heart surgery. A retrospective, repeated cross-sectional analysis was performed, using discharges from the 2006 and 2009 Kids' Inpatient Database. All pediatric admissions (<18 years) with a Risk Adjustment for Congenital Heart Surgery procedure and hospitalization cost/charge data were included. Multivariate, linear mixed regression models were run on hospitalization costs, with and without adjustment for indicators of quality (hospital mortality rate and complication rate). Both medium and high-volume hospitals (200-400 cases/year and >400 cases/year, respectively) were associated with lower odds of mortality but not occurrence of a complication. Hospital mortality was associated with the largest increase in hospitalization costs. High-volume hospitals (>400 cases/year) were associated with the lowest hospitalization costs per discharge ($37,775, p < 0.01) when compared to low-($43,270) and medium($41,085)-volume hospitals, prior to adjusting for quality indicators. However, when adjusting for hospital mortality rate, high-volume hospitals no longer demonstrated significant cost savings. When adjusting for hospital complication rate, high-volume hospitals continued to have the lowest hospitalization costs. High-volume hospitals are associated with a reduction in hospitalization costs that appear to be mediated through improvements in quality.

Admission to a dedicated cardiac intensive care unit is associated with decreased resource use for infants with prenatally diagnosed congenital heart disease.

Many factors in the delivery and perinatal care of infants with a prenatal diagnosis of congenital heart disease (CHD) have an impact on outcome and costs. This study sought to determine the modifiable factors in perinatal management that have an impact on postnatal resource use for infants with CHD. The medical records of infants with prenatally diagnosed CHD (August 2006-December 2011) who underwent cardiac surgery before discharge were reviewed. The exclusion criteria ruled out prematurity and intervention or transplantation evaluation before surgery. Clinical characteristics, outcomes, and cost data were collected. Multivariate linear regression models were used to determine the impact of perinatal decisions on hospitalization cost and surrogates of resource use after adjustment for demographic and other risk factors. For the 126 patients who met the study criteria, the median hospital stay was 22 days (range 4-122 days), and the median inflation-adjusted total hospital cost was $107,357 (range $9,746-602,320). The initial admission to the neonatal versus the cardiac intensive care unit (NICU vs. CICU) was independently associated with a 19 % longer hospital stay, a 26 % longer ICU stay, and 47 % more mechanical ventilation days after adjustment for Risk Adjustment for Congenital Heart Surgery, version 1 score, gestation age, genetic abnormality, birth weight, mode of delivery, and postsurgical complications. Weekend versus weekday delivery was not associated with hospital cost or length of hospital stay. For term infants with prenatally diagnosed CHD undergoing surgery before discharge, preoperative admission to the NICU (vs. the CICU) resulted in a longer hospital stay and greater intensive care use. Prenatal planning for infants with CHD should consider the initial place of admission as a modifiable factor for potential lowering of resource use.

Longitudinal assessment of growth in hypoplastic left heart syndrome: results from the single ventricle reconstruction trial.

BACKGROUND: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. METHODS AND RESULTS: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients <37 weeks gestation (N=498). We determined length-for-age z score (LAZ) and weight-for-age z score (WAZ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth (P<0.001, P=0.05, respectively) and age 3 years (P<0.001 each). The largest decrease in WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were <-2 in 6% and 18%, respectively. Factors associated with change in WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock-Taussig shunt had a greater decline in WAZ than those with a right ventricle-pulmonary artery shunt (P=0.002). CONCLUSIONS: WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (>2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient- and stage-specific targets. CLINICAL TRIAL REGISTRATION URL: http://clinicaltrials.gov/. Unique identifier: NCT00115934.

Challenges in echocardiographic assessment of mitral regurgitation in children after repair of atrioventricular septal defect.

The validity and reproducibility of echocardiographic methods used to quantify mitral regurgitation (MR) in children with congenital heart disease are unknown. We evaluated the usefulness of methods used to quantify MR in children enrolled in a multicenter trial of enalapril 6 months after surgical repair of an atrioventricular septal defect (AVSD). MR severity in this trial was assessed using body surface area (BSA)-adjusted vena contracta lateral (i-VCW(lat)) and anterior-posterior (i-VCW(ap)) dimensions and cross-sectional area (i-VCA), regurgitant volume/BSA, regurgitant fraction, and qualitative MR grade. For each method, association with left ventricular end-diastolic volume (LVEDVz) and end-diastolic dimension (LVEDDz) z-scores and interobserver agreement were assessed. In 149 children (median age 1 year), i-VCW(lat), i-VCW(ap), and i-VCA were best associated with LVEDVz (r (2) = 0.54, r (2) = 0.24, and r (2) = 0.46, respectively; p < 0.001 for all) and showed the highest interobserver agreement (intraclass correlation coefficient = 0.62, 0.73, and 0.68, respectively). Qualitative MR grade was also associated with LVEDVz (r (2) = 0.31, p < 0.001) and showed modest interobserver agreement (kappa 0.56). Regurgitant volume/BSA and regurgitant fraction were associated with LVEDVz (r (2) = 0.45 and r (2) = 0.45, p < 0.001 for both) but showed poor interobserver agreement [ICC = 0.28 (n = 91) and ICC = 0.17 (n = 76), respectively], and their values were negative in 75% of subjects. In conclusion, echocardiographic assessment of MR severity after AVSD remains challenging. Among the quantitative methods used in this trial, i-VCW and i-VCA performed the best but offered little advantage compared with qualitative MR grade. The utility of regurgitant volume and fraction was severely limited by poor interobserver agreement and frequently negative values.

An assessment of the electrocardiogram as a screening test for large atrial septal defects in children.

BACKGROUND AND PURPOSE: The electrocardiogram (ECG) is commonly used as a screening tool for diagnosis of the ostium secundum atrial septal defect (ASD). We sought to analyze the utility of conventional ECG criteria in detecting right ventricular enlargement (RVE) due to the presence of an ASD. METHODS: Patients who underwent transcatheter or surgical closure of an isolated ASD between 1997 and 2004 were included if an ECG was performed less than 9 months before ASD closure and had echocardiographic RVE. RESULTS: Of 99 children (aged 6.8 +/- 4.7 years; range, 1-18 years) with RVE and ASD, 57% had an ECG that met 1 or more RVE criteria. The sensitivity of ECG increased to 70% in younger patients and to 80% for the largest defects. CONCLUSIONS: Electrocardiographic criteria for RVE are present in just more than over half of young patients with large ASDs. Although ECG is more sensitive in younger patients, it is unreliable as a screen for this lesion.

Assessment of right ventricular size and function: echo versus magnetic resonance imaging.

OBJECTIVE: The assessment of right ventricular (RV) size and function is important in the management of many patients with heart disease. Although magnetic resonance imaging (MRI) is considered the gold standard for quantitation of ventricular volumes and systolic function, subjective assessment ("eyeball") by echocardiography is the modality most often used for the RV. The echocardiographic "eyeball" method of assessing RV size and systolic function was compared with quantitative MRI. DESIGN: Patients with right-sided congenital heart disease who underwent an echocardiogram within 6 months of MRI formed the study group. Four echocardiographers blinded to the MRI results reviewed the echocardiograms to subjectively assess RV size and systolic function. The reliability of an echocardiographer in accurately identifying a severely dilated RV and moderately to severely diminished RV systolic function was measured using the Kappa coefficient. Inter-rater agreement was also assessed using Kappa. RESULTS: The study group consisted of 22 patients aged 16.6 +/- 7.1 years, with interval between MRI and echocardiogram of 49 +/- 54 days. Using echocardiography, reliability for accurately identifying a severely dilated RV was "slight" with a prevalence-adjusted bias-adjusted Kappa (PABAK) of 0.25; and for identifying moderately to severely diminished RV systolic function was fair with a PABAK of 0.43. Inter-rater agreement analysis was poor for both with Kappas of 0.07 (P = .22) and 0.12 (P = .09), respectively. CONCLUSION: The usefulness of the echocardiographic "eyeball" method to estimate RV size and systolic function in patients with right heart disease has limitations when compared with MRI, specifically in regard to the variability between echocardiographers.