Objectives The study sought to assess the impact of pregnancy on the rate of aortic growth as well as on short- and long-term clinical outcomes in women with Marfan syndrome. Background There is a ...paucity of data on peripartum and long-term clinical outcomes in women with Marfan syndrome who are followed prospectively during pregnancy. Methods Echocardiographic, demographic, and surgical data review of all adult females with a confirmed diagnosis of Marfan syndrome was performed. Results Of the 98 women identified, 69 (72%) experienced a total of 199 pregnancies resulting in 170 (86%) live births. The median number of pregnancies per women was 3 (interquartile range: 1 to 12). Obstetrical complications occurred in 17 (10%) and adverse fetal outcomes in 22 (13%). No woman experienced aortic dissection or required cardiac surgery during pregnancy. Aortic growth rate increased during pregnancy and did not return to baseline following pregnancy completion. Despite the lack of catastrophic peripartum complications, the prevalence of both aortic dissection and elective aortic surgery during long-term follow-up was higher in those women who had a prior pregnancy. Risk factors for adverse cardiac outcome included greater aortic diameter, greater rate of aortic growth during pregnancy, increased number of pregnancies, lack of beta-blocker use during pregnancy, and lack of prospective pregnancy follow-up. Conclusions There is a low incidence of aortic complications during pregnancy in women with Marfan syndrome and an aortic diameter <4.5 cm. However, pregnancy does increase the risk of aortic complications in the long-term in this group of patients.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
BACKGROUND: The interstage period after discharge from stage 1 palliation carries high morbidity and mortality. The impact of social determinants of health on interstage outcomes is not well ...characterized. We assessed the relationship between childhood opportunity and acute interstage outcomes. METHODS: Infants discharged home after stage 1 palliation in the National Pediatric Quality Improvement Collaborative Phase II registry (2016–2022) were retrospectively reviewed. Zip code–level Childhood Opportunity Index (COI), a composite metric of 29 indicators across education, health and environment, and socioeconomic domains, was used to classify patients into 5 COI levels. Acute interstage outcomes included death or transplant listing, unplanned readmission, intensive care unit admission, unplanned catheterization, and reoperation. The association between COI level and acute interstage outcomes was assessed using logistic regression with sequential adjustment for potential confounders. RESULTS: The analysis cohort included 1837 patients from 69 centers. Birth weight ( P <0.001) and proximity to a surgical center at birth ( P =0.02) increased with COI level. Stage 1 length of stay decreased ( P =0.001), and exclusive oral feeding rate at discharge increased ( P <0.001), with higher COI level. More than 98% of patients in all COI levels were enrolled in home monitoring. Death or transplant listing occurred in 101 (5%) patients with unplanned readmission in 987 (53%), intensive care unit admission in 448 (24%), catheterization in 345 (19%), and reoperation in 83 (5%). There was no difference in the incidence or time to occurrence of any acute interstage outcome among COI levels in unadjusted or adjusted analysis. There was no interaction between race and ethnicity and childhood opportunity in acute interstage outcomes. CONCLUSIONS: Zip code COI level is associated with differences in preoperative risk factors and stage 1 palliation hospitalization characteristics. Acute interstage outcomes, although common across the spectrum of childhood opportunity, are not associated with COI level in an era of highly prevalent home monitoring programs. The role of home monitoring in mitigating disparities during the interstage period merits further investigation.
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.
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.
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.
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.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery.
Children <6 years old at discharge after congenital heart ...surgery (Risk Adjustment for Congenital Heart Surgery-1 score 2-6) were identified using Pediatric Health Information System data (46 hospitals, 2004-2015). Residential distance from the surgery center, calculated using ZIP code centroids, was categorized as <15, 15-29, 30-59, 60-119, and ≥120 miles. Rurality was defined using rural–urban commuting area codes. Geographic risk factors for unplanned readmissions to the surgical center and associated burden (total hospital length of stay LOS, costs, and complications) were analyzed using multivariable regression.
Among 59 696 eligible children, 19 355 (32%) had ≥1 unplanned readmission. The median LOS was 9 days (IQR 22) across the entire cohort. In those readmitted, median total costs were $31 559 (IQR $90 176). Distance from the center was inversely related but rurality was positively related to readmission risk. Among those readmitted, increased distance was associated with longer LOS, more complications, and greater costs. Compared with urban patients, highly rural patients were more likely to have an unplanned readmission but had fewer average readmission days.
Geographic measures of access differentially affect readmission to the surgery center. Increased distance from the center was associated with fewer unplanned readmissions but more complications. Among those readmitted, the most isolated patients had the greatest readmission costs. Understanding the contribution of geographic access will aid in developing strategies to improve care delivery to this population.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic ...factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada.
In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ
score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers.
Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 95% CI, 0.64-0.85, quartile 2, 0.77 95% CI, 0.64-0.93, quartile 3, 0.83 95% CI, 0.69-1.00, quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 95% CI, 0.72-0.99) and rural residence (relative risk, 0.78 95% CI, 0.64-0.95) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND.
We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.
Recent studies suggest that suboptimal cardiac imaging on routine obstetric anatomy ultrasound (OB-scan) is not associated with a higher risk for congenital heart disease (CHD) and, therefore, should ...not be an indication for fetal echocardiography (F-echo). We aim to determine the incidence of CHD in patients referred for suboptimal imaging in a large catchment area, including regions that are geographically distant from a tertiary care center. We conducted a retrospective chart review of patients referred to Seattle Children's Hospital (SCH) and SCH Regional Cardiology sites (SCH-RC) from 2011 to 2021 for F-echo with the indication of suboptimal cardiac imaging by OB-scan. Of 454 patients referred for suboptimal imaging, 21 (5%) of patients were diagnosed with CHD confirmed on postnatal echo. 10 patients (2%) required intervention by age one. Mean GA at F-echo was significantly later for suboptimal imaging compared to all other referral indications (27.5 ± 3.9 vs 25.2 ± 5.2 weeks, p < 0.01). Mean GA at F-echo was also significantly later at SCH-RC compared to SCH (29.2 ± 4.6 vs 24.2 ± 2.9 weeks; p < 0.01). In our experience, CHD in patients referred for suboptimal imaging is higher (5%) than previously described, suggesting that routine referral for is warranted. Furthermore, while suboptimal imaging was associated with a delayed F-echo compared to other indications, this delay was most striking for those seen at regional sites. This demonstrates a potential disparity for these patients and highlights opportunities for targeted education in cardiac assessment for primary providers in these regions.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Newborn screening for critical congenital heart defects (CCHD) was added to the US Recommended Uniform Screening Panel in 2011. Within 4 years, 46 states and the District of Columbia had adopted it ...into their newborn screening program, leading to CCHD screening being nearly universal in the United States. This rapid adoption occurred while there were still questions about the effectiveness of the recommended screening protocol and barriers to follow-up for infants with a positive screen. In response, the Centers for Disease Control and Prevention partnered with the American Academy of Pediatrics to convene an expert panel between January and September 2015 representing a broad array of primary care, neonatology, pediatric cardiology, nursing, midwifery, public health, and advocacy communities. The panel's goal was to review current practices in newborn screening for CCHD and to identify opportunities for improvement. In this article, we describe the experience of CCHD screening in the United States with regard to: (1) identifying the target lesions for CCHD screening; (2) optimizing the algorithm for screening; (3) determining state-level challenges to implementation and surveillance of CCHD; (4) educating all stakeholders; (5) performing screening using the proper equipment and in a cost-effective manner; and (6) implementing screening in special settings such as the NICU, out-of-hospital settings, and areas of high altitude.
Many studies of racial and insurance disparities after congenital heart surgery have used limited regional data over short periods. This study examines the association of race and insurance with ...hospital mortality using a national hospitalization database spanning almost a decade. A retrospective, repeated cross-sectional analysis was performed. All the admissions from the Kids’ Inpatient Database from 1997 through 2006 that fit a Risk Adjustment for Congenital Heart Surgery-1 category were examined. Multivariate logistic regression models examining hospital mortality, nonelective admission, and referral to high-mortality hospitals were constructed. Medicaid insurance odds ratio (OR) 1.26, 95% confidence interval (CI) 1.09–1.46 and nonwhite race (OR 1.36, 95% CI 1.19–1.54) were independent risk factors for mortality. Furthermore, Medicaid insurance (OR 1.23, 95% CI 1.15–1.31) and nonwhite race (OR 1.26, 95% CI 1.19–1.34) were associated with nonelective admission for congenital heart surgery. Finally, children with Medicaid insurance (OR 1.18, 95% CI 1.10–1.27) and black children (OR 1.30, 95% CI 1.17–1.44) had higher odds of referral to high-mortality hospitals. Over the past decade, children undergoing congenital heart surgery continued to experience admission, referral, and survival disparities based on insurance and racial status.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Background There is an increasing focus on optimizing health care quality and reducing costs. The care of children undergoing heart surgery requires significant investment of resources, and it ...remains unclear how costs of care relate to quality. We evaluated this relationship across a multicenter cohort. Methods Clinical data from The Society of Thoracic Surgeons Database were merged with cost data from the Pediatric Health Information Systems Database for children undergoing heart surgery (2006 to 2010). Hospital-level costs were modeled using Bayesian hierarchical methods adjusting for case-mix, and hospitals were categorized into cost tertiles. The primary quality metric evaluated was in-hospital mortality. Results Overall, 27 hospitals (30,670 patients) were included. Median adjusted cost per case was $82,360 and varied fivefold across hospitals, while median adjusted mortality was 3.4% and ranged from 2.4% to 5.0% across hospitals. Overall, hospitals in the lowest cost tertile had significantly lower adjusted mortality rates compared with the middle and high cost tertiles (2.5% vs 3.8% and 3.5%, respectively, both p < 0.001). When assessed at the individual hospital level, most (75%) but not all hospitals in the lowest cost tertile were also in the lowest mortality tertile. Similar relationships were seen across the spectrum of surgical complexity. Lower cost hospitals also had shorter length of stay and trends toward fewer major complications. Conclusions Lowest cost hospitals generally deliver the highest quality care for children undergoing heart surgery, although there is some variation in this relationship. This information is important in the design of initiatives aiming to optimize health care value in this population.
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