Lifetime Burden of Adult Congenital Heart Disease in the USA Using a Microsimulation Model.

Congenital heart defects (CHD) represent a growing burden of illness among adults. We estimated the lifetime health, education, labor, and social outcomes of adults with CHD in the USA using the Future Adult Model, a dynamic microsimulation model that has been used to study the lifetime impacts of a variety of chronic diseases. We simulated a cohort of adult heads of households > 25 years old derived from the Panel Survey of Income Dynamics who reported a childhood heart problem as a proxy for CHD and calculated life expectancy, disability-free and quality-adjusted life years, lifetime earnings, education attainment, employment, development of chronic disease, medical spending, and disability insurance claiming status. Total burden of disease was estimated by comparing to a healthy cohort with no childhood heart problem. Eighty-seven individuals reporting a childhood heart problem were identified from the PSID and were used to generate the synthetic cohort simulated in the model. Life expectancy, disability-free, quality-adjusted, and discounted quality-adjusted life years were an average 4.6, 6.7, 5.3, and 1.4 years lower than in healthy adults. Lung disease, cancer, and severe mental distress were more common compared to healthy individuals. The CHD cohort earned $237,800 less in lifetime earnings and incurred higher average total medical spend by $66,600 compared to healthy individuals. Compared to healthy adults, the total burden of CHD is over $500K per adult. Despite being among the healthiest adults with CHD, there are significant decrements in life expectancy, employment, and lifetime earnings, with concomitant increases in medical spend.

Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery.

The objective of this analysis was to update trends in LOS and costs by survivorship and ECMO use among neonates with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation surgery using 2016 data from the Healthcare Cost and Utilization Project Kids' Inpatient Database. We identified neonates ≤ 28 days old with HLHS undergoing Stage 1 surgery, defined as a Norwood procedure with modified Blalock-Taussig (BT) shunt, Sano modification, or both. Multivariable regression with year random effects was used to compare LOS and costs by hospital region, case volume, survivorship, and ECMO vs. no ECMO. An E-value analysis, an approach for conducting sensitivity analysis for unmeasured confounding, was performed to determine if unmeasured confounding contributed to the observed effects. Significant differences in total costs, LOS, and mortality were noted by hospital region, ECMO use, and sub-analyses of case volume. However, other than ECMO use and mortality, the maximum E-value confidence interval bound was 1.71, suggesting that these differences would disappear with an unmeasured confounder 1.71 times more associated with both the outcome and exposure (e.g., socioeconomic factors, environment, etc.) Our findings confirm previous literature demonstrating significant resource utilization among Norwood patients, particularly those undergoing ECMO use. Based on our E-value analysis, differences by hospital region and case volume can be explained by moderate unobserved confounding, rather than a reflection of the quality of care provided. Future analyses on surgical quality must account for unobserved factors to provide meaningful information for quality improvement.

Short-term outcomes following implementation of a dedicated young adult congenital heart disease transition program.

OBJECTIVE: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease. DESIGN, SETTING, AND PATIENTS: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively. INTERVENTION AND OUTCOME MEASURES: Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer. RESULTS: Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P = .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P = .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted. CONCLUSIONS: Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes.

Inpatient admissions and costs of congenital heart disease from adolescence to young adulthood.

BACKGROUND: Health care utilization patterns and associated costs of congenital heart disease (CHD) in young adulthood are not well understood. METHODS: California State Inpatient Databases 2005 to 2009 were used to conduct a retrospective study on inpatient admissions of CHD patients 10 to 29 years old (n = 7,419) and of all patients of the same age (n = 1,195,233). RESULTS: Compared with all patients, annual admission costs of CHD patients monotonically decreased, from $13.9 million at age 10 to 14 years to $7.2 million at age 25 to 29 years, which were due to lower costs per admission and somewhat fewer annual hospitalizations. Admissions from the emergency department in CHD patients increased with age regardless of insurance status: at age 25 to 29 years, 62% of admissions with public insurance, 43% with private insurance, and 78% with no insurance were admitted from the emergency department. Cardiac surgery, catheterization, and electrophysiologic study admissions decreased with age in CHD patients, whereas admissions due to arrhythmia and congestive heart failures became more prevalent. Results from multivariate linear regression in CHD patients showed that cardiac surgery was the most costly factor, associated with $29,543 more in costs per admission, followed by the use of a children's hospital, at $10,533. Factors predicting lower costs included female gender, low-complexity CHD, and shorter stay, all P < .001. CONCLUSIONS: Compared with adolescents, young adults with CHD use fewer resources because the natural history of CHD results in fewer surgical admissions and more frequent but less expensive medical admissions.

Caring for ACHD in a market-driven society.

Our cardiology community is responding to the growing number of emerging adults with often complex congenital heart disease. Collaborations are springing up between adult and pediatric cardiologists, advanced practice nurses, patients, and families to address the health care, research, and advocacy needs for this population. Workforce and institutional needs are being defined and research collaborations are being formed. Meanwhile, health care reform is evolving through fits and starts with little predictability regarding its medium and long-term impact. Since ultimately finances trump philosophy, it is essential that we understand the financial underpinnings of healthcare delivery to patients with this unique model of chronic disease in order to carry out these plans. What is unique about this population with chronic disease? The most obvious feature is that they have the potential of contributing to the GDP for 40+ years. Another is that for the more complex lesions, society has already invested a considerable amount to achieve survival into adult life. Finally, the period of early adulthood is relatively uneventful in terms of complications and resource utilization compared with early childhood and later adult life. Thus, the basic needs to maintain cardiovascular status and prevent secondary disability may be modest in comparison with treating some of the severe consequences of their disease, such as poorly managed valve regurgitation or arrhythmia that eventually requires costly solutions such as transplantation. It is important, therefore, to define the resource requirements and potential health outcomes of a healthcare system that would be designed for this population.

Adults with chronic health conditions originating in childhood: inpatient experience in children's hospitals.

OBJECTIVE: To describe the rate of increase of the population of adults seeking care as inpatients in children's hospitals over time. PATIENTS AND METHODS: We analyzed data from January 1, 1999, to December 31, 2008, from patients hospitalized at 30 academic children's hospitals, including growth rates according to age group (pediatric: aged <18 years; transitional: aged 18-21 years; or adult: aged >21 years) and disease. RESULTS: There were 3 343 194 hospital discharges for 2 143 696 patients. Transitional patients represented 2.0%, and adults represented 0.8%, totaling 59 974 patients older than 18 years. The number of unique patients, admissions, patient-days, and charges increased in all age groups over the study period and are projected to continue to increase. Resource use was disproportionately higher in the older ages. The growth of transitional patients exceeded that of others, with 6.9% average annual increase in discharges, 7.6% in patient-days, and 15% in charges. Chronic conditions occurred in 87% of adults compared with 48% of pediatric patients. Compared with pediatric patients, the rates of increase of inpatient-days increased significantly for transitional age patients with cystic fibrosis, malignant neoplasms, and epilepsy, and for adults with cerebral palsy. Annual growth rates of charges increased for transitional and adult patients for all diagnoses except cystic fibrosis and sickle cell disease. CONCLUSIONS: The population of adults with diseases originating in childhood who are hospitalized at children's hospitals is increasing, with varying disease-specific changes over time. Our findings underscore the need for proactive identification of strategies to care for adult survivors of pediatric diseases.

Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

TACE is required for fetal murine cardiac development and modeling.

Tumor necrosis factor-alpha converting enzyme (TACE) is a membrane-anchored, Zn-dependent metalloprotease, which belongs to the ADAM (a disintegrin and metalloprotease) family. TACE functions as a membrane sheddase to release the ectodomain portions of many transmembrane proteins, including the precursors of TNFalpha, TGFalpha, several other cytokines, as well as the receptors for TNFalpha, and neuregulin (ErbB4). Mice with TACE(DeltaZn/DeltaZn) null mutation die at birth with phenotypic changes, including failure of eyelid fusion, hair and skin defects, and abnormalities of lung development. Abnormal fetal heart development was not previously described. Herein, we report that TACE(DeltaZn/DeltaZn) null mutant mice by late gestation exhibit markedly enlarged fetal hearts with increased myocardial trabeculation and reduced cell compaction, mimicking the pathological changes of noncompaction of ventricular myocardium. In addition, larger cardiomyocyte cell size and increased cell proliferation were observed in ventricles of TACE(DeltaZn/DeltaZn) knockout mouse hearts. At the molecular level, reduced expression of epidermal growth factor receptor, attenuated protein cleavage of ErbB4, and changes in MAPK activation were also detected in TACE(DeltaZn/DeltaZn) knockout heart tissues. The data suggest that TACE-mediated cell surface protein ectodomain shedding plays an essential and a novel regulatory role during cardiac development and modeling.