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Introduction: Congenital heart disease (CHD) is the most prevalent congenital disability globally. This study aimed to describe parents' perspectives on financial stressors related to having a child with CHD using a descriptive qualitative approach., Method: Qualitative data were obtained from parents of children with CHD in a cross-sectional web-based survey study. Iterative data analysis was used to develop essential themes that enabled a rich description of 147 parents' perspectives., Results: Parents identified five financial stressors: perpetual worries about health insurance, facing the dilemma of "making too much money," struggling to balance work, worrying over having an emerging adult with CHD, and constant constraints because of financial needs., Discussion: As experts in pediatric care, pediatric advanced practice providers need to work with policymakers to provide further financial assistance and sufficient insurance coverage for families that struggle to balance finances for the whole family and children with CHD., Competing Interests: CONFLICTS OF INTEREST None to report., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Importance: Individuals with congenital heart disease (CHD) are increasingly reaching childbearing age, are more prone to adverse pregnancy events, and uncommonly undergo recommended cardiac evaluations. Data to better understand resource allocation and financial planning are lacking., Objective: To examine health care use and costs for patients with CHD during pregnancy., Design, Setting, and Participants: This retrospective cohort study was performed from January 1, 2010, to December 31, 2016, using Merative MarketScan commercial insurance data. Participants included patients with CHD and those without CHD matched 1:1 by age, sex, and insurance enrollment year. Pregnancy claims were identified for all participants. Data were analyzed from September 2022 to March 2024., Exposures: Baseline characteristics (age, US region, delivery year, insurance type) and pregnancy-related events (obstetric, cardiac, and noncardiac conditions; birth outcomes; and cesarean delivery)., Main Outcomes and Measures: Health service use (outpatient physician, nonphysician, emergency department, prescription drugs, and admissions) and costs (total and out-of-pocket costs adjusted for inflation to represent 2024 US dollars)., Results: A total of 11 703 pregnancies (mean [SD] maternal age, 31.5 [5.4] years) were studied, with 2267 pregnancies in 1785 patients with CHD (492 pregnancies in patients with severe CHD and 1775 in patients with nonsevere CHD) and 9436 pregnancies in 7720 patients without CHD. Compared with patients without CHD, pregnancies in patients with CHD were associated with significantly higher health care use (standardized mean difference [SMD] range, 0.16-1.46) and cost (SMD range, 0.14-0.55) except for out-of-pocket inpatient and ED costs. After adjustment for covariates, having CHD was independently associated with higher total (adjusted cost ratio, 1.70; 95% CI, 1.57-1.84) and out-of-pocket (adjusted cost ratio, 1.40; 95% CI, 1.22-1.58) costs. The adjusted mean total costs per pregnancy were $15 971 (95% CI, $15 480-$16 461) for patients without CHD, $24 290 (95% CI, $22 773-$25 806) for patients with any CHD, $26 308 (95% CI, $22 788-$29 828) for patients with severe CHD, and $23 750 (95% CI, $22 110-$25 390) for patients with nonsevere CHD. Patients with vs without CHD incurred $8319 and $700 higher total and out-of-pocket costs per pregnancy, respectively., Conclusions and Relevance: This study provides novel, clinically relevant estimates for the cardio-obstetric team, patients with CHD, payers, and policymakers regarding health care and financial planning. These estimates can be used to carefully plan for and advocate for the comprehensive resources needed to care for patients with CHD.

Background: The Physician Payments Sunshine Act was enacted to understand financial relationships with industry that might influence provider decisions. We investigated how industry payments within the congenital heart community relate to experience and reputation., Methods: Congenital cardiothoracic surgeons and pediatric cardiologists were identified from the Open Payments Database. All payments from 2013 through 2017 were matched to affiliated hospitals' U.S. News & World Report (USNWR) rankings, The Society of Thoracic Surgeons-Congenital Heart Surgery Public Reporting Star Ratings, and Optum Center of Excellence (COE) designation. Surgeon payments were linked to years since terminal training. Univariable analyses were conducted., Results: The median payment amount per surgeon ($71; interquartile range [IQR], $41-$99) was nearly double the median payment amount per cardiologist ($41; IQR, $18-$84; P < .05). For surgeons, median individual payment was 56% higher to payees at USNWR top 10 children's hospitals ($100; IQR, $28-$203) vs all others ($64; IQR, $23-$140; P < .001). For cardiologists, median individual payment was 26% higher to payees at USNWR top 10 children's hospitals ($73; IQR, $28-$197) vs all others ($58; IQR, $19-$140; P < .001). Findings were similar across The Society of Thoracic Surgeons-Congenital Heart Surgery star rankings and Optum Center of Excellence groups. By surgeon experience, surgeons 0 to 6 years posttraining (first quartile) received the highest number of median payments per surgeon (17 payments; IQR, 6.5-28 payments; P < .001). Surgeons 21 to 44 years posttraining (fourth quartile) received the lowest median individual payment ($51; IQR, $20-132; P < .001)., Conclusions: Industry payments vary by hospital reputation and provider experience. Such biases must be understood for self-governance and the delineation of conflict of interest policies that balance industry relationships with clinical innovation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We performed a retrospective study of cardiology telemedicine visits at a large academic pediatric center between 2016 and 2019 (pre COVID-19). Telemedicine patient visits were matched to data from their previous in-person visits, to evaluate any significant differences in total charge, insurance compensation, patient payment, percent reimbursement and zero reimbursement. Miles were measured between patient's home and the address of previous visit. We found statistically significant differences in mean charges of telemedicine versus in-person visits (2019US$) (172.95 vs 218.27, p=0.0046), patient payment for telemedicine visits versus in-person visits (2019US$) (11.13 vs 62.83, p≤0.001), insurance reimbursement (2019US$) (65.18 vs 110.85, p≤0.001) and insurance reimbursement rate (43% vs 61%, p=0.0029). Rate of zero reimbursement was not different. Mean distance from cardiology clinic was 35 miles. No adverse outcomes were detected. This small retrospective study showed cost reduction and a decrease in travel time for families participating in telemedicine visits. Future work is needed to enhance compensation for telemedicine visits., Competing Interests: Competing interests: None declared., (© American Federation for Medical Research 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To compare the safety and resource-efficacy of the fast-track (FT) concept (extubation ≤8 hours after surgery) versus the conventional approach (non-FT, >8 hours postoperatively) in infants undergoing open-heart surgery., Methods: Infants <7 kg operated on cardiopulmonary bypass between 2014 and 2018 were analyzed. Propensity score matching (1:1) was performed for group comparison (FT vs non-FT). Intensive care unit (ICU) personnel use and unit performance were evaluated. Postoperative outcome and reimbursement based on German diagnosis-related groups were compared., Results: Of 717 infants (median age: 4 months, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality score: 0.1-4), FT extubation was achieved in 182 infants (25%). After matching, 123 pairs (FT vs non-FT) were formed without significant differences in baseline characteristics. FT versus non-FT showed a significantly shorter ICU stay (in days): 1.8 (0.9-2.8) versus 4.2 (1.9-6.4), P < .01, and postoperative length of stay (in days): 7 (6-10) versus 10 (7-15.5), P < .01; significantly lower postoperative transfusion rates: 61.3% versus 77%, P < .01; and tendency toward lower early mortality: 0% versus 2.8%, P = .08. Reintubation rate did not differ between the groups (P = .7). Despite a decrease in personnel capacity (2014 vs 2018), the unit performance was maintained. The mean case-mix-index of FT versus non-FT was 8.56 ± 6.08 versus 11.77 ± 12.10 (P < .01), resulting in 27% less reimbursement in the FT group., Conclusions: FT concept can be performed safely and resource-effectively in infants undergoing open-heart surgery. Since German diagnosis-related group systems reimburse costs, not performance, there is little incentive to avoid prolonged mechanical ventilation. Greater ICU turnover rates and excellent postoperative outcomes are not rewarded adequately., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Prolonged cardiopulmonary bypass time (prolonged CPBT; PCPBT) during operations for adult congenital heart disease (ACHD) may lead to worse postoperative outcomes, which could add a significant burden to hospitals in developing countries. This study aimed to identify risk factors and outcomes of PCPBT in patients undergoing operations for ACHD., Methods: This retrospective study included all adult patients (≥18 years) who underwent cardiac surgery with cardiopulmonary bypass for their congenital heart defect from 2011-2016 at a tertiary-care private hospital in Pakistan. Prolonged CPBT was defined as CPBT>120 minutes (65 th percentile)., Results: This study included 166 patients (53.6% males) with a mean age of 32.05±12.11 years. Comorbid disease was present in 59.0% of patients. Most patients underwent atrial septal defect repair (42.2%). A total of 58 (34.9%) of patients had a PCPBT. Postoperative complications occurred in 38.6% of patients. Multivariable analysis adjusted for age, gender and RACHS-1 Categories showed that mild preoperative left ventricular (LV) dysfunction was associated with PCPBT (OR=3.137 [95% CI: 1.003-9.818]), while obesity was found to be protective (0.346 [0.130-0.923]). PCPBT was also associated with a longer duration of ventilation (1.298 [1.005-1.676]), longer cardiac ICU stay (1.204 [1.061-1.367]) and longer hospital stay (1.120 [1.005-1.247])., Conclusions: While mild preoperative LV dysfunction was associated with PCPBT, obesity was found to be protective. Postoperatively, PCPBT was associated with longer duration of ventilation, cardiac ICU stay, and hospital stay. Operations with shorter CPBT may help minimize the occurrence and impact of these postoperative adverse outcomes especially in resource-constrained developing countries.

Objective: To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery., Study Design: 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., Results: 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., Conclusions: 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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.

Objectives: To determine if a policy of universal fetal echocardiography (echo) in pregnancies conceived by in-vitro fertilization (IVF) is cost-effective as a screening strategy for congenital heart defects (CHDs) and to examine the cost-effectiveness of various other CHD screening strategies in IVF pregnancies., Methods: A decision-analysis model was designed from a societal perspective with respect to the obstetric patient, to compare the cost-effectiveness of three screening strategies: (1) anatomic ultrasound (US): selective fetal echo following abnormal cardiac findings on detailed anatomic survey; (2) intracytoplasmic sperm injection (ICSI) only: fetal echo for all pregnancies following IVF with ICSI; (3) all IVF: fetal echo for all IVF pregnancies. The model initiated at conception and had a time horizon of 1 year post-delivery. The sensitivities and specificities for each strategy, the probabilities of major and minor CHDs and all other clinical estimates were derived from the literature. Costs, including imaging, consults, surgeries and caregiver productivity losses, were derived from the literature and Medicare databases, and are expressed in USA dollars ($). Effectiveness was quantified as quality-adjusted life years (QALYs), based on how the strategies would affect the quality of life of the obstetric patient. Secondary effectiveness was quantified as number of cases of CHD and, specifically, cases of major CHD, detected., Results: The average base-case cost of each strategy was as follows: anatomic US, $8119; ICSI only, $8408; and all IVF, $8560. The effectiveness of each strategy was as follows: anatomic US, 1.74487 QALYs; ICSI only, 1.74497 QALYs; and all IVF, 1.74499 QALYs. The ICSI-only strategy had an incremental cost-effectiveness ratio (ICER) of $2 840 494 per additional QALY gained when compared to the anatomic-US strategy, and the all-IVF strategy had an ICER of $5 692 457 per additional QALY when compared with the ICSI-only strategy. Both ICERs exceeded considerably the standard willingness-to-pay threshold of $50 000-$100 000 per QALY. In a secondary analysis, the ICSI-only strategy had an ICER of $527 562 per additional case of major CHD detected when compared to the anatomic-US strategy. All IVF had an ICER of $790 510 per case of major CHD detected when compared with ICSI only. It was determined that it would cost society five times more to detect one additional major CHD through intensive screening of all IVF pregnancies than it would cost to pay for the neonate's first year of care., Conclusion: The most cost-effective method of screening for CHDs in pregnancies following IVF, either with or without ICSI, is to perform a fetal echo only when abnormal cardiac findings are noted on the detailed anatomy scan. Performing routine fetal echo for all IVF pregnancies is not cost-effective. © 2020 International Society of Ultrasound in Obstetrics and Gynecology., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

Importance: Congenital heart disease (CHD) carries significant health care costs and out-of-pocket expenses for families. Little is known about how financial hardship because of medical bills affects families' access to essential needs or medical care., Objective: To assess the national prevalence of financial hardship because of medical bills among families of children with CHD in the US and the association of financial hardship with adverse outcomes., Design, Setting, and Participants: This cross-sectional survey study used data on children 17 years and younger with self-reported CHD from the National Health Interview Survey of US households between 2011 and 2017. Data were analyzed from March 2019 to April 2020., Exposures: Financial hardship because of medical bills was classified into 3 categories: no financial hardship, financial hardship but able to pay medical bills, and unable to pay medical bills., Main Outcomes and Measures: Food insecurity, delayed care because of cost, and cost-related medication nonadherence., Results: Of 188 families of children with CHD (weighted sample of 151 537 families), 48.9% reported some financial hardship because of medical bills, with 17.0% being unable to pay their medical bills at all. Compared with those who denied financial hardships because of medical bills, families who were unable to pay their medical bills reported significantly higher rates of food insecurity (61.8% [SE, 11.0] vs 13.6% [SE, 4.0]; P < .001) and delays in care because of cost (26.2% [SE, 10.4] vs 4.8% [SE, 2.5]; P = .002). Reported medication adherence did not differ across financial hardship groups. After adjusting for age, race/ethnicity, and maternal education, the differences between the groups persisted. The association of financial hardship with adverse outcomes was stronger among patients with private insurance than those with Medicaid., Conclusions and Relevance: In this study, financial hardship because of medical bills was common among families of children with CHD and was associated with high rates of food insecurity and delays in care because of cost, suggesting possible avenues for intervention.

Background: There is a paucity of contemporary information regarding the prevalence and related health care expenditure of the most common cardiac conditions in Australian children, including congenital heart disease (CHD)., Methods: The 10 most common cardiac conditions managed by Queensland Paediatric Cardiac Service during 2014-2015 were identified from an electronic database: ventricular septal defect (VSD), pulmonary stenosis, aortic stenosis, tetralogy of Fallot, atrioventricular septal defect, transposition, Ebstein's anomaly, long QT syndrome, dilated cardiomyopathy, and rheumatic carditis. Demographic data, clinic attendance, investigational procedures, and therapeutic interventions were extracted from the electronic health records to derive indicative population estimates and direct health care expenditure relating to CHD., Results: A total of 2,519 patients diagnosed with the 10 target conditions were being actively managed, including 456 (18.1%) new-born and 787 prevalent cases (2.5/1,000 population) aged <5 years. A total of 12,180 (4.8/case) investigations were performed (6,169 echocardiographic and 279 cardiac catheterisation procedures) costing $2.25 million/annum. A further 5,326 clinic visits (2.1/case, 22% regional) were conducted at a cost of $550,000/annum. A combined total of 804 catheter-based interventions and surgical procedures were performed in 300 cases (11.9%) at a cost of $13.6 million/annum. VSD (38.6% of cases) was the single greatest contributor ($5.1 million/annum) to total combined direct health care costs of $13.6 million/annum for the 2,519 patients., Conclusions: These pilot data indicate a significant patient population and health care burden imposed by CHD in Queensland. Future initiatives to better quantify this burden, from an individual to health system perspective, are urgently needed., (Copyright © 2020 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

Little is known about the relation between socioeconomic factors and health outcomes in adults and adolescents with congenital heart defects (CHD). Population-level data from the Colorado CHD surveillance system from 2011 to 2013 was used to examine the association between area deprivation and outcomes including hospitalizations, emergency department visits, cardiac procedures, all-cause and cardiac-related mortality, and major adverse cardiac events. Socioeconomic context was measured by the Area Deprivation Index at census tract level. Missing race/ethnicity was imputed using the Bayesian Improved Surname Geocoding algorithm. Generalized linear models were utilized to examine health disparities across deprivation quintiles after adjusting for insurance type, race/ethnicity, age, gender, urbanicity, and CHD severity in 5,748 patients. Cases residing in the most deprived quintile had 51% higher odds of inpatient admission, 74% higher odds of emergency department visit, 41% higher odds of cardiac surgeries, and 45% higher odds of major adverse cardiac events compared with cases in the least deprived quintile. Further, rates of hospitalizations, emergency department admissions, and cardiac surgeries were elevated in the most deprived compared with the least deprived quintile. Mortality was not significantly different across quintiles. In conclusion, findings suggest significant health equity issues for adolescent and adults with CHD based on area-based deprivation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

One in a hundred babies, or 1.3 million per year around the world, are born with congenital heart defects (CHD), of which over 70% will require medical or surgical treatment within their first year of life. In low- and middle-income countries, the majority does not receive the treatment they need or receive suboptimal care. Despite a higher pediatric cardiac surgical disease burden, low-income countries only have 0.07 pediatric cardiac surgeons per million pediatric population, compared to 9.51 per million in high-income countries. Here, we perform a stakeholder analysis of existing advocacy bodies within the field of pediatric global cardiac surgery and identify gaps and opportunities in advocating for cardiac care for children with CHD. We propose a framework to optimize civil society messaging in order to more effectively advocate domestically and internationally to hold national and international policy makers accountable for existing gaps and disparities in CHD care around the world.

Objectives: Infants undergoing congenital heart surgery require central venous lines which can be achieved by various combinations of transthoracic lines, percutaneous-indwelling central catheters and tunneled Broviac catheters. Transthoracic lines are removed by protocol prior to cardiac intensive care unit discharge (risk of bleeding), at which time percutaneous-indwelling central catheters are placed. Transdiaphragmatic tunneled Broviac catheters placed at the time of sternotomy, remain in place until hospital discharge, when they are safely removed at bedside. We characterized actual cost profiles associated with strategies that do versus do not include tunneled Broviac catheters., Methods: From January 2014 to December 2016, we identified a study population of 220 consecutive patients under 1 year of age undergoing congenital heart surgery. Cost data were acquired from our electronic patient system interface database and office of finance. Our cohort was divided into 2 groups, tunneled Broviac catheter and nontunneled Broviac catheter. We calculated the total cost associated with each groups' central venous lines, propensity matched, and used the Mann-Whitney U test to analyze the results., Results: Eighty-three (37.7%) of the 220 patients had tunneled Broviac catheters. The tunneled Broviac catheter group had 4 percutaneous-indwelling central catheter insertions and 6 radiological interventions while the nontunneled Broviac catheter group had 90 percutaneous-indwelling central catheters and 203 radiologic interventions. After propensity score matching, both groups were reduced to 82 patients and sum, median and interquartile range cost for tunneled Broviac catheters and nontunneled Broviac catheters was $17,351.84, $159.76 (128-159.76) versus $72,809.32, $1277.26 (31.76-1277.26), P < .02 respectively., Conclusions: Tunneled Broviac catheters, placed routinely at cardiac surgery, incur lower costs than the conventional combination of transthoracic lines and percutaneous-indwelling central catheters. The cost-effectiveness is achieved by reducing the number of percutaneous-indwelling central catheters and associated radiologic interventions., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Early mortality rates for paediatric cardiac surgery have fallen due to advancements in care. Alternative indicators of care quality are needed. Postoperative morbidities are of particular interest. However, while health impacts have been reported, associated costs are unknown. Our objective was to calculate the costs of postoperative morbidities following paediatric cardiac surgery., Design: Two methods of data collection were integrated into the main study: (1) case-matched cohort study of children with and without predetermined morbidities; (2) incidence rates of morbidity, measured prospectively., Setting: Five specialist paediatric cardiac surgery centres, accounting for half of UK patients., Patients: Cohort study included 666 children (340 with morbidities). Incidence rates were measured in 3090 consecutive procedures., Methods: Risk-adjusted regression modelling to determine marginal effects of morbidities on per-patient costs. Calculation of costs for hospital providers according to incidence rates. Extrapolation using mandatory audit data to report annual financial burden for the health service., Outcome Measures: Impact of postoperative morbidities on per-patient costs, hospital costs and UK health service costs., Results: Seven of the 10 morbidity categories resulted in significant costs, with mean (95% CI) additional costs ranging from £7483 (£3-£17 289) to £66 784 (£40 609-£103 539) per patient. On average all morbidities combined increased hospital costs by 22.3%. Total burden to the UK health service exceeded £21 million each year., Conclusion: Postoperative morbidities are associated with a significant financial burden. Our findings could aid clinical teams and hospital providers to account for costs and contextualise quality improvement initiatives., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Children and adolescents with congenital heart disease (CHD) are at an increased risk of neuropsychiatric disorders (NPDs). The purpose of this study is to determine how a comorbid NPD affects hospital outcomes and costs for CHD patients undergoing cardiac surgery., Methods: Retrospective review of the 2000-2012 Healthcare Cost and Utilization Project Kids' Inpatient Databases for admissions 10 to 21 years old with an ICD-9 code for moderate or severe CHD and a procedure code for cardiopulmonary bypass as a marker for cardiac surgery; admissions with syndromes that could be associated with NPD were excluded. Demographics, hospital outcomes, and charges were compared between admissions with and without NPD ICD-9 codes using analysis of variance, independent samples Kruskal-Wallis, and χ 2 , as appropriate., Results: There were 4768 admissions with CHD and cardiac surgery: 4285 (90%) with no NPD, 93 (2%) with cognitive deficits, 390 (8%) with mood/behavior deficits. Patients with NPD had a longer length of stay and higher mean charges (P < .001 for both). Patients with mood/behavior deficits were older and patients with cognitive deficits were more likely female (P < .001 for both)., Conclusions: Children and adolescents with moderate or severe CHD and NPD who undergo cardiac surgery incur longer hospital stays and higher charges. Recognizing and addressing the underlying NPDs may be important to improve postoperative progression for children and adolescents with CHD hospitalized for cardiac surgery., (© 2020 Wiley Periodicals LLC.)

Objective: To perform a cost-effectiveness analysis of different follow-up strategies for non-obese and obese women who had incomplete fetal cardiac screening for major congenital heart disease (CHD)., Methods: Three decision-analytic models, one each for non-obese, obese and Class-III-obese women, were developed to compare five follow-up strategies for initial suboptimal fetal cardiac screening. The five strategies were: (1) no follow-up ultrasound (US) examination but direct referral to fetal echocardiography (FE); (2) one follow-up US, then FE if fetal cardiac views were still suboptimal; (3) up to two follow-up US, then FE if fetal cardiac views were still suboptimal; (4) one follow-up US and no FE; and (5) up to two follow-up US and no FE. The models were designed to identify fetuses with major CHD in a theoretical cohort of 4 000 000 births in the USA. Outcomes related to neonatal mortality and neurodevelopmental disability were evaluated. A cost-effectiveness willingness-to-pay threshold was set at US$100 000 per quality-adjusted life year (QALY). Base-case and sensitivity analysis and Monte-Carlo simulation were performed., Results: In our base-case models for all body mass index (BMI) groups, no follow-up US, but direct referral to FE led to the best outcomes, detecting 7%, 25% and 82% more fetuses with CHD in non-obese, obese and Class-III-obese women, respectively, compared with the baseline strategy of one follow-up US and no FE. However, no follow-up US, but direct referral to FE was above the US$100 000/QALY threshold and therefore not cost-effective. The cost-effective strategy for all BMI groups was one follow-up US and no FE. Both up to two follow-up US with no FE and up to two follow-up US with FE were dominated (being more costly and less effective), while one follow-up US with FE was over the cost-effectiveness threshold. One follow-up US and no FE was the optimal strategy in 97%, 93% and 86% of trials in Monte-Carlo simulation for non-obese, obese and Class-III-obese models, respectively., Conclusion: For both non-obese and obese women with incomplete fetal cardiac screening, the optimal CHD follow-up screening strategy is no further US and immediate referral to FE; however, this strategy is not cost-effective. Considering costs, one follow-up US and no FE is the preferred strategy. For both obese and non-obese women, Monte-Carlo simulations showed clearly that one follow-up US and no FE was the optimal strategy. Both non-obese and obese women with initial incomplete cardiac screening examination should therefore be offered one follow-up US. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.)

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.

Background: Optimal methods to assess resource utilization in congenital heart surgery remain unclear. We compared traditional cost-to-charge ratio methods with newer standardized cost methods that aim to more directly assess resources consumed., Methods: Clinical data from The Society of Thoracic Surgeons Database were linked with resource use data from the Pediatric Health Information Systems Database (2010 to 2015). Standardized cost methods specific to the congenital heart surgery population were developed and compared with cost-to-charge ratio methods. Resource use in the overall population and variability across hospitals were described using hierarchical mixed effect models adjusting for case-mix., Results: Overall, 43 hospitals (65,331 patients) were included. There were minimal population-level differences in the distribution of resource use as estimated by the two methods. At the hospital level, there was less apparent variability in resource use across centers with the standardized cost vs cost-to-charge ratio method, overall (coefficient of variation 20% vs 25%) and across complexity (The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT]) categories. When hospitals were categorized into tertiles by resource use, 33% changed classification depending on which resource use method was used (26% by one tertile and 7% by two tertiles)., Conclusions: In this first evaluation of standardized cost methodology in the congenital heart population, we found minimal differences vs traditional methods at the population level. At the hospital level, the magnitude of variation in resource use was less with standardized cost methods, and approximately one third of centers changed resource use categories depending on the methodology used. Because of these differences, care should be taken in future studies and in benchmarking and reporting efforts in selecting optimal methodology., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The East Timor Hearts Fund (ETHF) is a charitable organisation of Australian cardiologists providing outreach screening in Timor-Leste. For patients requiring intervention, ETHF arranges logistics, procedures, and postoperative care. The aim of this project is to evaluate outcomes of patients requiring intervention., Methods: The ETHF database of all patients was utilised to identify patients with disease warranting surgical or percutaneous intervention. Both patients who underwent intervention and those who did not proceed to intervention were included in this study. Patients who had intervention arranged by other organisations but have then had follow-up with ETHF were also included. Overall demographics and pre and postoperative factors were assessed, with sub-group analysis of adult and paediatric patients to identify any differences in care., Results: Of 221 patients requiring intervention, 101 patients underwent intervention, receiving 22 different operations or procedures. Patients were predominantly young (median age 17.5 years) and female (64.7%), with rheumatic heart disease (63.8%). Twenty-four (24) (33.3%) women aged 15-45 years old with cardiac disease warranting intervention were documented as pregnant or breastfeeding at time of clinic assessment. Of patients who did not proceed to intervention, adults were more likely to be lost to follow-up (42.4% vs 18.5%) while paediatric patients were more likely to experience progression of disease (18.5% vs 7.5%, p=0.005). Median waitlist time was 5 months, with no significant difference between adults and children, correlating with a preoperative mortality rate of 5.4%. For patients who underwent intervention, post-procedure mortality was extremely low (0.9%) and attendance of at least one post-procedure review was excellent (99.0%). Eleven (11) (10.9%) patients have required repeat intervention, with no difference in rates between adult and paediatric patients. Length of follow-up extends up to 20 years for some patients., Conclusion: The Timor-Leste interventional cohort was predominantly a young female population with rheumatic and congenital cardiac disease. There were also high rates of pregnancy amongst female patients with severe cardiac disease. Delayed access to intervention may result in preoperative adverse events and mortality, and is a key target for improvement. Patients who undergo intervention have very low post-procedural mortality, good adherence to early medical follow-up and good long-term outcomes., (Copyright © 2019 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: As a result of advances in pediatric care and diagnostic testing, there is a growing population of adults with congenital heart disease (ACHD). The purpose of this study was to better define the epidemiology and changes in the trend of hospitalizations for ACHD in Korean society., Methods: We reviewed outpatient and inpatient data from 2005 to 2017 to identify patient ≥18 years of age admitted for acute care with a congenital heart disease (CHD) diagnosis in the pediatric cardiology division. We tried to analyze changes of hospitalization trend for ACHD., Results: The ratio of outpatients with ACHD increased 286.5%, from 11.1% (1748/15,682) in 2005 to 31.8% (7795/24,532) in 2017. The number of ACHD hospitalizations increased 360.7%, from 8.9% (37/414) in 2005 to 32.1% (226/705) in 2017. The average patient age increased from 24.3 years in 2005 to 27.4 in 2017. The main diagnosis for admission of ACHD is heart failure, arrhythmia and Fontan-related complications. The annual ICU admission percentage was around 5% and mean length of intensive care unit (ICU) stay was 8.4 ± 14.6 days. Mean personal hospital charges by admission of ACHD increased to around two times from 2005 to 2017. (from $2578.1 to $3697.0). Total annual hospital charges by ACHD markedly increased ten times (from $95,389.7 to $831,834.2)., Conclusions: The number of hospital cares for ACHD dramatically increased more than five times from 2005 to 2017. We need preparations for efficient healthcare for adults with CHD such as a multi-dimensional approach, effective communication, and professional training.

The etiology of congenital heart disease (CHD) is multifactorial. The birth prevalence of CHD is shaped by a wide variety of maternal, fetal, and neonatal risk factors, along with the rates of prenatal diagnosis and terminations of pregnancy, all of which have geographic variability Epidemiology data availability from low-and-middle-income countries (LMIC) on CHD prevalence, morbidity, and mortality are far more limited than from high income countries. Data on specific genetic, environmental, and prenatal risk associated with CHD are almost nonexistent. In this article, we will focus on defining what data are available, genetic risk factors, birth and overall prevalence, morbidity, and the impact of limited access to interventions, both surgery and cardiac catheterizations. We will highlight CHD in sub-Saharan Africa to detail epidemiology studies in the poorest regions of the world. Existing literature as well as estimates from the Global Burden of Disease Study (http://ghdx.healthdata.org) form the basis for this review. The intersection of poverty, high fertility rates, and limited access to care results in a unique profile of CHD in LMIC. CHD is not a preventable disease (by most standards), so early detection and access are our key interventions to improve the dire outcomes for children in low-resources settings of the world., (© 2020 Wiley Periodicals, Inc.)

Pediatric cardiology and cardiovascular surgery have witnessed significant advancements over the last two decades. In spite of this progress, congenital heart disease (CHD) still remains as one of the major causes of death in infants and young children in the United States. Many patient-related and patient-independent factors influence the outcomes in patients with CHD, one of which is the geographical location. In the US-Mexico border, management and outcomes of patients with CHD are further complicated by additional problems stemming from complex interplay between two different health systems, and socioeconomic disparities. In this article, the authors evaluate the various interplaying factors and describe the difficulties facing the practicing pediatric cardiologists in a US-Mexico border city.

Aims: This study aimed to examine the long-term clinical and economic burden of adults with congenital heart disease (ACHD) in Hong Kong. Methods: It retrospectively analyzed 336 consecutive ACHD patients who attended the Adult Congenital Heart Clinic between January 1, 2009 and December 31, 2014. Direct medical costs and clinical outcomes over the 5 years were calculated and documented. The economic evaluation was from the hospital's perspective. Results: The median age of ACHD patients was 47 (31-62) years old, with female predominance (61.5%). Ventricular and atrial septal defects accounted for 70% and severe ACHD for 10% of the study cohort. The prevalence of arrhythmia and heart failure increased with the complexity of CHD. The total mean annual cost for managing each ACHD patient was USD 2,913. The annual cost of management of simple ACHD was USD 2,638 vs complex ACHD (USD 6,425) ( p  = 0.013). Conclusions: This study demonstrated severe ACHD patients accounted for higher cardiovascular morbidities in arrhythmias and heart failure with a higher cost of management.

Background: The impact of socio-economic status (SES) on congenital heart disease (CHD)-related mortality in children is not well established., Objectives: We aimed to systematically review and appraise the existing evidence on the association between SES (including poverty, parental education, health insurance, and income) and mortality among children with CHD., Data Sources: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, citations, and key journals were searched., Study Selection and Data Extraction: We included articles reporting original research on the association between SES and mortality in children with CHD if they were full papers published in the English language and regardless of (a) timing of mortality; (b) individual or area-based measures of SES; (c) CHD subtype; (d) age at ascertainment; (e) study period examined. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate., Synthesis: Meta-analyses were performed to estimate pooled ORs for in-hospital mortality according to health insurance status., Results: Of 1388 identified articles, 28 met the inclusion criteria. Increased area-based poverty was associated with increased odds/risk of postoperative (n = 1), neonatal (n = 1), post-discharge (n = 1), infant (n = 1), and long-term mortality (n = 2). Higher parental education was associated with decreased odds/risk of neonatal (n = 1) and infant mortality (n = 5), but not with long-term mortality (n = 1). A meta-analysis of four US articles showed increased unadjusted odds of in-hospital mortality in those with government/public versus private health insurance (OR 1.40, 95% CI 1.24, 1.56). The association between area-based income and CHD-related mortality was conflicting, with three of eight articles reporting significant associations., Conclusion: This systematic review provides evidence that children of lower SES are at increased risk of CHD-related mortality. As these children are over-represented in the CHD population, interventions targeting socio-economic inequalities could have a large impact on improving CHD survival., (© 2019 John Wiley & Sons Ltd.)

Objectives: The purpose of this study was to evaluate the association between the method of procedural sedation and outcomes for congenital cardiac catheterization procedures., Background: The safety of operator-directed sedation (ODS) in the pediatric/congenital cardiac catheterization laboratory has been questioned. To our knowledge, the relative safety of ODS versus general anesthesia (GA) in these cases has not to date been critically evaluated., Methods: A single-center retrospective cohort study was performed to compare the relative safety, cost, and times of catheterization procedures performed with ODS and those performed with GA from a cardiac anesthesiologist. The risk of adverse outcomes was compared using propensity-score-adjusted models. Using the same propensity score, procedure times and relative charges were also compared., Results: Over the study period, 4,424 procedures in 2,547 patients were studied. Of these, 27% of cases were performed with ODS. ODS procedures were 70% diagnostic procedures, 17% device closure of patent ductus arteriosus, 5% balloon pulmonary valvuloplasty, and 3% pulmonary artery angioplasty. The risk of adverse event in adjusted models for ODS cases was significantly lower than in GA cases (odds ratio: 0.66; 95% confidence interval: 0.45 to 0.95; p = 0.03). Total room time and case time were also significantly shorter (p < 0.001). Professional (charge ratio: 0.88; p < 0.001) and hospital (charge ratio: 0.84; p < 0.001) charges for ODS cases were also lower than those for GA cases., Conclusions: This study demonstrates that clinical judgment can identify subjects in whom ODS is not associated with increased risk of adverse events. The use of ODS was associated with reduced case times and charges. In combination, these findings suggest that the selective use of ODS can allow for greater efficiency and higher value care without sacrificing safety., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background Cardiac catheterization is an important but costly component of health care for young patients with cardiac disease. Measurement of variation in their cost between hospitals and identification of the reasons for this variation may help reduce cost without compromising quality. Methods and Results Using data from Pediatric Health Information Systems Database from January 2007 to December 2015, the costs of 9 procedures were measured. Mixed-effects multivariable models were used to generate case-mix-adjusted estimates of each hospital's cost for each procedure and measure interhospital variation. Procedures (n=35 637) from 43 hospitals were studied. Median costs varied from $8249 (diagnostic catheterization after orthotopic heart transplantation) to $38 909 (transcatheter pulmonary valve replacement). There was marked variation in the cost of procedures between hospitals with 3.5- to 8.9-fold differences in the case-mix-adjusted cost between the most and least expensive hospitals. No significant correlation was found between hospitals' procedure-specific mortality rates and costs. Higher procedure volume was not associated with lower cost except for diagnostic procedures in heart transplant patients and pulmonary artery angioplasty. At the hospital level, the proportion of cases that were outliers (>95th percentile) was significantly associated with rank in terms of cost (Spearman's ρ ranging from 0.37 to 0.89, P<0.01). Conclusions Large-magnitude hospital variation in cost was not explained by case-mix or volume. Further research is necessary to determine the degree to which variation in cost is the result of differences in the efficiency of the delivery of healthcare services and the rate of catastrophic adverse outcomes and resultant protracted and expensive hospitalizations.

Background: Cardiovascular disease is the number one global killer, with over three quarters of these deaths arising from the populations of low- and middle-income countries (LMICs). Addressing the burden of cardiovascular disease in LMICs must include medical and surgical services for these patients. In this article, we model the needs and costs to scale up the cardiac provider workforce in Kenya, which can be adapted to other LMICs based on country-specific workforce hours and workforce salaries., Methods: Using published epidemiological reports from sub-Saharan Africa, we structured the model based on the expected disease burden of congenital and rheumatic disease in a simulated 1,000-person population. Services modeled include clinic visits, echocardiograms, diagnostic cardiac catheterizations, interventional catheterizations, and heart surgery. Costs were modeled based on Kenyan public sector salaries. After scaling the model, we created a sensitivity analysis of change in service duration and salaries., Results: Based on a 1,000-person Kenyan population, we estimate that 2.5 heart surgeries will be needed every year, with a corresponding annual workforce cost of US$526. Including accompanying services of clinic visits, echocardiograms, and both diagnostic and interventional cardiac catheterizations, the total annual workforce cost is US$899. Based on estimated productive hours for public sector workforce, 196 full-time equivalent cardiac surgeons will be needed for the entire population of Kenya (2017 figure)., Conclusions: We present a model for appropriate cardiovascular service staffing based on disease burden and workforce costs. This model can be scaled up as needed to plan for local capacity building.

We sought to analyze the trends and resource utilization of adult congenital heart disease (ACHD)-related heart failure admissions at children's hospitals. Heart failure admissions in patients with ACHD continue to rise at both pediatric and adult care facilities. Data from the Pediatric Health Information Systems database (2005 to 2015) were used to identify patients (≥18 years) admitted with congenital heart disease (745.xx-747.xx) and principal diagnosis of heart failure (428.xx). High resource use (HRU) admissions were defined as those over the 90th percentile. There were 562 admissions (55.9% male) across 39 pediatric hospitals. ACHD-related heart failure admissions increased from 4.1% in 2006 to 6.3% in 2015 (p = 0.015). Median hospital charge for ACHD-related heart failure admissions was $59,055 [IQR $26,633 to $156,846]. Total charges increased with more complex anatomic category (p = 0.049). Though HRU admissions represented 10% of ACHD-related heart failure admissions, they accounted for >66% of the total charges. The median total hospital charges for HRU admissions were $1,018,656 [IQR $722,574 to $1,784,743], compared with $58,890 [IQR $26,456 to $145,890] for non-HRU admissions (p < 0.001). Inpatient mortality rate (26.3% vs 4.0%) and the presence of ≥2 comorbidities (68% vs 31%) were higher for HRU admissions (p < 0.001). On multivariable analysis, technology dependence (aOR: 4.4, p < 0.001) and renal comorbidities (aOR: 3.0, p = 0.04) were associated with HRU. In conclusion, heart failure-related ACHD admissions in pediatric hospitals are increasing. Compared with non-HRU, HRU admissions had higher inhospital mortality and greater comorbidities. Additional care strategies to reduce resource use among these patients and improve overall quality of care merits further study., (Copyright © 2018. Published by Elsevier Inc.)

Background Socioeconomic status ( SES ) is associated with health-related quality of life ( HRQOL ) for children with critical congenital heart disease; however, literature from newly industrialized countries is scarce. Methods and Results This cross-sectional study included 2037 surviving patients operated on for critical congenital heart disease at a tertiary hospital in China between May 2012 and December 2015. All eligible patients were aged 2 to 12 years. HRQOL was measured by the Pediatric Quality of Life Inventory 4.0 generic and 3.0 cardiac modules. Family SES was assessed by a composite of household income in the past year and occupation and education level of each parent in the family. Mean scores of major domains in HRQOL were significantly lower in the low- SES group than in the medium- and high- SES groups (total generic scores: 71.2±7.9 versus 75.0±8.0 and 76.0±7.9, respectively [ P<0.001]; psychosocial functioning: 70.8±9.0 versus 74.4±8.4 and 75.3±8.4 [ P<0.001]; physical functioning: 71.6±10.4 versus 76.0±9.7 and 77.1±9.4 [ P<0.001]; heart symptoms: 71.9±11.6 versus 75.7±11.0 and 76.8±10.3 [ P<0.001]; cognitive problems: 65.4±11.1 versus 69.4±12.1 and 74.6±13.6 [ P<0.001]). After adjustment for other clinical and demographic variables in the multivariable linear regression model, family SES significantly affected all dimensions of HRQOL except for treatment barriers, treatment anxiety, physical appearance and communication. Conclusions Family SES is an important factor associated with HRQOL in patients with critical congenital heart disease. Further targeted interventions to improve HRQOL that consider the family and environmental issues confronted by those who are economically disadvantaged might help these patients have better outcomes.

Objectives: The recent trend to optimize the efficiency of health-care systems requires objective clinical and economic data. European data on the cost of surgical procedures to repair or palliate congenital heart disease in pediatric patients are lacking., Methods: A single-center study was conducted. Bootstrap analysis of variance and bootstrap independent t test assessed the excess direct medical costs associated with minor and major complications in nine surgical procedure types, from a health-care payer perspective. Generalized linear models with log-link function and inverse Gaussian family were used to determine associated covariates with the total hospitalization cost. Descriptive statistics show the repartition between out-of-pocket expenditures and reimbursed costs., Results: Four hundred thirty-seven patients were included. Mean hospitalization costs ranged from €11,106 (atrial septal defect repair) to €33,865 (Norwood operation). Operations with major complications yielded excess costs compared to operations with no complications, ranging from €7,105 (+65.2%) for a truncus arteriosus repair to €27,438 (+251.7%) for a tetralogy of Fallot repair. Differences in costs were limited between operations with minor versus no complications. Age at procedure, intensive care unit stay, procedure risk category, reintervention, and postoperative mechanical circulatory support were associated with higher total hospitalization costs. Out-of-pocket expenditures represented 6% of total hospitalization costs., Conclusion: Operations with major complications yield excess costs, compared to operations with minor or no complications. Cost data and attribution are important to improve clinical practice in a cost-effective manner. The health-care system benefits from strategies and technological advancements that have an impact on modifiable cost-affecting parameters.

Background: Scarce data on crowdfunding report a maximal funding of €10.000,-, and state that research is needed to attract attention of larger granting organizations. The aims of this project were 1) to fund an eHealth study in grown-up congenital heart disease (GUCH) patients 2) to contemplate on critical success factors., Methods: After peer review of the Dutch Heart Foundation a project was published at a donation platform, which was open for donations during a predetermined period of two months. Copywriters were hired to create an easy-to-understand message to donors. A video teaser was created with a motivated patient, and rewards were available. The crowdfunding targeted €25.000 and the Dutch Heart Foundation doubled the donations to €50.000, and return of donations were guaranteed in case this was not met., Results: Initially, donations came from the investigators' private inner circle. In total, 44 potential donors were contacted, but refused to donate originally. Multiple (social) media campaigns were published to promote the project, and an offline mailing was sent to contributors to the Dutch Heart Foundation. During the project support emerged, resulting in extra donations and public awareness. In the lasts three weeks, after sufficient private donations, five major donors decided to support the project. The project became a big success: the predetermined target was exceeded and a total of €74.450,- was raised., Conclusion: Innovative crowdfunding gave the opportunity to start eHealth research in GUCH patients. Critical success factors include support of a professional organization, support of stakeholders, and easy-to-understand messages., (Copyright © 2018 Elsevier B.V. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Importance: Endorsement of global humanitarian interventions is based on either proven cost-effectiveness or perceived public health benefits. The cost-effectiveness and long-term benefits of global humanitarian pediatric cardiac surgery are unknown, and funding for this intervention is insufficient., Objectives: To determine the cost-effectiveness of the intervention (multiple 2-week-long humanitarian pediatric cardiac surgery program assistance trips to various low- and middle-income countries [LMICs]) and to produce a measure of the long-lasting effects of global humanitarian programs., Design, Setting, and Participants: International, multicenter cost-effectiveness analysis of a cohort of children (aged <16 years) undergoing surgical treatment of congenital heart disease during 2015 in LMICs, including China, Macedonia, Honduras, Iran, Iraq, Libya, Nigeria, Pakistan, Russia, and Ukraine. The study also assessed estimated improvement in the United Nations Human Development Indicators (life expectancy, years of schooling, and gross national income) for each individual survivor, as a proxy for long-term benefits of the intervention., Main Outcomes and Measures: The primary outcome was cost-effectiveness of the intervention. The secondary outcomes were potential gains in life expectancy, years of schooling, and gross national income per capita for each survivor., Results: During 2015, 446 patients (192 [43%] female; mean [SD] age, 3.7 [5.4] years) were served in 10 LMICs at an overall cost of $3 210 873. Of them, 424 were children. The cost-effectiveness of the intervention was $171 per disability-adjusted life-year averted. Each survivor in the cohort (390 of 424) potentially gained 39.9 disability-adjusted life-years averted, 3.5 years of schooling, and $159 533 in gross national income per capita during his or her extended lifetime at purchasing power parity and 3% discounting., Conclusions and Relevance: Humanitarian pediatric cardiac surgery in LMICs is highly cost-effective. It also leaves behind a lasting humanitarian footprint by potentially improving individual development indices.

Objectives: There is limited data on congenital heart disease (CHD) from the lower- and middle-income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time., Design: A population-based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart disease for children., Setting: State of Johor, Malaysia., Patients: All children (0-12 years of age) born in the state of Johor between January 2006 and December 2015., Intervention: None., Outcome Measure: The birth prevalence, severity, and temporal trend over time., Results: There were 531,904 live births during the study period with 3557 new cases of CHD detected. Therefore, the birth prevalence of CHD was 6.7 per 1000 live births (LB) (95% confidence interval [CI]: 6.5-6.9). Of these, 38% were severe, 15% moderate, and 47% mild lesions. Hence, the birth prevalence of mild, moderate, and severe CHD was 3.2 (95% CI: 3.0-3.3), 0.9 (95% CI: 0.9- 1.1), and 2.6 (95% CI: 2.4-2.7) per 1000 LB, respectively. There was a significant increase in the birth prevalence of CHD, from 5.1/1000 LB in 2006 to 7.8/1000 LB in 2015 (P < .0001) due to increase in detection of both mild (1.9/1000 LB in 2006 to 3.9/1000 LB in 2015, P < .001) and severe CHD (1.8/1000 LB in 2005 to 2.9/1000 LB in 2015, P < .001)., Conclusions: The birth prevalence of CHD was 6.7 per 1000 live births, and two in five were severe and significantly associated with syndrome and extracardiac defect. There was a significant increase in the detection of severe lesions in recent years leading to more burden to resources that are already limited in the middle-income country. Therefore, strategic and comprehensive pediatric and congenital heart surgery program is required., (© 2018 Wiley Periodicals, Inc.)

Objective: To examine the financial burdens and mental health needs of families of children with special healthcare needs (CSHCN) with congenital heart disease (CHD)., Methods: Data from the 2009-2010 National Survey of Children with Special Health Care Needs (NS-CSHCN) were used to examine parent-reported financial burdens (out-of-pocket expenses, financial problems, employment impact, caregiving hours) and family members' need for mental health services in families of CSHCN with CHD. Multivariable logistic regression was used to compare financial burdens and family members' need for mental health services among CSHCN with and without CHD. Among CSHCN with CHD, multivariable logistic regression, stratified by age (0-5 and 6-17 years), was used to assess characteristics associated with the outcomes., Results: Overall, families of 89.1% of CSHCN with CHD experienced at least one financial burden and 14.9% needed mental health services due to the child's condition. Compared with CSHCN without CHD, those with CHD had families with a higher prevalence of all financial burdens (adjusted prevalence ratio [aPR] range: 1.4-1.8) and similar family member need for mental health services (aPR = 1.3, 95% CI [1.0, 1.6]). Across both age groups, insurance type, activity limitations, and comorbidities were significantly associated with financial burdens and/or family members' need for mental health services., Conclusions: CSHCN with CHD, compared with those without CHD, lived in families with more financial burdens. Interventions that reduce financial burdens and improve mental health of family members are needed, especially among CSHCN with CHD who are uninsured and have comorbidities or activity limitations., (© 2018 Wiley Periodicals, Inc.)

Background: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD)., Methods and Results: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. RDW was measured in fresh venous blood samples at inclusion in 592 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at four annual follow-up visits. During 4.3 [IQR 3.8-4.7] years of follow-up, the primary endpoint (death, heart failure, hospitalization, arrhythmia, thromboembolic events, cardiac intervention) occurred in 196 patients (33%). Median RDW was 13.4 (12.8-14.1)% versus 12.9 (12.5-13.4)% in patients with and without the primary endpoint (P < 0.001). RDW was significantly associated with the endpoint when adjusted for age, sex, clinical risk factors, CRP, and NT-proBNP (HR 1.20; 95% CI 1.06-1.35; P = 0.003). The C-index of the model including RDW was slightly, but significantly (P = 0.005) higher than the model without (0.74, 95% CI 0.70-0.78 versus 0.73, 95% CI 0.69-0.78). Analysis of repeated RDW measurements (n = 2449) did not show an increase in RDW prior to the occurrence of the endpoint., Conclusions: RDW is associated with cardiovascular events in patients with ACHD, independently of age, sex, clinical risk factors, CRP, and NT-proBNP. This readily available biomarker could therefore be considered as an additive biomarker for risk stratification in these patients., (Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort., Methods: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other. Diagnosis and lesion severity of ACHD were extracted from ICD-10 diagnostic codes and assigned according to the 2008 American College of Cardiology/American Heart Association ACHD guidelines. Age-matching was used to account for baseline age differences between ACHD and non-ACHD patients., Results: E ACHD and 17 154 non-ACHD patients were identified. Without age-matching, ACHD patients were significantly younger than non-ACHD patients (mean age 38.5 vs 63.8 years). After age-matching (N = 805 in each group), mean age was 39.5 years in both groups. ACHD patients had less HMO (29.1% vs 34.7%, P = .012) and Medicaid (12.4% vs 17.3%, P = .006) coverage, but more PPO (34.4% vs 27.5%, P = .003) and Medicare (23.2% vs 18.1%, P = .005) coverage compared to non-ACHD patients. No differences were found in private insurance, public insurance, or self-pay. Lesion complexity had no effect on insurance in ACHD patients. Eligibility of parental plan coverage did not affect use of private insurance. ACHD patients in states with Medicaid expansion had higher rates of Medicaid (15.6% vs 10.6%, P = .045) but lower rates of HMO coverage (24.5% vs 31.7%, P = .036) and self-pay (0% vs 3.3%, P < .001). ACHD status, age, income, and residence in Medicaid expansion states were independent determinants of insurance types., Conclusions: In the Affordable Care Act era, ACHD patients are a well-insured population. Governmental policy has substantial effects on individual-level choice and access to insurance., (© 2018 Wiley Periodicals, Inc.)

Background: The Norwood operation is associated with high health care utilization, and prior studies reported substantial variability in Norwood costs across centers. However, specific factors driving this cost variation are unclear. We assessed center variability in Norwood costs and underlying mechanisms in a multicenter cohort., Methods: Clinical data from the Pediatric Heart Network Single Ventricle Reconstruction trial were linked with cost data from the Children's Hospital Association Inpatient Essentials database. Center variation was assessed by modeling Norwood costs adjusted for baseline patient characteristics, and the relationship with complications, length of stay (LOS), and specific cost categories was examined. Patients undergoing transplantation or stage 2 palliation during the Norwood admission were excluded., Results: Nine centers (332 patients) were included. Adjusted mean cost/case varied 4.6-fold across centers (range: $50,559 to $230,851, p < 0.001). In addition, variation was found across centers in the adjusted mean number of complications/case (2.6-fold variation) and adjusted mean LOS/case (1.9-fold variation). Differences in complications explained 63% of the cost variation across centers. After accounting for complications, differences in LOS explained 66% of the remaining cost variation. Seven specific complications were found to occur more frequently at high-cost centers: pleural effusion, seizures, wound infection, thrombus, liver dysfunction, sepsis, necrotizing enterocolitis (all p < 0.001). With regard to types of cost, room and board/supplies and laboratory costs were the primary drivers of cost variation across centers., Conclusions: This study identified several factors associated with center variation in Norwood costs, which may be targeted in subsequent initiatives aimed at both improving quality of care and reducing costs., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD.

Background: Children with single ventricle heart disease require frequent interventions and follow-up. Low socioeconomic status (SES) may limit access to high-quality care and place these children at risk for poor long-term outcomes., Methods and Results: Data from the SVR (Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use) data set were used to examine the relationship of US neighborhood SES with 30-day and 1-year mortality or cardiac transplantation and length of stay among neonates undergoing the Norwood procedure (n=525). Crude rates of death or transplantation at 1 year after Norwood were highest for patients living in neighborhoods with low SES (lowest tertile 37.0% versus middle tertile 31.0% versus highest tertile 23.6%, P =0.024). After adjustment for patient demographics, birth characteristics, and anatomy, patients in the highest SES tertile had significantly lower risk of death or transplant than patients in the lowest SES tertile (hazard ratio 0.62, 95% confidence interval, 0.40, 0.96). When SES was examined continuously, the hazard of 1-year death or transplant decreased steadily with increasing neighborhood SES. Hazard ratios for 30-day transplant-free survival and 1-year transplant-free survival were similar in magnitude. There were no significant differences in length of stay following the Norwood procedure by SES., Conclusions: Low neighborhood SES is associated with worse 1-year transplant-free survival after the Norwood procedure, suggesting that socioeconomic and environmental factors may be important determinants of outcome in critical congenital heart disease. Future studies should investigate aspects of SES and environment amenable to intervention., Clinical Trial Registration: URL:http://www.clinicaltrials.gov> http://www.clinicaltrials.gov. Unique identifier: NCT00115934., (© 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

Background: Pulse oximetry screening is a highly accurate tool for the early detection of critical congenital heart disease (CCHD) in newborn infants. As the technique is simple, noninvasive, and inexpensive, it has potentially significant benefits for developing countries. The aim of this study is to provide information for future clinical and health policy decisions by assessing the cost-effectiveness of CCHD screening in China., Methods and Findings: We developed a cohort model to evaluate the cost-effectiveness of screening all Chinese newborns annually using 3 possible screening options compared to no intervention: pulse oximetry alone, clinical assessment alone, and pulse oximetry as an adjunct to clinical assessment. We calculated the incremental cost per averted disability-adjusted life years (DALYs) in 2015 international dollars to measure cost-effectiveness. One-way sensitivity analysis and multivariate probabilistic sensitivity analysis were performed to test the robustness of the model. Of the three screening options, we found that clinical assessment is the most cost-effective strategy compared to no intervention with an incremental cost-effectiveness ratio (ICER) of Int$5,728/DALY, while pulse oximetry plus clinical assessment with the highest ICER yielded the best health outcomes. Sensitivity analysis showed that when the treatment rate increased up to 57.5%, pulse oximetry plus clinical assessment showed the best expected values among the three screening options., Conclusion: In China, for neonatal screening for CCHD at the national level, clinical assessment was a very cost-effective preliminary choice and pulse oximetry plus clinical assessment was worth considering for the long term. Improvement in accessibility to treatment is crucial to expand the potential health benefits of screening.

Advances in pediatric cardiac surgical and medical care have led to increased survival of patients with congenital heart disease (CHD). Consequently, many CHD patients survive long enough to require cardiac surgery as adults. Using the 2013 Nationwide Inpatient Sample (NIS) database, we compared costs and outcomes for adult patients undergoing surgery for treatment of CHD to a reference population of adults undergoing CABG. Patients were identified using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 CM) procedure codes. We recorded the demographic characteristics, gender, ethnicity, hospital bed size, hospital length of stay, in-hospital mortality, and comorbidities. Patients with ACHD have higher incidences of in-hospital mortality (2.6 vs. 1.8%), and complication rates including neurologic complications (2.6 vs. 0.9%), thromboembolic complications (3.9 vs. 1.4%), arrhythmias (51.6 vs. 29.8%), hepatic failure (4.44 vs. 2.03%), and sepsis (7.24 vs. 4.61%) (all p < 0.001). In addition, cost is higher in patients with CHD (Coefficient = 0.116, 95% CI, 0.105-0.128; p < 0.001), Elixhauser score ≥ 7 (Coefficient = 0.114, 95% CI, 0.108-0.121; p < 0.001), neurologic complications (Coefficient = 0.169, 95% CI, 0.143-0.196; p < 0.001), thrombotic complications (Coefficient = 0.243, 95% CI, 0.222-0.265; p < 0.001), sepsis (Coefficient = 0.198, 95% CI, 0.185-0.211; p < 0.001), acute kidney injury (Coefficient = 0.056, 95% CI, 0.041-0.063; p < 0.001), elective cases (Coefficient = 0.047, 95% CI, 0.041-0.053; p < 0.001), and length of stay > 6 days (Coefficient = 0.703, 95% CI, 0.697-0.710; p < 0.001). This study shows that ACHD patients undergoing cardiac surgery experience higher hospital costs and poorer outcomes than a reference population of adult CABG patients. Recognition and treatment of comorbidities in ACHD patients undergoing cardiac surgery may provide an opportunity to improve perioperative outcomes in this growing patient population.

Genetic testing is routinely performed on infants with critical congenital heart disease (CHD). This project reviewed the effect of implementing a genetic testing protocol in this population. Charts of infants with critical CHD were reviewed for genetic testing and results across two time periods: the time before implementation of a genetic testing protocol (pre-protocol) and the time after implementation (post-protocol). The use of karyotype, 22q11.2 Deletion testing, and chromosomal microarray were compared across these two time periods. Records of 891 infants were reviewed. 562 (63%) had at least one of the target genetic tests completed. During the pre-protocol time period, 66% of patients who had genetic testing underwent multiple tests versus 24% during the post-protocol time period (p < 0.01). The rate of patients who underwent genetic testing increased from 60% in the pre-protocol time period to 77% in the post-protocol time period (p < 0.01). The rate of diagnosis of genetic conditions during the pre-protocol period was 26% versus 36% during the post-protocol period (p = 0.01). There was a reduction in cost to patients by $5105.59 per diagnosis during the post-protocol period. Patients with critical CHD in the post-protocol period were less likely to undergo multiple genetic tests and more likely to have a diagnosis of genetic disease. In addition there was a significant reduction in cost per diagnosis during the post-protocol time period. Genetic testing protocols for infants with critical CHD promoted more efficient use of genetic testing and increased the rate of diagnosis of genetic conditions in this population.

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95% confidence interval [CI] $264,703 to $516,499), corresponding to 164 (95% CI 98 to 231) inpatient days. The cost incurred from birth through to Fontan completion (the staged procedures period) was $219,482 (95% CI $202,410 to $236,553) and the cost thereafter over 15 years was $146,820 (95% CI $44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (p <0.001). Having fenestration was associated with higher costs in the staged procedures period (p <0.001) and lower cost after Fontan over 15 years (p = 0.66). In conclusion, patients with single ventricle congenital heart disease continue to demand considerable inpatient resources after the staged procedures period. Over 40% of the pediatric hospital costs for Fontan patients were estimated to occur after the last planned surgery., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective The aim of the study was to assess the feasibility of using commonly available catheterization laboratory equipment for radiofrequency perforation of the pulmonary valve in patients with pulmonary atresia and intact ventricular septum. Methods The system (off-label use for all items) is made up of a co-axial telescopic arrangement consisting of a 0.014" PT 2 ™ coronary guidewire, for insulation inside a 2.7-F microcatheter which has an inner lumen of 0.021". The microcatheter was passed via a standard 4-F right coronary catheter to just below the atretic pulmonary valve. Radiofrequency (RF) energy was delivered using a standard electrosurgical system. In vitro testing had been performed and indicated that 5-10 W for 2-5 s would be sufficient for valve perforation. Results Radiofrequency perforation was successfully performed in all (n = 5, 100%) patients at a median age of 3 days (range: 1-36) and weight 2.7 kg (range 2.3-3.0). In one patient the pericardium was entered during the initial attempt; the generator was put on coagulation mode during retrieval of the guidewire and no haemopericardium occurred. The pulmonary valve was dilated in all; in three patients (n = 3) the ductus arteriosus was stented during the same session. Conclusion Results of the study show that it is feasible to perforate the pulmonary valve safely using this system. Availability, simplicity and cost are noteworthy benefits.

Congenital heart disease (CHD) is often associated with chronic extracardiac co-morbid conditions (ECC). The presence of ECC has been associated with greater resource utilization during the operative period; however, the impact beyond hospital discharge has not been described. This study sought to understand the scope of chronic ECC in infants with CHD as well as to describe the impact of ECC on resource utilization after discharge from the index cardiac procedure. IRB approved this retrospective study of infants <1 year who had cardiac surgery from 2006 and 2011. Demographics, diagnoses, procedures, STAT score, and ECC were extracted from the medical record. Administrative data provided frequency of clinic and emergency room visits, admissions, cumulative hospital days, and hospital charges for 2 years after discharge from the index procedure. Data were compared using Mann-Whitney Rank Sum Test with p < 0.05 considered significant. ECC occurred in 55% (481/876) of infants. Median STAT score was higher in the group with ECC (3 vs. 2, p < 0.001). Resource utilization after discharge from the index procedure as defined by median hospital charges (78 vs. 10 K, p < 0.001 and unplanned hospital days 4 vs. 0, p < 0.001) was higher in those with ECC, and increased with the greater number of ECC, even after accounting for surgical complexity. STAT score and the presence of multiple ECC were associated with higher resource utilization following the index cardiac surgical procedure. These data may be helpful in deciding which children might benefit from a cardiac complex care program that partners families and providers to improve health and decrease healthcare costs.