Severe Congenital Heart Disease Research Articles

Association between insurance type, clinical characteristics, and healthcare use in adults with congenital heart disease

IntroductionAdults with congenital heart disease (CHD) represent a heterogeneous and growing population with high healthcare utilization. We sought to understand the association between insurance type, healthcare use, and outcomes among adults with CHD in Oregon. MethodsThe Oregon All Payers All Claims database from 2010-2017 was queried for adults aged 18-65 in 2014 with ICD-9 or 10 codes consistent with CHD; patient demographics, comorbidities, healthcare use, and disease severity were identified. Insurance type was categorized as either public (Medicare and Medicaid) or private (commercial). Descriptive statistics were used to compare groups. Use rates and odds ratios were calculated representing probability of at least one event per person-year using logistic regression with clustering on patients. ResultsOf 13,792 adults with CHD, 48% had a form of public insurance. More publicly insured patients had moderate or severe anatomic complexity (29.5% vs. 23.0%; p < 0.0001), treatment for drug and alcohol use (25.0% vs. 7.2%; p < 0.0001), and mental health diagnoses (66.6% vs. 51.0%; p < 0.0001). They were more likely to reside in a rural area (24.5% vs. 16.1%; p < 0.0001). Adjusted for age and CHD severity, publicly insured patients were less likely to access overall ambulatory care (aOR 0.72, 99% CI 0.66 to 0.80) but more likely to access emergency (aOR 3.86, 99% CI 3.62 to 4.12) and inpatient (aOR 3.06, 99% CI 2.81 to 3.33) care, as shown in Figure 1. Length of hospital stay (5.7 vs. 4.4 days, p<0.0001) and rates of 30-day readmission (17.1% vs. 11.0%, p<0.001) were higher in those with public insurance. However, individuals with public insurance were significantly more likely to undergo their annual guideline-indicated echocardiogram (aOR 1.49, 99% CI 1.23 to 1.80) and attend their annual ACHD visits (aOR 1.62, 99% CI 1.40 to 1.87). ConclusionsOur study shows that publicly insured adults with CHD in Oregon have more anatomically complex disease, more comorbidities, and higher healthcare use. While they were more likely to receive guideline-indicated ACHD care, they were also higher utilizers of emergency room and inpatient resources, implying that they may benefit from targeted interventions to improve outcomes and decrease unplanned healthcare use.

Abstract 4123182: Women with Severe Congenital Heart Disease Have Increased Risk of Fetal/Neonatal Death

Background: Women with congenital heart defects (CHD) now routinely survive to reproductive age. Offspring of these women have more complications, including but not limited to inherited CHD, prematurity, small for gestational age (SGA), and fetal/neonatal death. In the United States, pregnancy-related complications and death are more common in Black women and their offspring compared to White women and their offspring, and in those residing in rural areas compared to urban areas. Aims: This study evaluates fetal/neonatal outcomes among women with CHD covered by Georgia Medicaid at the time of pregnancy, looking for differences among racial, geographic, and CHD severity groups. Methods: This retrospective cohort study identified 4581 women with CHD aged 11-50 with 6522 pregnancies (28.5% with severe CHD, 50.9% Black, 23.2% rural) covered by Georgia Medicaid between 2008-2019. Fetal/neonatal death, defined as fetal death after 20 weeks gestation, stillbirth, or neonatal death, was examined by CHD severity, race, and rurality using chi-square analysis. Multivariate logistic regression models were conducted adjusting for age at first documented pregnancy, social deprivation index (SDI), number of pregnancies, and multiple gestations. Results: Of 6522 pregnancies, 3.0% resulted in fetal/neonatal death. Fetal/neonatal death was more common in women with severe CHD compared to those with non-severe CHD (4.1% severe vs. 2.6% non-severe [aOR 1.39, 95% CI 1.02-1.85, p = 0.0018]). No significant differences were revealed in fetal/neonatal death between racial (3.3% Black vs. 2.8% White) or geographic (2.9% rural vs. 3.1% urban) groups after controlling for other variables. Conclusions: Compared to reported fetal and neonatal death rates among all U.S. pregnancies in 2019, women with CHD experienced a higher rate of fetal/neonatal death. Fetal/neonatal death was even more prevalent for women with severe CHD (4.1%) versus women with non-severe CHD (2.6%). Women with CHD, especially those with severe disease, have a high risk of fetal/neonatal death and should be monitored closely throughout the course of pregnancy.

Factors contributing to severe scoliosis after open chest surgery for congenital heart disease: a case-control analysis.

Previous reports have identified factors associated with open chest surgery for congenital heart disease (CHD) and scoliosis. However, these reports included conditions such as Down syndrome and Marfan syndrome, which involve both cardiac disease and scoliosis. The relationships between these factors and open chest surgery remain unclear. This study aimed to identify factors contributing to severe scoliosis in CHD patients who have undergone open chest surgery. Seventy-four post-CHD surgery patients with severe scoliosis (Scoliosis group) and 30 post-CHD surgery patients without scoliosis (NS group), excluding those with any syndrome or intellectual disability, were retrospectively analyzed. Patient background characteristics and radiographic parameters were compared between the NS and Scoliosis groups. Furthermore, the patients in the Scoliosis group were classified into three categories, namely, mild scoliosis, moderate scoliosis, and severe scoliosis, and the results were compared among the four groups. Eighteen patients in the NS group and 63 in the Scoliosis group met the inclusion criteria. Compared with the NS group, the Scoliosis group included significantly more girls and patients who had younger ages at first CHD surgery and multiple open chest surgeries. Severe scoliosis progression was observed in patients who underwent multiple surgeries for severe CHD with cardiomegaly. Progression to severe scoliosis was noted in patients with younger ages at first CHD surgery and those who underwent multiple surgeries for severe CHD. Assessing spinal deformities should be a key aspect of postoperative care for CHD, particularly in patients with severe CHD who are undergoing multiple chest surgeries. III.

Abstract 4137064: High Incidence of Adverse Neonatal Outcomes of Pregnancy in Women with Congenital Heart Disease

Background: Neonates born to women with congenital heart disease (CHD) are at high risk for adverse outcomes. We sought to describe these outcomes and maternal/pregnancy risk factors. Methods: We analyzed linked clinical, administrative, and birth certificate data in 3 geographic areas in Georgia, North Carolina and Utah from 2011 through 2014. Birth certificates provided neonatal, maternal and pregnancy characteristics. Maternal CHD was identified from linked clinical and administrative data and categorized as severe , shunt , valve , or shunt+valve . Neonatal outcomes of interest included preterm delivery (&lt;37 wk), low birth weight (&lt;2500 g) (LBW), small for gestational age (SGA), neonatal intensive care unit admission (NICU), a composite “any poor outcome” (one or more of preterm, SGA, or NICU), and CHD. Neonatal outcomes were summarized using descriptive statistics. Differences by maternal CHD severity were assessed using Kruskal-Wallis and Chi-square tests for continuous and categorical variables, respectively. Association of maternal/pregnancy characteristics and neonatal outcomes were assessed using generalized estimating equations with logit link function clustered on mother and adjusted for mother’s age, birth year, and state. Results: We identified 2,422 liveborn neonates (median gestational age 38 wks, weight 3155 g) born to 1,987 women with CHD (34% severe , 32% non-White, median age 28yrs). Overall, 19% were preterm, 18% LBW and 14% SGA; 25% required NICU and 40% experienced the composite adverse outcome (preterm, SGA, and/or NICU). CHD was present in 4.2%. Infants born to mothers with severe CHD (vs. combined non-severe) were more likely to have an adverse composite outcome [aOR 2.1 (1.7, 2.5)] and CHD [aOR 17.1 (9.3, 31.5)]. Black race [vs. White, aOR 1.8 (1.4, 2.3)], pregestational diabetes (DM) [vs. no, aOR 2.5 (1.3, 5.0)], gestational hypertension (GHTN) [vs. no, aOR 2.1 (1.5, 3.1)], prior preterm birth [vs. no, aOR 2.0 (1.3, 3.1)] and multiples [vs. singleton, aOR 6.7 (3.8, 11.6)] were associated with increased risk of composite adverse outcome. Conclusions: Forty percent of neonates born to women with CHD experienced an adverse neonatal outcome. Risk increased with maternal CHD severity, Black race, GHTN, DM, prior preterm birth, and multiple pregnancy. These findings highlight the need for heightened awareness and surveillance of these risk factors and expert preconception and pregnancy counseling for woman with CHD.

Abstract 4137805: Loss to Follow-Up Among Adults with Congenital Heart Defects: A Report from Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE)

Background: Many of the 1.4 million adults with congenital heart disease (CHD) are lost to follow up (LTF). We aimed to identify factors associated with LTF and compare to those who remained in care. Methods: In the Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), we performed a cross-sectional survey in 2021-2023 of CHD survivors with a history of intervention at 11 centers in the Pediatric Cardiac Care Consortium, a large US-based registry of CHD procedures. Participants reported their cardiac history, general health, education, income, health insurance, healthcare utilization, and marital status. LTF was defined as having seen a cardiologist three or more years ago. Results: Among the 3109 respondents with CHD (median age 33) there were 1157 (37%) LTF and 1952 (63%) in care. Age, sex, marital status, and household income were not risk factors for LTF; but lack of health insurance, lower severity of CHD, and increasing time since last heart surgery were. Notably, respondents who reported being told in adolescence about the lifelong need for a cardiologist were almost three times as likely to report being in care (79% vs 28%, p&lt;0.0001). Among women, most reported no LTF if counseled about concerns with pregnancy or told to avoid pregnancy. The most common reasons for being LTF for 5 years or more (N=857) included feeling well (60%), not thinking there was a need for lifelong care (49%), and a doctor told them they no longer needed to see a cardiologist (47%). Those LTF were more likely to report excellent or very good general health, physical health, and quality of life than those not LTF. There were no differences in self-assessment of mental health between groups. Conclusion: Adults with CHD and LTF were more likely to report less severe heart defects, a younger age at last surgery and a lack of counseling about the need for lifelong care.

Abstract 4128352: Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes

Background: G6PD deficiency classically presents with hemolytic anemia, but associations have been described with atherogenesis and coronary artery disease. There are limited data that also suggest a link between G6PD deficiency and the development of congenital heart disease (CHD). Hypothesis: We hypothesize a higher incidence of CHD among individuals with G6PD deficiency as well as worse hospital outcomes for those with CHD and G6PD deficiency. Methods: We reviewed the Vizient® Clinical Data Base (a national, administrative database) from 10/2019-3/2024 for admissions of individuals aged 0-25 years with ICD-10 codes for moderate to severe CHD with and without and G6PD deficiency (D55.0, D75.A) to calculate and compare CHD incidence. Additional data included: demographics, hospital outcomes and costs. Hospital outcomes were compared using t-tests for normally distributed data, Mann-Whitney U test for non-normally distributed data and χ 2 for categorical data. Results: There were 213,708 admissions of individuals with CHD and &lt;1% had G6PD deficiency; findings in Table 1. Of the 6,363 admissions with G6PD, 289 (4.5%) had CHD and of 11,179,670 without G6PD, 213,419 (1.9%) had CHD ( p &lt;0.001). Patients with CHD and G6PD deficiency had male predominance and more Blacks (consistent with known G6PD deficiency epidemiology), longer length of stay and higher hospital costs than those without G6PD deficiency (Table 1). Conclusions: This review of a national database shows a higher incidence of CHD in admissions with G6PD deficiency as well as worse hospital outcomes (longer length of stay, higher costs) for those with CHD and comorbid G6PD deficiency. There were demographic differences, but these reflect the typical inheritance of G6PD deficiency. While there may be potential interplay between race/ethnicity and these outcomes as well, further investigation is needed.

Rationale and design of CHD PULSE: Congenital Heart Disease Project to Understand Lifelong Survivor Experience

BackgroundWith improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes. MethodsCHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least one intervention for CHD at one of 11 participating U.S. centers in the Pediatric Cardiac Care Consortium. Participants with CHD were asked to complete a 99-question survey on a variety of topics including: demographics, surgeries, health insurance, health care, heart doctors, general health, height and weight, education and work history, reproductive health (for women only), and COVID-19. To construct a control group for the study, siblings of survey respondents were invited to complete a similar survey. Descriptive statistics for demographics, disease severity, center, and method of survey completion were computed for participants and controls. Comparisons were made between participants and non-participants to assess for response bias and between CHD participants and sibling controls to assess for baseline differences. ResultsAmong the 14,322 eligible participants, there were 3133 respondents (21.9%) from 48 U.S. states with surveys returned for inclusion in the study. Sibling contact information was provided by 691 respondents, with surveys returned by 326 siblings (47.2%). The median age of participants was 32.8 years at time of survey completion, with an interquartile range of 27.2 years to 39.7 years and an overall range of 20.1 to 82.9 years. Participants were predominantly female (55.1%) and of non-Hispanic White race/ethnicity (87.1%). There were no differences between participants and non-participants regarding severity of CHD. Compared to non-participants, participants were more likely to be female, of older age, and be of non-Hispanic White race/ethnicity. Enrolled siblings were more likely to be female and slightly younger than participants. ConclusionsWith surveys from 3133 participants from across the U.S., CHD PULSE is poised to provide keen insights into the lifelong journey of those living with CHD, extending beyond mere survival. These insights will offer opportunities for informing strategies to enhance and improve future outcomes for this population of patients.

Advance Care Planning for Adults With Moderate-to-Severe Congenital Heart Disease: Readiness Trial

BackgroundAdults with congenital heart disease (CHD) are growing in number and living longer with complex lesions; however, many are at risk of death in midlife. Conversations about advance care planning (ACP) in this population have been found to be infrequent and not part of regular outpatient care. The intent of this study was to explore readiness of patients with adult CHD (ACHD) to discuss ACP and assess the impact of interventions to support ACP conversations. MethodsWe conducted a prospective, randomized, single-centre trial of adults with moderate to severe CHD. Simply, the control group was asked if they were ready to discuss their wishes if they were to become seriously ill. The intervention group was provided 2 additional resources with the readiness question, the modified Lyon Family-Centered ACP survey and a patient-partner created ACHD-ACP video. ResultsA total of 111 responses (control [n = 59] and intervention group [n = 52]) were collected for analysis. Women represented 59.0% of the participants. The mean age was 39.6 years (standard deviation = 14.8 years). No significant difference was found between the control and experimental groups’ readiness (94.9% and 90.4%, respectively). Most participants (92.8%) responded positively towards initiating conversations related to end-of-life and ACP discussions. ConclusionsWe found that adults with CHD are ready to have ACP conversations as part of their outpatient care. Patient preferences and values should guide ACP conversations; further research is needed to determine whether the modified Lyon Family-Centered ACP survey and ACHD-ACP video are helpful adjuncts for ACP in outpatient clinics.

Hearing loss in children with congenital heart disease

Background : Congenital heart disease (CHD) is a dangerous disease, about 50% of deaths will occur in the first month of life. In developed countries almost all types of congenital heart disease have been detected in infancy even at less than 1 month of age, whereas in developing countries many are only detected after the child is older, so in some severe CHD types may have died before being detected. Rubella infection in pregnancy or congenital rubella syndrome (CRS) can cause miscarriage, fetal death, or congenital abnormalities after birth. CRS is characterized by sensorineural hearing loss (SNHL), congenital cataracts or glaucoma, congenital heart disease, and developmental delays. Purpose : This case report was submitted in order to find out how to properly treat patients with hearing loss with sensory deafness and congenital heart disease. Case : Reported a 3-year-old 6-month-old boy diagnosed with very severe degree sensorineural deafness and congenital heart disease based on anamnesis, physical examination and examination of OAE, BERA and ASSR for examination of auditory function) and echocardiography examination for examination of cardiac function. Conclusion : From the history, physical examination and examination of OAE, BERA, ASSR showed patients with very severe sensorineural hearing loss of the left and right ear and congenital heart disease, planned use of hearing aids or cochlear implantation and cardiac consul for CHD treatment.

A national Australian Congenital Heart Disease registry; methods and initial results

BackgroundAlthough several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia. MethodsWe defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code – Short List. Methodological limitations were carefully documented. ResultsFrom 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %). ConclusionThe data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.

Characteristics and maternal-fetal outcomes of pregnant women with critical congenital heart disease from a single center in China

Objective: To summarize the characteristics of pregnant women with critical congenital heart disease, and to explore continuous, integrated, multidisciplinary management for this segment of population. Methods: The clinical records of pregnant women with severe congenital heart disease with a history of intensive care who were treated in Guangdong Provincial People's Hospital from January 1, 2008 to December 31, 2020 were retrospectively analyzed. Results: (1) A total of 132 cases were included, including 128 pregnant women [gestational age (28.0±8.8) weeks] and 4 puerpera cases (6-32 days postpartum), 63.6% (84/132) from economic underdeveloped rural areas, and 78.0% (103/132) by the municipal hospital, irregular prenatal examination accounted for 59.1% (78/132). The main type of congenital heart disease was shunt lesion (55.3%, 73/132). 90.9% (120/132) with mWHO risk classification stage Ⅳ were assigned to it. The main cardiovascular complication was pulmonary hypertension (64.4%, 85/132). 46.2% (61/132) of the patients had been diagnosed with congenital heart disease before pregnancy, and 70.5% (93/132) of the patients had not received any treatment before pregnancy. (2) All patients received obstetric-led, multidisciplinary care. The rescue success rate was 96.2% (127/132), and no serious obstetric complications occurred. The mortality within 24 hours after discharge was 3.8% (5/132). 16.7% (22/132) underwent cardiac surgery during pregnancy, of which 77.3% (17/22) continued their pregnancy beyond 34 weeks. Totally, the delivery week was (30.5±8.6) weeks, and the main mode was cesarean section (71.2%, 94/132). The average weight of 99 live births (including 1 twin pregnancy) was (2 167±698) g. Preterm birth, fetal growth restriction, and congenital malformations were the main fetal comorbidities. Conclusions: Pregnant women with severe congenital heart disease mainly come from areas with underdeveloped economic and medical levels. Later disease intervention, pregnancy retention despite of clear pregnancy contraindications are the distinctive features, which leaded to a significant increase of incidence of maternal and fetal complications, and an increase of the consumption of medical resources. Multidisciplinary active treatment and cardiac surgery during pregnancy could relatively improve maternal and fetal pregnancy outcomes.

Preeclampsia and neonatal outcomes in pregnancies with maternal congenital heart disease: A nationwide cohort study from Norway.

The prevalence of congenital heart disease (CHD) among women of reproductive age is rising. We aimed to investigate the risk of preeclampsia and adverse neonatal outcomes in pregnancies of mothers with CHD compared to pregnancies of mothers without heart disease. In a nationwide cohort of pregnancies in Norway 1994-2014, we retrieved information on maternal heart disease, the course of pregnancy, and neonatal outcomes from national registries. Comparing pregnancies with maternal CHD to pregnancies without maternal heart disease, we used Cox regression to estimate the adjusted hazard ratio (aHR) for preeclampsia and log-binomial regression to estimate the adjusted risk ratio (aRR) for adverse neonatal outcomes. The estimates were adjusted for maternal age and year of childbirth and presented with 95% confidence intervals (CIs). Among 1 218 452 pregnancies, 2425 had mild maternal CHD, and 603 had moderate/severe CHD. Compared to pregnancies without maternal heart disease, the risk of preeclampsia was increased in pregnancies with mild and moderate/severe maternal CHD (aHR1.37, 95% CI 1.14-1.65 and aHR 1.62, 95% CI 1.13-2.32). The risk of preterm birth was increased in pregnancies with mild maternal CHD (aRR 1.33, 95% CI 1.15-1.54) and further increased with moderate/severe CHD (aRR 2.49, 95% CI 2.03-3.07). Maternal CHD was associated with elevated risks of both spontaneous and iatrogenic preterm birth. The risk of infants small-for-gestational-age was slightly increased with mild maternal CHD (aRR 1.12, 95% CI 1.00-1.26) and increased with moderate/severe CHD (aRR 1.63, 95% CI 1.36-1.95). The prevalence of stillbirth was 3.9 per 1000 pregnancies without maternal heart disease, 5.6 per 1000 with mild maternal CHD, and 6.8 per 1000 with moderate/severe maternal CHD. Still, there were too few cases to report a significant difference. There were no maternal deaths in women with CHD. Moderate/severe maternal CHD in pregnancy was associated with increased risks of preeclampsia, preterm birth, and infants small-for-gestational-age. Mild maternal CHD was associated with less increased risks. For women with moderate/severe CHD, their risk of preeclampsia and adverse neonatal outcomes should be evaluated together with their cardiac risk in pregnancy, and follow-up in pregnancy should be ascertained.

Self-Reported Management of Incidentally Detected Heart Murmurs in Puppies: A Survey among Veterinarians.

Heart murmurs in puppies can be innocent or pathologic; the latter is almost always related to a congenital heart disease. Differentiating between these murmurs can be challenging for practicing veterinarians, but this differentiation is essential to ensure the best prognosis for puppies having a congenital heart disease. Our study aimed to reveal how veterinarians manage puppies with a heart murmur. A web-based questionnaire was sent to Dutch and Belgian veterinary practices. Data from 452 respondents were analyzed. Though 88% of the respondents find detecting a heart murmur easy, only 9% find differentiating innocent murmurs from pathologic murmurs in puppies easy. Of the respondents, only 80% recommend immediate additional examination when detecting a loud heart murmur during the first veterinary health check at 6 weeks of age. Most of the respondents are aware that normal growth and the absence of clinical signs do not exclude severe congenital heart disease. Of the respondents, 31% were uncertain whether early surgical intervention could lead to improved outcomes. Veterinarians are aware of the importance of echocardiography for puppies with a loud heart murmur, and recognize their limitations when differentiating an innocent from a pathological heart murmur in a puppy.

Remote magnetic navigation for catheter ablation of complex arrhythmias in congenital heart disease: an initial experience using a transnational telerobotic approach

Abstract Background The field of congenital electrophysiology (EP) is still in its relative infacncy with a limited number of electrophysiologists capable of performing catheter ablation in patients with congenital heart disease (CHD). As a result, patients or physicians are often required to travel great distances to performed specialised ablation procedures, something made near impossible during the COVID-19 pandemic. Objective We aimed to explore the feasibility and safety of a telerobotic approach to catheter ablation where the remote operator was situated approximately 1200km away from the patient and local team. Methods A 3D-printed 'phantom' heart was used in a pilot experiment in which no significant difference was seen between the local and remote operators in electroanatomic (EAM) mapping of the phantom heart using remote magnetic navigation (RMN). Catheter ablation was subsequently performed in patients with severe CHD using a transnational telerobotic approach to catheter ablation where the remote operator was able to view the dedicated console with live fluoroscopy, EAM and EP signals, as well as control the mapping and ablation catheters from 1200km away. As a safety precaution, radiofrequency (RF) could only be delivered by the local team. Results In total, 10 procedures were performed in 9 patients with severe CHD (mean age 38) between April and December 2021. All patients underwent an atrial ablation and one additional non-CHD patient underwent denervation of the pulmonary arteries. Median procedure duration was 335 (minutes) (IWR 230-349) with 11.5 minutes (QRS 3-15.4) of fluoroscopy exposure and 752 (IQR 674-905) seconds of RF delivery. There were no acute procedural complications. At one-year follow-up, one patient died as a result of heart failure and there were two arrhythmia recurrences. Conclusions Telerobotic catheter ablation is feasible and effective and creates the possibility of exciting new approaches to remote ablation and its use in the tele-proctoring of international fellows.

A “twelve-section ultrasonic screening and diagnosis method” and management system for screening and treating neonatal congenital heart disease at the grassroots level in Tang County, Hebei Province, China

BackgroundTo explore a method for screening and diagnosing neonatal congenital heart disease (CHD) applicable to grassroots level, evaluate the prevalence of CHD, and establish a hierarchical management system for CHD screening and treatment at the grassroots level.MethodsA total of 24,253 newborns born in Tang County between January 2016 and December 2020 were consecutively enrolled and screened by trained primary physicians via the “twelve-section ultrasonic screening and diagnosis method” (referred to as the “twelve-section method”). Specialized staff from the CHD Screening and Diagnosis Center of Hebei Children’s Hospital regularly visited the local area for definite diagnosis of CHD in newborns who screened positive. Newborns with CHD were managed according to the hierarchical management system.ResultsThe centre confirmed that, except for 2 newborns with patent ductus arteriosus missed in the diagnosis of ventricular septal defect combined with severe pulmonary hypertension, newborns with other isolated or concomitant simple CHDs were identified at the grassroots level. The sensitivity, specificity and diagnostic coincidence rate of the twelve-section method for screening complex CHD were 92%, 99.6% and 84%, respectively. A total of 301 children with CHD were identified. The overall CHD prevalence was 12.4‰. According to the hierarchical management system, 113 patients with simple CHD recovered spontaneously during local follow-up, 48 patients continued local follow-up, 106 patients were referred to the centre for surgery (including 17 patients with severe CHD and 89 patients with progressive CHD), 1 patient died without surgery, and 8 patients were lost to follow-up. Eighteen patients with complex CHD were directly referred to the centre for surgery, 3 patients died without surgery, and 4 patients were lost to follow-up. Most patients who received early intervention achieved satisfactory results. The mortality rate of CHD was approximately 28.86 per 100,000 children.ConclusionsThe “twelve-section method” is suitable for screening neonatal CHD at the grassroots level. The establishment of a hierarchical management system for CHD screening and treatment is conducive to the scientific management of CHD, which has important clinical and social significance for early detection, early intervention, reduction in mortality and improvement of the prognosis of complex and severe CHDs.

Health Service Use and Costs During Pregnancy Among Privately Insured Individuals With Congenital Heart Disease

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. To examine health care use and costs for patients with CHD during pregnancy. 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. Baseline characteristics (age, US region, delivery year, insurance type) and pregnancy-related events (obstetric, cardiac, and noncardiac conditions; birth outcomes; and cesarean delivery). 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). 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. 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.

Fluid Dynamic Study of the Penn State Pediatric Total Artificial Heart.

Penn State University is developing a pediatric total artificial heart (TAH) as a bridge-to-transplant device that supports infants and small children with single ventricle anomalies or biventricular heart failure to address high waitlist mortality rates for pediatric patients with severe congenital heart disease (CHD). Two issues with mechanical circulatory support devices are thrombus formation and thromboembolic events. This in vitro study characterizes flow within Penn State's pediatric total artificial heart under physiological operating conditions. Particle image velocimetry (PIV) is used to quantify flow within the pump and to calculate wall shear rates (WSRs) along the internal pump surface to identify potential thrombogenic regions. Results show that the diastolic inflow jets produce sufficient wall shear rates to reduce thrombus deposition potential along the inlet side of the left and right pumps. The inlet jet transitions to rotational flow, which promotes wall washing along the apex of the pumps, prevents flow stasis, and aligns flow with the outlet valve prior to systolic ejection. However, inconsistent high wall shear rates near the pump apex cause increased thrombogenic potential. Strong systolic outflow jets produce high wall shear rates near the outlet valve to reduce thrombus deposition risk. The right pump, which has a modified outlet port angle to improve anatomical fit, produces lower wall shear rates and higher thrombus susceptibility potential (TSP) compared to the left pump. In summary, this study provides a fluid dynamic understanding of a new pediatric total artificial heart and indicates thrombus susceptibility is primarily confined to the apex, consistent with similar pulsatile heart pumps.

Machine Learning to Predict Outcomes of Fetal Cardiac Disease: A Pilot Study.

Prediction of outcomes following a prenatal diagnosis of congenital heart disease (CHD) is challenging. Machine learning (ML) algorithms may be used to reduce clinical uncertainty and improve prognostic accuracy. We performed a pilot study to train ML algorithms to predict postnatal outcomes based on clinical data. Specific objectives were to predict (1) in utero or neonatal death, (2) high-acuity neonatal care and (3) favorable outcomes. We included all fetuses with cardiac disease at Sunnybrook Health Sciences Centre, Toronto, Canada, from 2012 to 2021. Prediction models were created using the XgBoost algorithm (tree-based) with fivefold cross-validation. Among 211 cases of fetal cardiac disease, 61 were excluded (39 terminations, 21 lost to follow-up, 1 isolated arrhythmia), leaving a cohort of 150 fetuses. Fifteen (10%) demised (10 neonates) and 65 (48%) of live births required high acuity neonatal care. Of those with clinical follow-up, 60/87 (69%) had a favorable outcome. Prediction models for fetal or neonatal death, high acuity neonatal care and favorable outcome had AUCs of 0.76, 0.84 and 0.73, respectively. The most important predictors for death were the presence of non-cardiac abnormalities combined with more severe CHD. High acuity of postnatal care was predicted by anti Ro antibody and more severe CHD. Favorable outcome was most predicted by no right heart disease combined with genetic abnormalities, and maternal medications. Prediction models using ML provide good discrimination of key prenatal and postnatal outcomes among fetuses with congenital heart disease.

Neonatal outcomes in preterm infants with severe congenital heart disease: a national cohort analysis.

Prematurity and congenital heart disease (CHD) are the leading causes of neonatal mortality and morbidity. Limited data are available about the outcomes of premature infants with severe CHD. We queried The National Inpatient Database using ICD-10 codes for premature patients (<37 weeks) with severe CHD from 2016 to 2020. Severe CHDs were grouped into three categories: A. left-sided lesions with impaired systemic output, B. Cyanotic CHD, and C. Shunt lesions with pulmonary overcirculation. Patients with isolated atrial or ventricular septal defects and patent ductus arteriosus were excluded. We also excluded patients with chromosomal abnormalities and major congenital anomalies. Patients' demographics, clinical characteristics, and outcomes were evaluated by comparing premature infants with vs. without CHD adjusting for gestational age (GA), birth weight, and gender. A total of 27710 (1.5%) out of 1,798,245 premature infants had severe CHD. This included 27%, 58%, and 15% in groups A, B, and C respectively. The incidence of severe CHD was highest between 25 and 28 weeks of gestation and decreased significantly with increasing GA up to 36 weeks (p < 0.001). Premature infants with severe CHD had a significantly higher incidence of neonatal morbidities including necrotizing enterocolitis (NEC) [OR = 4.88 (4.51-5.27)], interventricular hemorrhage [OR = 6.22 (5.57-6.95)], periventricular leukomalacia [OR = 3.21 (2.84-3.64)] and bronchopulmonary dysplasia [OR = 8.26 (7.50-10.06) compared to preterm infants of similar GA without CHD. Shunt lesions had the highest incidence of NEC (8.5%) compared to 5.3% in cyanotic CHD and 3.7% in left-sided lesions (p < 0.001). Mortality was significantly higher in premature infants with CHD compared to control [11.6% vs. 2.5%, p < 0.001]. Shunt lesions had significantly higher mortality (11.0%) compared to those with left-sided lesions (8.3%) and cyanotic CHD (6.4%), p < 0.001. Premature infants with severe CHD are at high risk of neonatal morbidity and mortality. Morbidity remains increased across all GA groups and in all CHD categories. This significant risk of adverse outcomes is important to acknowledge when managing this patient population and when counseling their families. Future research is needed to examine the impact of specific rather than categorized congenital heart defects on neonatal outcomes.

Morbidity and mortality in premature or low birth weight patients with congenital heart disease in three European pediatric heart centers between 2016 and 2020.

The treatment of preterm and low birth weight (LBW) neonates born with congenital heart disease (CHD) requiring early cardiac intervention remains challenging. We aimed to analyze morbidity and mortality in this combined high-risk patient group. A retrospective cohort study was conducted of preterm [<37 weeks gestational age (GA)] and/or LBW neonates (<2,500 g) born with a diagnosis of CHD, which requires invasive cardiac intervention (surgery or catheter) within their first year of life. Patients born between 2016 and 2020 and treated in three European pediatric heart centers were included. A total of 308 neonates (51% male) with CHD were included. Of those, 237 (77%) were born preterm, 259 (84%) were LBW, and 188 (61%) were both. The median GA was 35.4 weeks (interquartile range 33.3-36.9) and the mean birth weight was 2,016 ± 580 g. CHD was categorized as simple (12%), moderate (64%), or severe (24%). The overall complication rate was 45% and was highest in patients with severe CHD (p = 0.002). One-year mortality (19%) was associated with severe CHD, low relative birth weight in patients with genetic diagnoses, and low GA at birth, whereas GA at birth significantly impacted survival only after 3 months of life. The high morbidity and mortality in preterm and LBW neonates with CHD reflect their complexity and consequent limited treatment feasibility.