Congenital Heart Disease Survivors Research Articles

Cardiovascular disease risk factors in congenital heart disease survivors are associated with heart failure.

Despite advances in treatment and survival, individuals with congenital heart defects (CHD) have a higher risk of heart failure (HF) compared to the general population. To evaluate comorbidities associated with HF in patients with CHD with a goal of identifying potentially modifiable risk factors that may reduce HF-associated morbidity and mortality. Five surveillance sites in the United States linked population-based healthcare data and vital records. Individuals with an ICD-9-CM code for CHD aged 11-64 years were included and were stratified by presence of HF diagnosis code. Prevalence of death and cardiovascular risk factors based on diagnosis codes were compared by HF status using log-linear regression. A total of 25,343 individuals met inclusion/exclusion criteria. HF was documented for 2.2% of adolescents and 12.9% of adults with CHD. Adolescents and adults with HF had a higher mortality than those without HF. In both age groups, HF was positively associated with coronary artery disease, hypertension, obesity, diabetes, and increased healthcare utilization compared to those without HF. Within this population-based cohort, over 1 in 50 adolescents and 1 in 8 adults with CHD had HF, which was associated with increased mortality. Modifiable cardiovascular comorbidities were associated with HF. Five sites in the United States linked population-based healthcare data and vital records to establish surveillance network for identifying the factors which influence congenital heart disease (CHD) outcomes. Survivors of CHD frequently develop heart failure across the lifespan. Over 1 in 50 adolescent and 1 in 8 adult survivors of CHD have heart failure which is associated with increased mortality compared to CHD survivors without heart failure. Heart failure development is associated with potentially modifiable cardiovascular risk factors such as hypertension, coronary artery disease, and diabetes. Controlling modifiable cardiovascular risk factors may serve to lower the risk of heart failure and mortality in survivors of congenital heart disease of all ages.

Language brain responses and neurodevelopmental outcome in preschoolers with congenital heart disease: A fNIRS study

Neurodevelopmental disabilities affect up to 50% of survivors of congenital heart disease (CHD). Language difficulties are frequently identified during preschool period and can lead to academic, social, behavioral, and emotional difficulties. Structural brain alterations are associated with poorer neurodevelopmental outcomes in patients with CHD during infancy, childhood, and adolescence. However, evidence is lacking about the functional brain activity in children with CHD and its relationship with neurodevelopment. This study therefore aimed to characterize brain responses during a passive story-listening task in 3-year-old children with CHD, and to investigate the relationship between functional brain patterns of language processing and neurodevelopmental outcomes. To do so, we assessed hemodynamic concentration changes, using functional near-infrared spectroscopy (fNIRS), and neurodevelopmental outcomes, using the Wechsler Preschool and Primary Scale of Intelligence – 4th Edition (WPPSI-IV), in children with CHD (n = 19) and healthy controls (n = 23). Compared to their healthy peers, children with CHD had significantly lower scores on the Verbal comprehension index (VCI), the Vocabulary acquisition index (VAI), the General ability index (GAI), and the Information and the Picture Naming subtests of the WPPSI-IV. During the passive story-listening task, healthy controls showed significant hemodynamic brain responses in the temporal and the temporal posterior regions, with stronger activation in the temporal posterior than in the temporal regions. In contrast, children with CHD showed reduced activation in the temporal posterior regions compared to controls, with no difference of activation between regions. Reduced brain responses in the temporal posterior regions were also correlated with lower neurodevelopmental outcomes in both groups. This is the first study that reveals reduced brain functional responses in preschoolers with CHD during a receptive language task. It also suggests that the temporal posterior activation could be a potential brain marker of cognitive development. These findings provide support for the feasibility of identifying brain correlates of neurodevelopmental vulnerabilities in children with CHD.

Survival outcomes for congenital heart disease from Southern Malaysia: results from a congenital heart disease registry.

Limited population-based studies are available on the survival of congenital heart disease (CHD) from lower- and middle-income countries. Therefore, we evaluated the survival from birth until 15 years and associated factors for mortality. This population-based cohort study included all children with CHD registered in the Pediatric Cardiology Clinical Information System born between 2006 and 2020 in Johor, Malaysia. The mortality rate was calculated, and Cox proportional hazard regression analysis was used to determine factors associated with mortality. The Kaplan-Meier analysis was used to estimate the survival rates at 1, 5, 10 and 15 years. There were 5728 patients with CHD studied, with 1543 (27%) lesions resolved spontaneously, 322 (5.6%) were treated with comfort care, 1189 (21%) required no intervention, and 2674 (47%) needed surgery or intervention. The overall mortality rate was 15%, with a median age of death of 3.7 months (IQR 0.9-9.8 months). Preoperative/intervention death was observed in 300 (11%), and 68 (3.2%) children died within 30 days of surgery or intervention. The overall estimated survival at 1, 5, 10 and 15 years was 88%, 85%, 84% and 83%, respectively. The independent factors associated with mortality were male gender, associated syndrome or extra-cardiac defect, pulmonary hypertension, antenatal diagnosis and severe lesions. Eight out of 10 patients with CHDs survived up to 15 years of age. However, 10% of CHDs who require intervention die before the procedure. Thus, improving congenital cardiac surgery and enhancing the overall healthcare system are crucial to improve survival.

MATERNAL PREGNANCY OUTCOMES IN WOMEN WITH CARDIOMYOPATHY: A SYSTEMATIC REVIEW

Introduction: Pregnancy poses significant cardiovascular challenges, affecting 1 to 4 of pregnancies due to maternal heart disease. Cardiomyopathies, leading indirect causes of maternal mortality globally, raise concerns during pregnancy, despite limited data on their impact and the accuracy of predictive scores like Carpreg and Zahara. Maternal heart disease, comprising hypertensive disorders and cardiomyopathies, presents substantial risks during pregnancies, driving increased maternal mortality rates. The evolving landscape, marked by improved survival in congenital heart disease and delayed pregnancies with associated comorbidities, necessitates a more nuanced understanding and comprehensive predictive models for tailored care during pregnancy. Methods: The researchers in this study followed the 2020 Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines to ensure that their work met the required standards. This was done to ensure the precision and reliability of the conclusions derived from the research. Result: Our search produced 15 results. After looking at the titles and summaries, we found 11 papers that fit our criteria. At first, we excluded several articles because they were written in review and case report style. But after reading the full papers carefully, we included five papers in our final analysis. These papers included prospective and retrospective studies. Conclusion: The investigation of pregnant women in Canadian obstetric centers unveiled that cardiac issues affect 16 of pregnancies, primarily with arrhythmias and heart failure, even in lower-risk cases, with complications emerging before birth. The study's precision in evaluating pregnancy-related cardiac risks, leading to a new CARPREG II risk index and emphasizing tailored surveillance and timely referrals, was notable. The ROPAC registry highlighted higher maternal mortality rates, stressing vigilant management throughout pregnancy and after delivery, emphasizing the necessity for specialized care and research advancements in this field.

CE Workshop 06: The Cumulative Burden of Congenital Heart Disease Across the Lifespan: Implications for Neuropsychologists in Pediatrics Through Geriatrics

Abstract & Learning Objectives:The heart and the brain are inextricably linked across development by overlapping genetic programs and transacting physiologies that exist long before birth and endure throughout the lifespan. Congenital heart disease (CHD) refers to a diverse array of conditions in which structural heart development is atypical. Of the roughly 1 million babies born with CHD each year, some 40,000 are born with a “critical” form of CHD that will require intensive surgical intervention within the first year of life. As recently as the 1980s, children born with some forms of critical CHD did not survive; palliation was their only option. This has changed dramatically over the past 30-40 years. Driven by momentous breakthroughs in medical science and technology, approximately 80-95% of children born with CHD today will reach adulthood.But increased survival is only a part of the CHD story. Indeed, like extreme prematurity, leukemia, and many other previously fatal medical conditions with which neuropsychologists are familiar, increases in longevity among CHD survivors have come with increasing recognition of the many challenging transitions and cumulative medical, neurobehavioral, and psychosocial burdens inherent to “living with CHD.” CHD begins to alter expected brain development in utero with evidence of structural, volumetric, and metabolic differences documented as early as the second or third prenatal trimester. Brain dysmaturation, in turn, increases one’s risk for further acquired brain injury and gives rise to a range of neurobehavioral deficits and psychosocial difficulties that consistently rank among the most salient threats to quality of life among children, adolescents, and adults with CHD.More recently, as survival into adulthood has become increasingly common for individuals with CHD, we have also begun to more fully appreciate the cascading impact and cumulative neuropsychological burden of CHD across the lifespan, which impact a range of long-term outcomes such as educational and occupational attainment, living independently, and risk for dementia.In short, CHD can no longer reasonably be considered a child or pediatric condition, but rather a lifespan condition with the potential to adversely impact neurobehavioral and psychosocial outcomes in different ways and at different times across infancy, childhood, adolescence, and adulthood. Over a series of talks presented by a panel of recognized neuropsychologists and experts in CHD, this symposium aims to review the neuropsychology of CHD across the lifespan and to present an integrative lifespan developmental neuropsychological model of CHD that eschews prevailing “child” vs. “adult” distinctions. Each presentation will address a salient developmental epoch (prenatal-early childhood, school-age/adolescence, and adulthood/geriatric timeframes) and will include a comprehensive review of the extant literature pertaining to relevant neuroanatomical and neurodevelopmental/neuropsychological considerations for individuals with CHD during each epoch. Transitions, of which there are myriad for individuals living with CHD (e.g., from acute inpatient care to stepdown unit care; from inpatient to outpatient settings; from early intervention to the school system; from childhood to adolescence; from adolescence to young adulthood; from pediatric to adult CHD care), will feature prominently throughout the symposium, as will recommendations for competent, developmentally-informed clinical neuropsychological management and intervention planning throughout the lifespan. Upon conclusion of this course, learners will be able to:1.Describe the mechanisms by which congenital heart disease (CHD) impacts brain development and functioning across the lifespan (from infancy to older adulthood).2.Discuss neurodevelopmental/neuropsychological sequelae of CHD for children, adolescents, and adults.3.Explain the role of clinical neuropsychologists in evaluating, supporting, and optimizing the neuropsychological trajectories of individuals with CHD across the lifespan.

Cardiogenic shock in pregnancy.

Cardiac disease complicates 1%-4% of pregnancies globally, with a predominance in low and middle-income countries (LMICs). Increasing maternal age, rates of obesity, cardiovascular comorbidities, pre-eclampsia and gestational diabetes all contribute to acquired cardiovascular disease in pregnancy. Additionally, improved survival in congenital heart disease (CHD) has led to increasing numbers of women with CHD undergoing pregnancy. Implementation of individualised care plans formulated through pre-conception counselling and based on national and international guidance have contributed to improved clinical outcomes. However, there remains a significant proportion of women of reproductive age with no apparent comorbidities or risk factors that develop heart disease during pregnancy, with no indication for pre-conception counselling. The most extreme manifestation of cardiac disease is cardiogenic shock (CS), where the primary cardiac pathology results in inadequate cardiac output and hypoperfusion, and is associated with significant mortality and morbidity. Key to management is early recognition, intervention to treat any potentially reversible underlying pathology and supportive measures, up to and including mechanical circulatory support (MCS). In this narrative review we discuss recent developments in the classification of CS, and how these may be adapted to improve outcomes of pregnant women with, or at risk of developing, this potentially lethal condition.

School Readiness Among Children With Congenital Heart Disease.

School Readiness Among Children With Congenital Heart Disease.

Double-Trouble: Atherosclerotic Risk Factors and Congenital Heart Disease.

Youth with congenital heart disease (CHD) are uniquely vulnerable to genetic and acquired atherosclerotic cardiovascular disease (ASCVD) risk factors. With the increasingly successful management of CHD, it is important to prevent or optimally managed risk factors with the goal of improving outcomes and longevity. This review summarizes guidelines for the evaluation and management of obesity, dyslipidemia, and hypertension in youth (< 18years of age), focusing on the special vulnerabilities associated with the type of repair and the presence of residual disease in those who undergo cardiac surgery. Clinicians must focus on targeting these highly prevalent ASCVD risk factors to protect CHD survivors from preventable ASCVD morbidity and mortality by applying lifestyle, pharmacologic, or surgical therapies as needed. Future work should examine interventions to identify and treat ASCVD risk factors in CHD patients. Given the increased prevalence of ASCVD risk factors in youth and the morbidity and premature mortality associated with CHD, it is important for clinicians to assess global risk factors in these patients frequently, encourage adherence to lifestyle changes, and recommend pharmacotherapy and surgical interventions when clinically indicated. Future efforts should identify barriers and opportunities for improving risk factor assessment and timely intervention as a routine part of clinical care.

Sex-Specific Cerebral Blood Flow Alterations in Youth Operated for Congenital Heart Disease.

Background Lower cerebral blood flow (CBF) has previously been documented preoperatively in neonates with congenital heart disease (CHD). However, it remains unclear if these CBF deficits persist over the life span of CHD survivors following heart surgery. When exploring this question, it is critical to consider the sex differences in CBF that emerge during adolescence. Therefore, this study aimed to compare global and regional CBF between postpubertal youth with CHD and healthy peers and examine if such alterations are related to sex. Methods and Results Youth aged 16 to 24 years who underwent open heart surgery for complex CHD during infancy and age- and sex-matched controls completed brain magnetic resonance imaging, including T1-weighted and pseudo-continuous arterial spin labeling acquisitions. Global gray matter CBF and regional CBF in 9 bilateral gray matter regions were quantified for each participant. Compared with female controls (N=27), female participants with CHD (N=25) presented with lower global and regional CBF. In contrast, there were no differences in CBF between male controls (N=18) and males with CHD (N=17). Concurrently, female controls had higher global and regional CBF compared with male controls, with no differences in CBF between female and male participants with CHD. CBF was lower in individuals with a Fontan circulation. Conclusions This study provides evidence of altered CBF in postpubertal female participants with CHD despite undergoing surgical intervention during infancy. Alterations to CBF could have implications for later cognitive decline, neurodegeneration, and cerebrovascular disease in women with CHD.

Age moderates change in disease-related stress among congenital heart disease survivors: a 6-year follow-up.

As congenital heart disease (CHD) survivors age, they are confronted with elevated risk of cardiovascular morbidity and increasingly complex disease self-management demands. Given that stress is associated with poor physical and psychosocial outcomes, it is crucial to examine how disease-related stress changes over time in this population. However, this outcome has received little research attention to date. This study aimed to identify demographic and clinical predictors of change in disease-related stress over 6 years among CHD survivors. Congenital heart disease survivors (N = 252, Mage = 25.6 ± 7.1, 52.9% female) completed the first 13 items of the Responses to Stress Questionnaire, adapted for use among CHD survivors, to assess disease-related stressors at study entry (T1) and 6-year follow-up (T2). Age, gender, estimated family income, and New York Heart Association (NYHA) functional class at T1 were entered into mixed linear models to determine their impact on change in disease-related stress. Older age (P < 0.001), lower income (P < 0.001), and presence of functional limitations (NYHA ≥ II) (P < 0.001) predicted greater increases in disease-related stress. When controlling for NYHA, functional class, and income, a significant time by age interaction was identified such that disease-related stress increased over time among those who were adolescents at T1 [b = 4.20, P = 0.010, 95% confidence interval (1.01, 7.40)], but remained stable among young adults. The transition from adolescence to adulthood may be a period of increasing disease-related stress. Healthcare providers should consider screening adolescents for elevated disease-related stress during transition education and provide resources to bolster resilience.

PO-04-145 ATRIAL FIBRILLATION AND PULMONARY HYPERTENSION IN ADULT CONGENITAL HEART DISEASE

ith the improvement of surgery and perioperative care, the survival of congenital heart disease (CHD) has improved much in recent decades. The long-term complication especially atrial fibrillation (Af) has gained attention. With a large ACHD cohort, we tried to find out the epidemiology data and risk factors of Af in adult CHD patients and see the impact of Af on the late outcome. From 2007 to 2018, all patients older than 18 years of age and diagnosed as CHD in National Taiwan University Hospital were enrolled in our study. The basic clinical characteristics, ECG and Holter result, and follow-up data were collected through the National Taiwan University Hospital-Integrated Medical Database. Atrial fibrillation status was categorized as persistent Af, paroxysmal Af, and atrial flutter (AFL). CHD was categorized as simple, severe, or complex CHD (according to the World Symposia on Pulmonary Hypertension classification). There were 4403 patients (women, 55.9%), 15.4% with severe and 3.9% with complex CHD. The cumulative incidence of Af was 6.9% (54.7% as paroxysmal Af, 26.9% as persistent Af, and 18.4% as AFL). Mean onset age of AFL, paroxysmal Af, and persistent Af was 35.7±15.8, 48.4±19.3, and 57.2±14.3 years-old. The 70-years old cardiovascular accident (CVA) free survival rate was 67.1% in those with Af (vs. 80.7% in those without, p<0.001). The 5 years CVA free survival rate since the onset of Af was low in paroxysmal and persistent Af (71.2% and 72.1%) compared to those of AFL (91.5%, p=0.004). Patients with pulmonary hypertension has significantly higher risk of Af. The multivariable regression model indicated that Af and PAH were most significant risk factors of mortality (odds ratio 2.8 (95% CI 2.0-4.0) and 9.4 (95% CI 6.6-13.2) respectively, both p<0.001). In this large ACHD cohort, the cumulative incidence of Af was high, increasing with age and presence of PH. CVA and Mortality increased with presence of Af.

Impact of 2016 UNOS pediatric heart allocation policy changes on VAD utilization, waitlist, and post-transplant survival outcomes in children with CHD versus Non-CHD.

We analyzed the impact of the revised pediatric heart allocation policy on types of ventricular assist device (VAD) utilization, and waitlist (WL) and post-heart transplant (HT) survival outcomes in congenital heart disease (CHD) versus non-CHD patients before (Era-1) and after (Era-2) pediatric heart allocation policy implementation. We retrospectively reviewed the UNOS database from December 16, 2011, through March 31, 2021, for patients<18 years old and listed for primary HT. We compared the differences observed between Era-1 and Era-2. 5551 patients were listed for HT, of whom 2447(44%) were in Era-1 and 3104(56%) were in Era-2. CHD patients were listed as status 1A unchanged, but the number of patients listed as status 1B decreased in Era-2, whereas the number of non-CHD patients listed as status 1A decreased, but status 1B increased. In Era-2 compared to Era-1, both temporary (1% to 4%, p<.001) and durable VAD (13.6% to 17.8%, p<.001) utilization increased, and the transplantation rate per 100-patient years increased in both groups. The median WL period for CHD patients increased marginally from 70 to 71 days (p=.06), whereas for non-CHD patients it decreased from 61 to 54 days (p<.001). Adjusted 90-day WL survival increased from 84% to 88%, p=.016 in CHD, but there was no significant change in non-CHD patients (p=.57). There was no significant difference in 1-year post-HT survival in CHD and non-CHD patients between Era-1 and Era-2. In summary, after the revised heart allocation policy implementation, temporary and durable VAD support increased, HT rate increased, waitlist duration marginally increased in the CHD cohort and decreased in the non-CHD cohort, and 90-day WL survival probability improved in children with CHD without significant change in 1-year post-HT outcomes. Future studies are needed to identify changes to the policy that may further improve the listing criteria to improve WL duration and post-HT survival.

Abstract 15282: COVID-19 Cases, Hospitalizations, and Vaccination Among Adults With Congenital Heart Disease

Background: There are conflicting data as to whether congenital heart disease (CHD) should be considered a condition that places a person at high risk of severe illness from COVID-19. The objective of our study was to compare COVID-19 cases, hospitalization rate, and vaccine uptake among adults with history of cardiac intervention vs. the general US adult population. Methods: We performed a cross-sectional study of adults using survey data from the Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE). This survey, conducted in 2021-2022, queried CHD survivors regarding a variety of domains, including history of COVID-19 infection and vaccination. Adults with a history of surgical, catheter, or electrophysiologic intervention at 1 of 8 Pediatric Cardiac Care Consortium centers were eligible for inclusion. Results were limited to those aged 18-49 years to allow comparison to age-specific data from the US Centers for Disease Control and Prevention. Results: Among 1,277 respondents (20.2% response rate), the median age was 32 years (IQR: 27-38 years), the majority were female (55.6%), and the most common race/ethnicity was Non-Hispanic White (89.9%). There was no difference in history of a positive COVID-19 test in persons with CHD compared to adults of a similar age (26.8% vs 28.1%, p=0.86). However. those with CHD and COVID-19 were significantly more likely to require hospitalization than adults in the general population with COVID-19 (1.75% vs. 0.26%, p=0.004) (Figure). Regarding COVID-19 vaccination, 76.5% of CHD PULSE respondents reported a history of at least 1 COVID-19 vaccine, with no differences vs. the general population when compared by age (p=0.99). Conclusions: Given higher hospitalization rates among adults with CHD and COVID-19, CHD should be considered a medical condition that places one at increased risk of severe COVID-19. These findings underscore the importance of COVID-19 vaccination in this population.

Altered myelination in youth born with congenital heart disease.

Brain injury and dysmaturation is common in fetuses and neonates with congenital heart disease (CHD) and is hypothesized to result in persistent myelination deficits. This study aimed to quantify and compare myelin content in vivo between youth born with CHD and healthy controls. Youth aged 16 to 24 years born with CHD and healthy age‐ and sex‐matched controls underwent brain magnetic resonance imaging including multicomponent driven equilibrium single pulse observation of T1 and T2 (mcDESPOT). Average myelin water fraction (MWF) values for 33 white matter tracts, as well as a summary measure of average white matter MWF, the White Matter Myelination Index, were calculated and compared between groups. Tract‐average MWF was lower throughout the corpus callosum and in many bilateral association tracts and left hemispheric projection tracts in youth with CHD (N = 44) as compared to controls (N = 45). The White Matter Myelination Index was also lower in the CHD group. As such, this study provides specific evidence of widespread myelination deficits in youth with CHD, likely representing a long‐lasting consequence of early‐life brain dysmaturation in this population. This deficient myelination may underlie the frequent neurodevelopmental impairments experienced by CHD survivors and could eventually serve as a biomarker of neuropsychological function.

Impact of perinatal management on neurodevelopmental outcomes in congenital heart disease

With advancements in cardiopulmonary bypass technique and perioperative care, there has been a progressive decline in mortality associated with neonatal surgical correction of congenital heart disease (CHD). Thus, there is now increased focus on improving neurodevelopmental outcomes in CHD survivors. While the cause of these neurodevelopmental impairments is multifactorial, there is increasing evidence that structural and functional cerebral abnormalities are present before cardiac corrective repair. This suggests that in addition to patient specific risk factors, underlying cardiac physiology and clinical hemodynamics are critical to brain health and development. Prenatal diagnosis of CHD and subsequent optimization of perinatal care may therefore be important modifiable factors for long-term neurodevelopmental outcome.This article reviews the impact that prenatal diagnosis of CHD has on perinatal care and the preoperative clinical status of a neonate, as well as the potential influence this may have on lessening the degree of cerebral injury and long-term neurodevelopmental impairments.

Brain Oxygen Perfusion and Oxidative Stress Biomarkers in Fetuses with Congenital Heart Disease-A Retrospective, Case-Control Pilot Study.

Fetuses with congenital heart disease (CHD) have circulatory changes that may lead to predictable blood flow disturbances that may affect normal brain development. Hypoxemia and hypoperfusion may alter the redox balance leading to oxidative stress (OS), that can be assessed measuring stable end-products. OS biomarkers (OSB) were measured in amniotic fluid in fetuses with (n = 41) and without CHD (n = 44) and analyzed according to aortic flow, expected cyanosis after birth, and a CHD classification derived from this. Birth head circumference (HC) was used as a neurodevelopment biomarker. CHD fetuses had higher levels of ortho-Tyrosine (o-Tyr) than controls (p = 0.0003). There were no differences in o-Tyr levels considering aortic flow obstruction (p = 0.617). Fetuses with expected extreme cyanosis presented the highest levels of o-Tyr (p = 0.003). Among groups of CHD, fetuses without aortic obstruction and extreme cyanosis had the highest levels of o-Tyr (p = 0.005). CHD patients had lower HC than controls (p = 0.023), without correlation with OSB. Patients with HC < 10th percentile, presented high levels of o-Tyr (p = 0.024). Fetuses with CHD showed increased OSB and lower HC when compared to controls, especially those with expected extreme cyanosis. Our results suggest that increased levels of OSB are more influenced by the effect of low oxygenation than by aortic flow obstruction. Future studies with larger sample size are needed to further investigate the role of OSB as an early predictor of neurodevelopmental problems in CHD survivors.

Cognitive impairment in adult CHD survivors: A pilot study

The most common comorbidities in children with congenital heart disease (CHD) are neurodevelopmental impairments, particularly in areas of executive function, memory and attention. Limited studies have demonstrated similar impairments in CHD adults although no studies have screened specifically for mild cognitive impairment and dementia. Methods We performed a prospective cross-sectional study of CHD patients, ages 30-65 years, who were coming for clinic visits. We administered the Mini-Mental State Exam (MMSE), and scores were compared with population norms adjusted by age and education level. Results A total of 125 patients were recruited (55% male). The median age was 40 years (range 30-65). More than a half (80%) had some college education or advanced degrees. Adjusting for age and education, CHD participants scored significantly lower than the general population (median 1 point lower, p<0.001) on the MMSE. The greatest impairments occurred in recall and orientation. Five percent of the total cohort met the general threshold for mild cognitive impairment (MMSE < 24). Clinical factors associated with this degree of cognitive impairment were duration of cyanosis (p=0.005) and decreased systemic ventricular function (p=0.003). Conclusions Our pilot study showed that, when adjusted for age and education level, CHD adults had significantly lower MMSE scores than the general population, with 5% meeting criteria for mild cognitive impairment. These findings suggest that subtle and early cognitive changes are present in the adult CHD population. Further studies are needed to investigate those changes that might influence long-term outcomes in the adult CHD population.

Factors That Influence Adherence to Medication in Adults With Congenital Heart Disease (ACHD).

Objective: Innovative operative and interventional procedures have improved survival in congenital heart disease (CHD), and today more than 90% of these children reach adulthood. Consequently, adherence and psychosocial issues are becoming increasingly important because non-adherence to treatment recommendations worsens morbidity and mortality. This study aimed to identify factors modifying adherence to medication in adult congenital heart disease (ACHD).Methods: This cross-sectional study included 451 outpatients (female 47.9%, average age ± SD: 37.9 ± 12 years) from the ACHD department, who completed a questionnaire assessing medication non-adherence and individual barriers to treatment. Further assessments included psychological well-being (Hospital Anxiety and Depression scale; HADS), childhood traumatization, sociodemographic, and clinical data. Binary logistic regression analysis calculated the impact of these factors on drug adherence.Results: Of the 451 patients 162 participants (35.9%) reported to be non-adherent. In univariate analysis non-adherence to treatment was associated with smoking (P = < 0.001) and internet addiction (P = 0.005). Further factors negatively influencing adherence were the presence of depressive symptoms (P = 0.002), anxiety (P = 0.004), and childhood traumatization (p = 0.002). Factors positively associated with adherence were older age (P = 0.003) and more advanced heart disease as indicated by NYHA class (P = 0.01), elevated NT-proBNP (P = 0.02), device therapy (P = 0.002) and intermittent arrhythmias (P = 0.01). In multivariate analysis especially psychopathological factors such as depression (P = 0.009), anxiety (P = 0.032) and childhood traumatization (P = 0.006) predicted non-adherence.Conclusion: Adherence is a critical issue in the long-term management of ACHD. Identifying modifiable factors that worsen adherence offers the opportunity for targeted interventions. Depressive symptoms, anxiety, and adverse childhood experiences are amenable to psychosocial interventions, as well as cigarette smoking. Our study suggests that a multimodal and interdisciplinary treatment concept for the long-term management of adults with congenital heart disease could be beneficial. Whether it will further improve morbidity and mortality, should be assessed in prospective interventions.

Abstract 9937: Three Times Higher Risk of Stroke in Adult Congenital Heart Disease on a Median Follow-Up of Over a Decade: A Global Meta-Analysis

Background: With the advent of medicine, there is an increasing number of congenital heart disease survivors, which mandates assessment of the long-term risk of stroke and outcomes. We aimed to systematically review the pooled global prevalence of stroke in adults with congenital heart disease (ACHD). Methods: PubMed/Medline, SCOPUS and EMBASE were reviewed until May 2021 to ascertain articles describing stroke rates in ACHD compared to non-ACHD patients over a long follow-up (at least ≥3 years). Random effects models were used to estimate the pooled prevalence of stroke in ACHD and risk of stroke in ACHD vs non ACHD. Heterogeneity was assessed using the I 2 statistics with &gt;75% value suggesting substantial heterogeneity, for which subgroup analysis was performed. Results: We included 14 studies comprising 96,626 participants with ACHD and 751,460 without ACHD. The overall pooled prevalence of stroke in ACHD was 3.7% (95CI 2.5%-5.0%, I 2 = 99%) over a median follow-up period of 11.5 years ( Fig. 1a ). The ACHD patients showed nearly 3 times higher risk of stroke [OR 3.11 (95% CI 2.09-4.63), I 2 =97.99%, p&lt;0.01] on 11.5 years median follow-up ( Fig. 1b ). On subgroup analysis, studies with older ACHD patients showed nearly two times higher rate of stroke compared to studies with younger patients (mean age&gt;40 years: 4.8% vs. mean age ≤40 years, 2.6%). Furthermore, the prevalence of stroke was higher among the ACHD cohorts with shorter median follow-up (&lt;10 years) vs. studies with a median follow-up of &gt;10 years [5.0% vs 3.0%]. Studies published from Denmark (4.5%) and Canada/USA (3.2%) revealed greater rates of stroke in ACHD than the studies from Sweden (2.8%). Conclusion: This meta-analysis found nearly three times higher risk of stroke in ACHD patients over a decade follow-up compared to controls. The demographic, chronological and regional variation of the stroke prevalence in ACHD indicates that the risk could be multifactorial and warrants further risk stratification measures

Gender Differences in Physical Activity Engagement Among Adolescents With Congenital Heart Disease.

The current study aimed to (a) describe moderate-to-vigorous physical activity (MVPA), sedentary behavior (SB), and cardiorespiratory fitness (VO2Peak) via objective assessment among adolescents with congenital heart disease (CHD), (b) examine gender differences on MVPA, SB, VO2Peak, and the Theory of Planned Behavior elements, and (c) identify whether gender moderates the relationships between the Theory of Planned Behavior elements and MVPA, SB, and VO2Peak. Adolescent CHD survivors (N = 86; ages 15-18 years) wore an accelerometer to assess MVPA and SB, underwent an exercise stress test to assess VO2Peak, and completed a survey of the Theory of Planned Behavior elements as measured by perceived benefits (attitudes), family/friend support and perceived norms (social norms), and self-efficacy and barriers (perceived behavioral control) to engaging in physical activity. On average, CHD survivors engaged in 22.3 min (SD = 15.3) of MVPA/day and 9 hr of SB/day (M = 565.8, SD = 102.5 min). Females engaged in less MVPA but not more SB had a lower mean VO2Peak, reported lower self-efficacy, and perceived greater barriers than males. In a regression model, barriers explained unique variance in MVPA and VO2Peak, but the relationship between barriers and MVPA/VO2Peak did not vary by gender. Self-efficacy did not explain unique variance in MVPA and VO2Peak when included in a model with gender and barriers. Family/friend support for physical activity engagement may be an important consideration when developing physical activity interventions for adolescent CHD survivors. The role of gender differences in self-efficacy and perceived barriers on physical activity engagement warrants further investigation.