Congenital Heart Defects In Adults Research Articles

Hubungan Aktivitas Fisik, Psikologis , Dan Sosial Dengan Kualitas Hidup Penderita Atrial Septal Defect Closure Usia 19 – 45 Tahun Di Yayasan Hipertensi Paru Indonesia Tahun 2023

Atrial Septal Defect (ASD) is a common congenital heart defect in adults, often undetected early due to lack of symptoms. Patients with ASD who have undergone closure experience physical limitations and low physical activity, also face low psychological and social conditions. Therefore, they need social and emotional support. This study examined the relationship between physical, psychological, and social activities with quality of life in patients with ASD Closure at the Indonesian Pulmonary Hypertension Foundation. The cross-sectional research method involved 30 adults (19-45 years old). The results of the study with Spearman Rank and chi square showed a significant relationship between physical activity (p value 0,00 < 0.05), psychological aspects (p value 0,00 < 0.05), and social aspects (p value 0.00 < 0.05) with quality of life. This study is expected to provide benefits for ASD closure patients in improving their quality of life

Cardiology Care and Loss to Follow-Up Among Adults With Congenital Heart Defects in CH STRONG

Many of the estimated 1.4 million adults with congenital heart defects (CHDs) in the United States are lost to follow-up (LTF) despite recommendations for ongoing cardiology care. Using 2016 to 2019 CH STRONG (Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG) data, we describe cardiac care among community-based adults with CHD, born in 1980 to 1997, identified through state birth defects registries. Our estimates of LTF were standardized to the CH STRONG eligible population and likely more generalizable to adults with CHD than clinic-based data. Half of our sample were LTF and more than 45% had not received cardiology care in over 5years. Of those who received care, only 1 in 3 saw an adult CHD physician at their last encounter. Not knowing they needed to see a cardiologist, being told they no longer needed cardiology care, and feeling "well" were the top reasons for LTF, and only half of respondents report doctors discussing the need for cardiac follow-up.

Abstract 15501: Rare Genomic Copy Number Variants Implicate New Candidate Genes for Bicuspid Aortic Valve

Introduction: Bicuspid Aortic Valve (BAV), the most common adult congenital heart defect, is a major cause of aortic insufficiency or stenosis requiring valve replacement and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications to sporadic late onset disease. We determined the frequency and gene content of rare genomic copy number variants (CNVs) in isolated BAV cases. Methods: We performed genome-wide SNP microarray analysis of familial BAV cases with early onset complications in the UTHealth BAV Research Registry (EBAV, n=394) and elderly probands from the International BAV Consortium (BAVCON, n=4216). CNVs were detected in Illumina genotypes using the PennCNV, QuantiSNP and cnvPartition algorithms. BAV cases were compared to 16,576 controls without known cardiovascular disease from the Database of Genotypes and Phenotypes. Only CNV calls with >6 consecutive variants, >20 kb in length, identified by at least two algorithms and intersected with known genes were included. For comparison, we assessed a cohort with left ventricular outflow tract obstructive lesions including BAV (LVOTO, n=1561) using identical methods. CNV burden and associations were tested using PLINK. Results: We identified 84 large recurrent CNVs in BAV cases that are absent or rare (<0.1%) in controls. 34 rare CNVs overlap between BAV and LVOTO cases and involve candidate genes that interact with each other during heart development. The largest and most prevalent of the recurrent CNVs are 8p23 duplications involving GATA4 (OR 340, CI: 42-2700). CNVs of genes that cause BAV when mutated were significantly enriched in cases (n=135, P =1x10 -27 ). The overall burden of rare genic CNVs, specifically large deletions, was also increased in BAV cases (empiric P < 1x10 -5 ) and was higher in younger EBAV cases than in older BAVCON cases. Conclusion: We identified likely pathogenic CNVs in more than 10% of BAV cases, implicating alterations of candidate genes at these loci in the pathogenesis of BAV.

Difficulties With Executive Function Are Associated With Risky Health Behaviors Among Young Adult Congenital Heart Defect Survivors.

Adults with congenital heart defects (CHDs) have an increased risk for cardiovascular complications, but few are in optimal cardiovascular health. Executive function deficits, which are elevated among CHD survivors, may contribute to engagement in behaviors that increase cardiovascular risk. The aim of this study was to identify the association between executive function and the health risk behaviors of tobacco use, saturated fat intake, and insufficient physical activity among young adults with CHDs. Young adults with CHDs completed the Behavior Rating Inventory of Executive Function-Adult Version, which yields composite and 9 individual domain scores (eg, inhibition, working memory). Participants also completed self-report measures of tobacco use, saturated fat intake, and physical activity. Linear and logistic regression models determined the association between executive function and risky health behaviors, adjusting for demographic factors, disease status, and emotional distress. One in 10 young adults with CHDs reported clinically significant difficulties with global executive function. Difficulties with global executive function were associated with increased tobacco use ( P = .018) and greater saturated fat intake ( P = .015) but not insufficient physical activity ( P = .902). Difficulties with inhibition were specifically associated with increased tobacco use ( P = .003) and greater saturated fat intake ( P = .007), and problems with self-monitoring were associated with increased tobacco use ( P = .017). Executive function difficulties are associated with self-reported engagement in health risk behaviors among young adults with CHDs. Health behavior interventions for adult CHD survivors may require additional modifications that consider executive function problems, particularly difficulties with inhibition.

Evaluation of mortality scores in patients with adult congenital heart defects

Objectives: Currently, there is no approved risk stratification for adult congenital heart surgery; accordingly, this study aimed to evaluate risk stratification for congenital heart surgery in the pediatric age in terms of its prognostic value in adult patients as well as the effectiveness of the newly developed Adult Congenital Heart Surgery (ACHS) score in this patient group. Patients and methods: A total of 205 patients (115 males, 90 females; mean age: 25.0±11.4 years; range, 18 to 65 years) operated on due to congenital heart disease between January 1, 2011, and August 1, 2019, were studied retrospectively. Aristotle Basic Complexity (ABC) score, Society of Thoracic Surgery European Association for Cardiothoracic Surgery (STAT) score, and ACHS score were evaluated. Receiver operating characteristic (ROC) curves were created to evaluate the ability of scoring systems to predict mortality. Results: The mortality rate was 4.4% (n=9). For mortality, areas under the ROC curve were 0.89, 0.89, and 0.70 for ABC, STAT, and ACHS scores, respectively. The mean ACHS score was 0.42±0.34. The cut-off point of the ACHS score was identified as 0.7 and above. The specificity of the cut-off value of 0.7 regarding the ACHS score was 94.39%. Adult Congenital Heart Surgery scores were found to be statistically high in patients with mortality (p=0.037; p<0.05). Conclusion: Adult Congenital Heart Surgery scores had higher specificity in determining mortality in cases with an ACHS score of 0.7 and above. The ACHS score could also be used to determine the expected mortality rate, similar to the ABC and STAT scores.

Energy Level and Success of Internal Defibrillation for Shockable Rhythm during Cardiopulmonary Bypass in Cardiac Surgery: A Retrospective Study

Internal defibrillation is commonly indicated for shockable rhythm following cross-clamp removal in cardiac surgery. Low energy decreases the success rate of defibrillation but high energy can cause myocardial damage. This study aimed to determine the success rate of internal defibrillation for shockable arrhythmias after cardiac surgery.Retrospective data of 1,424 patients who developed shockable rhythms (ventricular fibrillation or ventricular tachycardia), and required internal defibrillation after aortic cross-clamp removal during cardiac surgery, without deep hypothermic circulatory arrest technique, from August 2015 to July 2017, were reviewed.The overall success rate of internal defibrillation in the first attempt of defibrillation was 61.5%. The success rate of the energy levels at 30, 10, and 7 Jules were 66.7, 64.9, and 61.5%, respectively. The success rate was higher in patients who had a better ejection fraction than those who failed after defibrillation. This was significantly associated with higher pH, higher bicarbonate, lower serum calcium, and lower total cardioplegic volume during cardiopulmonary bypass (CPB). Redo-valve surgery, valvular surgery, and combined coronary artery bypass graft with valvular surgery had a non-significantly lower success rate (p-value = 0.989). Incidence of failure for defibrillate patients in redo-valvular surgery, combined coronary artery bypass graft with valve surgery, adult congenital heart defect, and valvular surgery; requiring four or five shocks was non-significantly increased. Recurrent rate of ventricular fibrillation/ventricular tachycardia was 13.5%.The success rate of internal defibrillation was not related to the dose of energy used after being weaned off CPB.

Systemic Screening for 22q11.2 Copy Number Variations in Hungarian Pediatric and Adult Patients With Congenital Heart Diseases Identified Rare Pathogenic Patterns in the Region.

Congenital heart defects (CHD) are the most common developmental abnormalities, affecting approximately 0.9% of livebirths. Genetic factors, including copy number variations (CNVs), play an important role in their development. The most common CNVs are found on chromosome 22q11.2. The genomic instability of this region, caused by the eight low copy repeats (LCR A-H), may result in several recurrent and/or rare microdeletions and duplications, including the most common, ∼3 Mb large LCR A-D deletion (classical 22q.11.2 deletion syndrome). We aimed to screen 22q11.2 CNVs in a large Hungarian pediatric and adult CHD cohort, regardless of the type of their CHDs. All the enrolled participants were cardiologically diagnosed with non-syndromic CHDs. A combination of multiplex ligation-dependent probe amplification (MLPA), chromosomal microarray analysis and droplet digital PCR methods were used to comprehensively assess the detected 22q11.2 CNVs in 212 CHD-patients. Additionally, capillary sequencing was performed to detect variants in the TBX1 gene, a cardinal gene located in 22q11.2. Pathogenic CNVs were detected in 5.2% (11/212), VUS in 0.9% and benign CNVs in 1.8% of the overall CHD cohort. In patients with tetralogy of Fallot the rate of pathogenic CNVs was 17% (5/30). Fifty-four percent of all CNVs were typical proximal deletions (LCR A-D). However, nested (LCR A-B) and central deletions (LCR C-D), proximal (LCR A-D) and distal duplications (LCR D-E, LCR D-H, LCR E-H, LCR F-H) and rare combinations of deletions and duplications were also identified. Segregation analysis detected familial occurrence in 18% (2/11) of the pathogenic variants. Based on in-depth clinical information, a detailed phenotype–genotype comparison was performed. No pathogenic variant was identified in the TBX1 gene. Our findings confirmed the previously described large phenotypic diversity in the 22q11.2 CNVs. MLPA proved to be a highly efficient genetic screening method for our CHD-cohort. Our results highlight the necessity for large-scale genetic screening of CHD-patients and the importance of early genetic diagnosis in their clinical management.

Access to cardiac surgery centers for cardiac and non-cardiac hospitalizations in adolescents and adults with congenital heart defects- a descriptive case series study

BackgroundIndividuals with congenital heart defects (CHDs) are recommended to receive all inpatient cardiac and noncardiac care at facilities that can offer specialized care. We describe geographic accessibility to such centers in New York State and determine several factors associated with receiving care there.MethodsWe used inpatient hospitalization data from the Statewide Planning and Research Cooperative System (SPARCS) in New York State 2008–2013. In the absence of specific adult CHD care center designations during our study period, we identified pediatric/adult and adult-only cardiac surgery centers through the Cardiac Surgery Reporting System to estimate age-based specialized care. We calculated one-way drive and public transit time (in minutes) from residential address to centers using R gmapsdistance package and the Google Maps Distance Application Programming Interface (API). We calculated prevalence ratios using modified Poisson regression with model-based standard errors, fit with generalized estimating equations clustered at the hospital level and subclustered at the individual level.ResultsIndividuals with CHDs were more likely to seek care at pediatric/adult or adult-only cardiac surgery centers if they had severe CHDs, private health insurance, higher severity of illness at encounter, a surgical procedure, cardiac encounter, and shorter drive time. These findings can be used to increase care receipt (especially for noncardiac care) at pediatric/adult or adult-only cardiac surgery centers, identify areas with limited access, and reduce disparities in access to specialized care among this high-risk population.

Hypertension and Heart Failure as Predictors of Mortality in an Adult Congenital Heart Defect Population

Early intervention to prevent premature mortality is vital for adults with congenital heart defects (CHD). Anatomic complexity and comorbid conditions are thought to contribute to CHD mortality. Since hypertension (HTN) and heart failure (HF) are the comorbid conditions among the most prevalent causes of death in the United States, and commonly accompany CHD, it is crucial to evaluate whether they are reliable predictors of mortality for adults with CHD (ACHD) independent of anatomic CHD complexity. A retrospective cross-sectional analysis of ACHD, aged 18–64, with concomitant HTN and/or HF and at least one health care encounter during 2008–2010 were assessed. Of 5,397 ACHD patients (18.3% HTN without HF, 4.4% HF without HTN, 8.3% with both), 3.0% died (n = 163) during the study period. Overall, the sample was 45.1% white, 61.4% female, and 29.0% had a complex CHD. Among those who died, 23.3% had HTN without HF, 17.2% had HF without HTN, and 42.3% had both. Crude analyses revealed that older age, male gender, black race, and having public health insurance were associated with increased mortality during the three-year study period compared to ACHD patients who were younger, female gender, white race, and covered by private health insurance. ACHD patients diagnosed with non-complex CHD lesions (i.e., shunts, valves, or shunts + valves) were at greater risk of dying compared to those with severe complex CHDs. When CHD type was assessed separately, those with valve lesions were more likely to die compared to those with complex CHD lesions. After adjustment for age, gender, race, insurance and CHD complexity, ACHD patients with HF, with or without HTN, were equally likely to die during the study period. However, ACHD patients with HF, without or without HTN, who had valve defects were more likely to die during the three-year study period compared to patients with complex CHDs.

Bicuspid aortic valve and sports: From the echocardiographic evaluation to the eligibility for sports competition.

Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults. Although a BAV may remain without clinical consequences for a lifetime, it can deteriorate in aortic valve stenosis and regurgitation and aortic dilatation. Unfortunately, the impact of regular training on patients with BAV and its natural course is not fully understood, although preliminary evidence suggests that the progression of valvular disease occurs primarily in an independent manner from sports practice. The current review aims to report how to perform a comprehensive echocardiographic examination in athletes with BAV and analyze the current literature on the influence of sports practice and how it impacts the aortic valve in athletes with BAV. The article also summarizes the current recommendations on sports eligibility and disqualification for competitive athletes with BAV.

Assessment of the Reversibility of Pulmonary Hypertension in Adult Congenital Heart Defects: When, how and why?

Pulmonary arterial hypertension (PAH) in the context of adult congenital heart disease (ACHD) can be reversed by early closure of the communication or pre-existing shunt. This "window of opportunity" is lost beyond a certain point of no return. Therefore, it is crucial to accurately assess the reversibility of this progressive vascular disease, which usually begins in early stages. The reversibility assessment is currently based on a combination of clinical symptoms, hemodynamic changes and fundamental variables such as pulmonary vascular resistance. However, its measurement has limited predictive value and leaves many patients in a “gray area” regarding decision-making. This review provides a concise overview of the mechanisms involved in the flow-dependent progression of PAH in CHD and assesses existing and future alternatives for a thorough assessment of existing pulmonary artery disease. The structural quantification of the pulmonary arterial tree using fractal branching algorithms, functional images with intravascular ultrasound, nuclear imaging, new serum biomarkers, genetic tests and the potential for transcriptomic analysis of circulating endothelial cells and platelets are being incorporated into the evaluation of this type of patients. Keywords: Pulmonary Hypertension-Congenital Heart Diseases-Pulmonary Vasculature-Heart Failure. Read more →

Estimating the prevalence of congenital heart disease among adolescents and adults in Colorado adjusted for incomplete case ascertainment

Congenital heart defects (CHDs), the most common type of birth defect in the United States, are increasing in prevalence in the general population. Though CHD prevalence at birth has been well described in the United States at about 1%, little is known about long-term survival and prevalence of CHDs beyond childhood. This study aimed to estimate the prevalence of CHDs among adolescents and adults in Colorado. The prevalence of CHDs among adolescents and adults residing in Colorado during 2011 to 2013 was estimated using log-linear capture-recapture methods to account for incomplete case ascertainment. Five case-finding data sources were used for this analysis including electronic health record data from 4 major health systems and a state-legislated all payer claims database. Twelve thousand two hundred ninety-three unique individuals with CHDs (2481 adolescents and 9812 adults) were identified in one or more primary data sources. We estimated the crude prevalence of CHDs in adolescents and adults in Colorado to be 3.22 per 1000 individuals (95% CI 3.19-3.53). After accounting for incomplete case ascertainment, the final capture-recapture model yielded an estimated total adolescent and adult CHD population of 23,194 (95% CI 22,419-23,565) and an adjusted prevalence of 6.07 per 1000 individuals (95% CI 5.86-6.16), indicating 47% of the cases in the catchment area were not identified in the case-identifying data sources. This statewide study yielded new information on the prevalence of CHDs in adolescents and adults. These high prevalence rates underscore the need for additional specialized care facilities for this population with CHDs.

Correction of simple congenital heart defects by right axillary thoracotomy in adults.

Right axillary thoracotomy has been performed for open-heart procedures as a more aesthetic alternative to standard median sternotomy. This study aimed to evaluate the efficacy and safety of right axillary thoracotomy for the correction of simple congenital heart defects in adults. Between January 2014 and December 2017, the clinical data of 180 adults who underwent right axillary thoracotomy for simple congenital heart defects were compared with the data of a paired group of 192 adults who underwent median sternotomy. Compared with the median sternotomy group, the right thoracotomy group showed shorter operative time, postoperative mechanical ventilation time, and postoperative hospitalization duration, as well as less drainage and transfusion volumes (P < .05). Aortic cross-clamping time, cardiopulmonary bypass time, and length of intensive care unit stay were similar between groups. No mortality or significant residual defects were reported in either group during follow-up. In total, 172 patients (95.6%) in the right thoracotomy group and 134 patients (69.8%) in the median sternotomy group were satisfied with their cosmetic results (P < .01). A right axillary thoracotomy is as safe and effective as a median sternotomy for the correction of simple congenital heart defects in adults. With lower trauma and better cosmetic results, this procedure provides a good alternative to the standard median sternotomy.

Misclassification of bicuspid aortic valves is common and varies by imaging modality and patient characteristics.

Bicuspid aortic valve (BAV) is the most prevalent adult congenital heart defect. BAV causes lifelong progressive disease that can be prevented by early diagnosis and long-term surveillance, but may be compromised by misclassification of valve morphology. The study population was derived from the UTHealth Bicuspid Aortic Valve Registry, which includes serial images on more than 200 participants over a mean follow-up interval of 2.8years. We abstracted descriptions of aortic valve morphology from transthoracic or transesophageal echocardiography, computed tomography angiography, and magnetic resonance angiography reports. We used chi-square and t tests to determine associations between reported valve morphologies (definitely bicuspid, possibly bicuspid, tricuspid, or uncertain) and clinical characteristics and assessed image quality using a validated tool. About 40% of participants were misclassified in at least one imaging report. The mean interval between misclassification and correct diagnosis was 22months. TEE, MR and CT were more sensitive than TTE and successfully reclassified 20% of participants, but were only used in 14% of patients. Misclassification was associated with age, the extent of valve calcification and image quality, but was not significantly associated with aortic regurgitation, gender, or cusp configuration. Misclassification of BAV is prevalent, frequently leads to delayed diagnosis, and is more likely to occur in the most severely affected cases. TEE, CT and MR may increase diagnostic accuracy in up to half of BAV cases but are underutilized. Additional studies are needed to determine if misclassification of BAV patients leads to increased long-term morbidity and mortality.

RECURRENT GENOMIC COPY NUMBER VARIANTS IMPLICATE NEW CANDIDATE GENES FOR EARLY ONSET BICUSPID AORTIC VALVE DISEASE

Bicuspid Aortic Valve (BAV), the most common adult congenital heart defect, is a major cause of aortic insufficiency or stenosis requiring valve replacement and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic

Estimates of adolescent and adult congenital heart defect prevalence in metropolitan Atlanta, 2010, using capture–recapture applied to administrative records

PurposeAlthough congenital heart defects (CHD) are one of the most common types of birth defects in the United States, subnational prevalence estimates beyond early childhood are limited. MethodsWe used capture–recapture methodology and logistic regression to estimate CHD prevalence per 1000 residents as of January 1, 2010, separately for adolescents and adults treated and living within five metropolitan Atlanta, Georgia counties, during 2008–2010. ResultsData sources differed by age. Adolescents (n = 1621, aged 11–20 years) and adults (n = 3176, aged 21–64 years) were captured from at least one source. We estimated CHD prevalence to be 7.85 per 1000 adolescents (estimated n = 3718 [95% CI: 3471–4004]) and 6.08 per 1000 adults (estimated n = 12,969 [95% CI: 13,873–18,915]). When we included persons found in age-inappropriate sources, prevalence estimates increased to 11 per 1000 adolescents and 6.5 per 1000 adults. ConclusionsThis method for obtaining subnational prevalence estimates provided reasonable prevalence results and identified needs for service improvement. Only one half of adolescents and one-quarter of adults with CHD were in health care within a 3-year time frame, suggesting need for better access to health insurance, transition care, and an increased number of physicians specializing in CHD care.

Detecting moderate or complex congenital heart defects in adults from an electronic health records system.

The prevalence of moderate or complex (moderate-complex) congenital heart defects (CHDs) among adults is increasing due to improved survival, but many patients experience lapses in specialty care or their CHDs are undocumented in the medical system. There is, to date, no efficient approach to identify this population. To develop and assess the performance of a risk score to identify adults aged 20-60 years with undocumented specific moderate-complex CHDs from electronic health records (EHR). We used a case-control study (596 adults with specific moderate-complex CHDs and 2384 controls). We extracted age, race/ethnicity, electrocardiogram (EKG), and blood tests from routine outpatient visits (1/2009 through 12/2012). We used multivariable logistic regression models and a split-sample (4: 1 ratio) approach to develop and internally validate the risk score, respectively. We generated receiver operating characteristic (ROC) c-statistics and Brier scores to assess the ability of models to predict the presence of specific moderate-complex CHDs. Out of six models, the non-blood biomarker model that included age, sex, and EKG parameters offered a high ROC c-statistic of 0.96 [95% confidence interval: 0.95, 0.97] and low Brier score (0.05) relative to the other models. The adult moderate-complex congenital heart defect risk score demonstrated good accuracy with 96.4% sensitivity and 80.0% specificity at a threshold score of 10. A simple risk score based on age, sex, and EKG parameters offers early proof of concept and may help accurately identify adults with specific moderate-complex CHDs from routine EHR systems who may benefit from specialty care.

Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Secundum atrial septal defect (ASD) is the most common adult congenital heart defect and can present with wide variation in clinical findings. With the intention of preventing morbidity and mortality associated with late presentation of ASD, consensus guidelines have recommended surgical or percutaneous ASD closure in adults with right heart enlargement, with or without symptoms. The aim of the present analysis was to determine if the protective effect of secundum ASD closure in adults could be qualified by pooling data from published studies. A systematic review and meta-analysis were performed by using EMBASE, MEDLINE (through PubMed), and the Cochrane Library databases to assess the effect of secundum ASD percutaneous or surgical closure in unoperated adults ≥18 years of age. Data were pooled across studies with the DerSimonian-Laird random-effects model or a Bayesian meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. Bias assessment was performed with the Newcastle-Ottawa Scale and the Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. A total of 11 nonrandomized studies met the inclusion criteria, contributing 603 patients. Pooled analysis showed a protective effect of ASD closure on New York Heart Association functional class and on right ventricular systolic pressure, volumes, and dimensions. Two additional studies comprising 652 patients were reviewed separately for mortality outcome and primary outcome of interest because they did not meet the inclusion criteria. Those studies showed that ASD closure was associated with a weak protective effect on adjusted mortality rate but no significant impact on atrial arrhythmias in patients >50 years of age. Across all studies, there was significant heterogeneity between studies for nearly all clinical outcomes. The overall body of evidence was limited to observational cohort studies, the limitations of which make for low-strength evidence. Even within the parameters of the included studies, quality of evidence was further diminished by the lack of well-defined clinical outcomes. In conclusion, pooled data analysis on the impact of secundum ASD closure in adults was notably limited because of the lack of randomized controlled trials in patients with only secundum ASD. The few cohort studies in this population demonstrated improvement in functional status and right ventricular size and function as shown by echocardiogram. However, our findings suggest that at the time of this publication, insufficient data are available to determine the impact of ASD repair on mortality rate in adults.

Abstract P256: Increased Risk of Ischemic Stroke in Adolescents and Adults with Congenital Heart Disease

Introduction: Improvements in the treatment of congenital heart defects (CHD) have resulted in the majority of infants born with CHD surviving into adulthood. This new population of adolescents and adults living with CHD have unique challenges for lifelong care, such as suffering prematurely from ischemic strokes. Hypothesis: The aim of this analysis was to examine specific cardiovascular abnormalities that increase the risk of the early development of an ischemic stroke in individuals with CHD. Methods: This study included all patients diagnosed with CHD age 18-65 that sought medical care from 2011 to 2013 at the University of Colorado Hospital, the only adult CHD clinic in Colorado. There were 3,255 individuals mean±SD age of 47±13 years diagnosed with a CHD. Marelli groups were created to categorize the severity of CHD and cardiovascular comorbidities (atrial fibrillation, hypertension, congestive heart failure, and diabetes mellitus) were extracted from the medical records. Logistic regression models were performed to evaluate the association between cardiovascular comorbidities and risk for stroke, adjusting for age, sex, race, insurance status and CHD severity. Results: Of the 3,255 adolescents and young adults with CHD (52.3% male, 47.7% female), 146 (4.49%) were diagnosed with an ischemic stroke. The greatest proportion of ischemic strokes occurred in the shunt CHD severity group (Table). In the multivariable analysis of CHD patients, atrial fibrillation (OR=2.13, 95% CI 1.45-3.12, p=0.0001) and congestive heart failure (OR=2.60, 95% CI 1.52-4.44, p=0.0005) were associated with greater than a 2-fold increase in the odds of ischemic stroke after adjusting for age, sex, race, insurance status and CHD severity. Hypertension and diabetes mellitus were not associated with the risk of stroke. Conclusion: Cardiovascular comorbidities were strongly associated with the development of an ischemic stroke in adolescents and adults with CHD independent of CHD severity.

Frequent atrial extrasystolic beats predict atrial fibrillation in patients with congenital heart defects.

Atrial fibrillation (AF) is increasingly observed in patients with congenital heart defects (CHDs) who survive nowadays into adulthood. Yet, predictors of AF are scarce in this high-risk population. This study therefore examined the predictive ability of atrial extrasystole (AES) for development of AF in CHD patients. Adult CHD patients who had a 24 h Holter registration were followed to determine who developed AF. A total of 573 patients (49% male, mean age 35 ± 12 years) were included; they had a simple/complete repaired CHD (n = 279), complex repaired CHD (n = 251), or univentricular heart (UVH, n = 43). Ageing (P < 0.0001), female gender (P = 0.028), UVH (P = 0.0010), and left atrial dilatation (P = 0.0025) were associated with the number of AES. During a median follow-up of 51.6 months (interquartile range 22.8-85.7), 29 patients (5%) developed de novo AF. An one-point increase in the number of logtotal-AES was associated with a two-fold higher risk of AF development (hazard ratio 1.95; 95% confidence interval 1.21-3.13; P = 0.016). C-statistic for left atrial dilatation, complexity, and age had a good discriminative ability for the incidence of AF with a C-statistic of 84.5%. The addition of the total number of AES/24 h to this model increased C-statistic to 88.4%. Atrial extrasystole occur relatively frequent in adult CHD patients compared with patients with other cardiac diseases. This is the first study that showed an association between an increased AES frequency and a higher risk of AF development in CHD patients.