Adult Congenital Heart Disease Patients Research Articles

Abstract 4147614: Prevalence and Impact of Stroke In Young (18-44 Years) ACHD-Related Admissions by Median Household Income Quartiles: A 2019 National Inpatient Sample Analysis

Background: The risk of stroke in Adult Congenital Heart Disease (ACHD) is well established. However, the prevalence and impact of stroke in young ACHD-related admissions stratified by median household income remain underexplored. Methods: The National Inpatient Sample (2019) was utilized to identify the rate of stroke admissions among young (18-44 years) ACHD patients. We also evaluated comorbidities and outcomes (all-cause mortality [ACM], length of stay [LOS], disposition,&charges) based on the median household income quartiles and compared two cohorts of lowest (0-25th percentile) and highest (75-100thpercentile) median household income (LMHI vs HMHI). Results: Of 41950 young (18-44 years) ACHD patients, 5360 patients had stroke (12.8%). Patients in the LMHI group were relatively older (median age 36 years vs 35 years) and had a greater proportion of males (53.8% vs 46.0%) when compared to patients in the HMHI group. Patients admitted with stroke were of greater proportion in the LMHI group (13.2% vs 12.1%) compared to the HMHI group. Comorbidities like alcohol abuse (4.7% vs. 2.3%), depression (9.8% vs. 7.0%), hypertension (13.0% vs. 5.6%), obesity (21.2% vs. 18%), drug abuse (12.3% vs 4.2%), and tobacco use disorder (30.4% vs 11.2%) were higher for LMHI when compared to HMHI. Regarding in-hospital outcomes, ACM (3.7 vs. 1.9%) and discharge to home (71.6 vs. 66.5) were higher with HMHI, whereas the LMHI cohort demonstrated fewer routine discharges, higher transfers to other facilities, and prolonged LOS (5 vs. 3 days, p<0.001). When adjusted for confounders, there were statistically higher odds of AIS in ACHD patients from the LMHI group vs. the HMHI group [aOR 1.33; 1.02-1.74; p=0.045]. However, no association of income quartile with overall stroke risk was observed. Conclusion: The prevalence of stroke is significantly higher in the LMHI group. When controlled for confounders, there was a higher risk of AIS without any impact on overall stroke. Furthermore, the lowermost income quartile was associated with fewer routine dispositions, and frequent transfers can further adversely increase healthcare inequalities and healthcare costs.

Abstract 4138620: Assessment of the Accuracy of the Perioperative Mortality Risk in Adults with Congenital Heart Disease (PEACH) Score

INTRODUCTION: Previous mortality risk scores for adult cardiac surgery patients did not include adult congenital heart disease (ACHD) patients. Development of ACHD specific mortality risk scores include the procedure-specific ACHS score and recently derived PEACH score. While validated in Europe, further evaluation of the PEACH score in additional settings is necessary. RESEARCH QUESTION: We assessed the efficacy of the PEACH score to predict mortality in ACHD patients undergoing congenital cardiac surgery at our institution. We compared the PEACH score to the ACHS and STS scores and assessed for additional risk factors which may further augment the PEACH score. Methods: A retrospective cohort study was conducted of patients who underwent congenital cardiac surgery at the University of Colorado Hospital and Children’s Hospital Colorado between 2010-2022. Patients were identified by the institutional STS congenital cardiac surgery database and were eligible if 18 years or older and underwent congenital cardiac surgery. Components of the PEACH score and additional postulated risk factors were evaluated by univariate analysis. A receiver operating characteristic (ROC) curve was used to compare the PEACH score to existing risk models. Logistic regression analysis determined additional risk factors to augment the PEACH score. Results: A total of 516 patients were included with a median age of 30 (IQR 23-42) and 51% were male. There were nine deaths overall. The PEACH score was predictive of mortality (OR: 2.09, p<0.001) with a score of 0: 0 deaths/240 patients (0%); 1-2: 6 deaths/229 patients (2.6%); 3+: 3 deaths/41 patients (7.3%). Of additional risk factors tested, platelets less than 184,000 per mcL (OR: 8.36, p=0.01) was significantly associated with mortality. Comparison of the models revealed PEACH score AUC 0.832 (95% CI: 0.740,0.924), ACHS score AUC 0.869 (95% CI: 0.806, 0.932), and STAT AUC 0.769 (95% CI: 0.616, 0.922, Figure 1). The addition of platelet level improved the predictive ability of the PEACH score (AUC 0.881). Conclusion: In this single-center analysis, the PEACH score provided strong predictive ability for peri-operative mortality. The addition of platelet levels may further augment this score.

Heart failure burden among adults with congenital heart disease

Abstract Background Adult congenital heart disease (ACHD) is associated with high rates of healthcare utilization and mortality. Data on Heart failure (HF) burden among ACHD patients are limited. Purpose To study the association between HF and healthcare utilization and mortality among ACHD patients in the community. Methods The retrospective cohort study comprised 11,653≥ 18 year old ACHD patients insured by two large Israeli healthcare providers between January 2007 and December 2011. We used multivariable Cox proportional hazard and negative binomial models to study the associations of HF with mortality and healthcare utilization, respectively. Inverse treatment probability weighting accounted for drug indication. Results ACHD-HF patients (N=908; 7.8%) were older (median: 69 vs. 45 years), more likely men (53% vs. 45%), with complex congenital heart disease (14% vs. 4%), and higher comorbidity rates than ACHD patients without HF. HF was associated with higher healthcare utilization. Compared to patients without HF, ACHD-HF patients had higher adjusted relative rates (RR) of primary care (1.26, 95% confidence interval [CI]: 1.17-1.3), and cardiology clinic visits (1.9, 95% CI: 1.7-2.0), higher hospitalization rates (RR=2.0, 95% CI: 1.7-2.1), and higher risk of death (mortality hazard ratio [HR]: 1.9, 95% CI: 1.5-20.) over the study period. Among ACHD-HF patients, Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, beta-blockers, and diuretics were associated with a lower mortality risk (treated vs. untreated HR=0.56, 95% CI: 0.48-0.65; HR=0.7, 95% CI: 0.6-0.8; HR=0.84, 95% CI: 0.73-0.97; respectively). Conclusions HF presents a major burden on ACHD patients and health care systems. Drugs recommended to the general HF patient population are also associated with lower risk of hospitalization and mortality in ACHD patients. Clinical trials are needed to establish the benefit of HF pharmacotherapy in ACHD patients.Age adjusted ACHD patient survival by HF

A meta-analysis of the effects of sacubitril-valsartan on surrogate markers of systemic right ventricular dysfunction in congenital heart disease

Abstract Introduction Dysfunction of systemic right ventricle (sRV) in adult congenital heart disease (ACHD) patients is expectable and often associated with heart failure and other dismal cardiovascular events. Despite limited evidence, several neurohormonal modulators approved for heart failure with reduced ejection fraction (HFrEF) have been used off-label in patients with RV dysfunction1. Sacubitril-valsartan (ARNI) effects on sRV remains poorly studied, with current evidence being limited to small cohorts. Purpose Review and summarize current evidence about the effects of ARNI in ACHD patients with sRV. Methods We performed a systematic review on MEDLINE, EMBASE, COCHRANE, and SCOPUS for articles that included "systemic right ventricle" and "sacubitril valsartan" until January 2024. Two investigators screened the literature and extracted data. Treatment effects were measured by Cohen's d effect-size (EZ) pre and post-ARNI, with a 95% confidence interval (CI), using a restricted maximum likelihood model. Results From 197 publications screened, five studies fulfilled our criteria, comprising a total of 135 patients. Follow-up after ARNI therapy ranged from 12 to 27 months. No randomized clinical trials were found, and only two studies had a prospective design. Dextro-transposition of the great arteries (D-TGA) after atrial switch operation was the most common type of congenital heart disease with sRV (n=95; 70%). Meta-analysis demonstrates that ARNI reduced NT-proBNP levels during follow-up (EZ= -0.34, CI -0.59 to 0.08; p=0.01; I2=4.0%). Regarding RV function, ARNI improved RV fractional area change (RVFAC) (EZ= 0.75, CI 0.01 to 1.50; p= 0.048; I2=85.0%). A trend for RV global longitudinal strain improvement was also observed with ARNI (EZ= 1.74, CI -0.05 to 3.54; p=0.06; I2=95.9%) but not for tricuspid annular plane systolic excursion (EZ= -0.07, CI -0.33 to 0.20; p=0.62; I2=0.0%). RV diastolic diameter did not change significantly with ARNI (EZ= 0.07, CI -0.43 to 0.56; p=0.79; I2=68.2%). A trend for improvement on the 6-minute walk test was observed (EZ= 1.89, CI -0.13 to 3.91; p=0.07; I2=96.3%), but no effect on exercise oxygen consumption (VO2) (EZ= -0.09, CI -1.32 to 1.15; p=0.89; I2=91.4%). During follow-up ARNI did not change significantly systolic blood pressure (EZ= -0.32, CI -0.74 to 0.11; p=0.15; I2=58.4%) or serum creatinine (EZ= 0.23, CI -0.04 to 0.49; p=0.09; I2=0.0%). Conclusions Despite heterogeneity among these studies, this meta-analysis demonstrates that ARNI might improve NT-proBNP levels and RV function. ARNI seems safe and well tolerated, with no significant effect on blood pressure and renal function. More well-powered studies are needed to confirm the beneficial effects of ARNI in ACHD with sRV.

Risk factors of 30-day mortality following cardiac surgery in adults with congenital heart disease: analysis from the Japanese registry

Abstract Background With advancements in pediatric therapies, the adult congenital heart disease (ACHD) population has grown consistently. ACHD patients often face complications necessitating cardiac surgery, and understanding surgical risk factors is essential. Purpose This study aims to assess ACHD surgical risk factors using big data, addressing evidence gaps in previous research limited to univariate analysis due to few perioperative adverse events. Methods We analyzed the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination (JROAD-DPC) database from April 2013 to March 2020. The JROAD-DPC database covers nearly all teaching hospitals with cardiovascular beds participation in JROAD. Our study included ACHD patients (>15 years) undergoing open-heart surgery. Our primary outcome was 30-day mortality. We assessed clinical backgrounds, admission status, treatment, and length of hospital stay. Results Out of 13,677 ACHD patients (mean age: 53 years, females 47%) undergoing cardiac surgery assessed for 30-day mortality, 13,516 (98.8%) patients were categorized into the survival group and 161 (1.2%) into the death group. Mortality rates varied by complexity of congenital heart disease (CHD): 0.8% for simple, 1.8% for moderate, and 0.9% for great. The figure shows that a significant increase in 30-day mortality rates with age was demonstrated in simple and moderate complexity (p<0.001 for trend analysis in both categories). In contrast, for cases of great complexity, the increase in 30-day mortality rates with aging was not statistically significant (p=0.443). The death group was significantly older and had higher rates of ambulance admissions, renal disease, and peripheral vascular disease. Intra-aortic balloon pumps (IABP), veno-arterial extracorporeal membrane oxygenation (VA-ECMO), and dialysis during hospitalization were significantly higher among deceased patients. Length of ICU stay was significantly longer in the death group (median [IQR]; 3 [2-4] vs. 6 [3-11], p<0.001). Cox proportional-hazards model showed that age, moderate complexity, emergency admission, hospital bed counts, renal disease, and use of IABP and VA-ECMO were independently associated with 30-day mortality (Table). Conclusions The present study, which utilizes the nationwide administrative DPC database, demonstrates that surgical outcomes for patients with ACHD, as measured by 30-day mortality rates, are generally favorable. These outcomes significantly depend on factors such as age, complexity of CHD, emergency admissions, hospital capacity, renal disease, and use of mechanical circulatory support.

E-selectin and asymmetric dimethylarginine plasma levels in adult cyanotic congenital heart disease patients: relation to biochemical parameters, vascular function, and clinical status.

Abstract Background and Aim Patients with cyanosis secondary to congenital heart disease (CHD) are characterized by erythrocytosis and increased blood viscosity, which contributes to endothelial dysfunction, increased arterial stiffness, and impaired vascular function, which may affect the final clinical presentation. Asymmetric dimethylarginine (ADMA) and e-selectin (e-sel) are valuable biomarkers for prognosis of endocardial dysfunction and vascular damage. Their concentration levels in blood serum have the potential to be a rapid and easily accessible tool that reflects the severity of the disease. We aimed to assess e-sel and ADMA levels, their relationship with clinical status, as well as endothelial and vascular function. Methods A cross-sectional study including 36 adult CHD cyanotic patients [(17 males)(42.3 +/- 16.3 years)] with arterial blood oxygen saturation less than 92% and 20 healthy controls [(10 males)( 38.2 +/- 8.5 years)] was performed. All patients underwent clinical examination, blood testing, and cardiopulmonary tests. Endothelial function was assessed using intima-media thickness and flow-mediated dilatation (FMD). Vascular function using applanation tonometry methods was completed while considering aortic systolic pressure, aortic pulse pressure (AoPP), augmentation pressure (AP), augmentation index (AI), pulse pressure amplification (PPampl), and pulse wave velocity (PWV). Results Concentrations of e-sel and ADMA were significantly higher in patients with CHD than in controls (56.3 ± 13.6 ng/mL vs. 15.5 +/- 8.6 ng/mL, p<0.001) and (1.48 ± 0.48 ng/mL vs. 0.46 ± 0.1 ng/mL, p<0.001) respectively. E-sel levels correlated with gender, blood oxygen saturation, red blood cell, hemoglobin concentration, hematocrit, augmentation pressure, forced expiratory one second volume, forced vital capacity, and oxygen uptake. ADMA levels were found to correlate with age only. Conclusions E-sel level, unlike ADMA concentration, reflects the degree of severity of erythrocytosis and hypoxia and, thus, the physical status of patients with cyanotic CHD. E-selectin predicts increased augmentation pressure.Figure 1.A) ROC analysis for e-selectin

The relation between atrial arrhythmias and heart failure in adult patients with congenital heart disease

Abstract Background Advances in care have significantly improved survival in patients with adult congenital heart disease (ACHD). This growing population is now confronted with new challenges due to their structural malformations, surgical scars, cyanosis and/or longstanding abnormal pressure and volume load that lead to progressive atrial fibrosis. Atrial arrhythmias (AA) and heart failure (HF) have become the leading causes of hospitalisation in patients with ACHD and pose specific challenges in management. Purpose To assess the relationship between a first AA diagnosis and development of HF in patients with ACHD and investigate the impact of HF on the treatment success of radiofrequency ablation (RFA) for AA. Methods This single-centre, retrospective study included 3995 patients with ACHD. AA was defined as: (I) Atrial fibrillation (AF); (II) Intra-atrial re-entrant tachycardia (IART); (III) Atrial flutter (AFL); or (IV) Focal atrial tachycardia (AT). HF was defined as clinical signs and/or symptoms of HF necessitating medical intervention, along with one of the following: (I) impaired ventricular function with elevated intracardiac pressures; (II) elevated N-terminal B-type natriuretic peptide; (III) peak oxygen consumption in the lowest quartile as per specific ACHD subtype; or (IV) distinctive HF manifestations in patients with a Fontan circulation. Both AA and HF were collected with corresponding dates of first presentation. Details on RFA procedures were collected and acute and long-term success rates were calculated using Kaplan-Meier analysis. Results The mean age of the ACHD cohort at last follow-up was 35.4 ± 13.2 y. As per Bethesda complexity classification, 28.0% of ACHD patients had mild defects, 59.4% moderate and 12.6% severe defects. AA was documented in 348 (8.7%) patients with mean age at first presentation of 37.3 ± 19.6 y (Figure 1). Patients with Fontan circulation (38.5%), cyanotic ACHD (21.0%), and systemic right ventricle (24.2%) were more likely to present with AA. Furthermore, patients with a Fontan circulation (18.8 ± 12.4 y) or a systemic right ventricle (29.7 ± 16.1 y) presented with AA at younger age. The overall prevalence of HF was 6.4%. A total of 130 (3.3%) patients were diagnosed with AA as well as HF. Of these, 103 (79.2%) patients were diagnosed with AA 8.2 ± 7.4 y before HF diagnosis. RFA procedures were performed in 119 patients (34.2%), of which 45 (21.0%) were performed in patients with HF. Overall, 72% of patients undergoing RFA remained free from AA at 2 years after RFA (Figure 2.A). Acute success rates were higher and recurrence rates lower in those without HF (Figure 2.B, p=0.0001). Conclusions In patients with ACHD AA and HF are prevalent, especially in complex ACHD, and a first AA occurs on average 8 years prior to HF diagnosis. The main outcome is driven by HF and not AA. RFA can be an effective treatment for AA with 72% being recurrence free 2 years after RFA, but outcome is affected by coinciding HF.Overview of relation between AA and HFKaplan-meier analysis of AA RFA.

Caught in the Act: A Detailed Analysis of Cardiac Event Monitoring in a Cohort of Pediatric and ACHD Patients.

Event monitors are being increasingly used in pediatric and adult congenital heart disease (ACHD) patients for arrhythmia evaluation. Data on their diagnostic yield are limited. To evaluate the diagnostic yield of event monitors, patient characteristics associated with critical events, and clinical response to events. We retrospectively assessed event monitors prescribed to patients at our institution's Heart Center from 2017 to 2020. Thirty-day event monitor tracings were reviewed by an electrophysiologist (EP) to identify critical events defined as supraventricular tachycardia (SVT, re-entrant, atrial tachycardia, atrial flutter, and atrial fibrillation), ventricular tachycardia (VT), atrioventricular block, and pauses greater than 3 s. Patient characteristics and treatment data were collected. Characteristics associated with events were assessed using multivariable logistic regression. Trends in monitor prescription over time, diagnostic yield, and clinical response to events were analyzed. 204/2330 (8.8%) event monitors had EP-confirmed critical events. Critical events included SVT (51.5%), VT (38.5%), atrioventricular block (4%), and pauses (6%). 129/198 (65%) patients with critical events underwent treatment. Event monitoring usage increased by 52% between 2017 and 2020 (p<0.0001). Complex CHD (OR 2.1, 95% CI 1.3-3.4, p=0.004), cardiomyopathy (OR 2.9, 95% CI 1.5-4.8, p<0.001), and EP-ordered monitors (OR 1.6, 95% CI 1.2-2.1, p=0.001) were more highly associated with critical events. Event monitor use is common, and critical events were captured in 8.8% of patients. The majority of patients with critical events underwent treatment. Factors associated with critical events include EPs as ordering providers, complex CHD, and cardiomyopathy.

Outcomes in infective endocarditis among adults with CHD: a comparative national study.

Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population. We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations. Outcome variables were mortality and surgery. The primary predictor variable was the presence or absence of CHD. We identified 1,096,858 estimated infective endocarditis hospitalisations, of which 17,729 (1.6%) were adults with CHD. A 125% increase in infective endocarditis hospitalisations occurred for adult CHD patients during the studied time period (p < 0.001). Adults with CHD were significantly less likely to experience mortality (5.4% vs. 9.5%, OR 0.54, CI 0.47-0.63, p < 0.001) and more likely to undergo in-hospital surgery (31.6% vs. 6.7%, OR 6.49, CI 6.03-6.98, p < 0.001) compared to the general population. CHD severity was not associated with increased mortality (p = 0.53). Microbiologic aetiology of infective endocarditis varied between groups (p < 0.001) with Streptococcus identified more commonly in adults with CHD compared to patients with structurally normal hearts (36.2% vs. 14.4%). Adults with CHD hospitalised for infective endocarditis are less likely to experience mortality and more likely to undergo surgery than the general population.

Genetic investigation and diagnosis in adults with congenital heart disease with or without structural or neurodevelopmental comorbidity: a retrospective chart review.

Genetic evaluation is indicated for individuals with congenital heart disease (CHD), especially if extracardiac anomalies are also present. Timely recognition of genetic diagnoses can facilitate medical management and as well as provide assessment of reproductive risk. At least 20% of the pediatric population with CHD has a syndrome or genetic diagnosis. Further, at least 30% have extracardiac congenital malformations and/or neurodevelopmental differences (NDD), and this is known to increase the likelihood of a genetic/syndromic diagnosis. However, little is known regarding whether these statistics also apply to the current population of adults living with CHD, many of whom were born prior to currently available genetic testing. The primary aim of this study was to determine the prevalence of documented genetic and syndromic diagnoses in a cohort of adults with CHD followed by a dedicated adult CHD (ACHD) clinic. The secondary aims were to describe genetic testing and genetic referral patterns in this population and identify the presence of extracardiac comorbidities which are known to be indicative of an underlying genetic diagnosis in the pediatric CHD population. To answer these questions, we performed a retrospective chart review on a sample of adults with CHD (excluding those with isolated bicuspid aortic valve) seen at Cincinnati Children's Hospital in the ACHD clinic between 2010-2021. Among 233 adult CHD patients, 36 (14%) had a documented genetic or syndromic diagnosis but only 29 (13.7%) had received genetic testing, while 27 (11.6%) had received genetic referrals. Furthermore, of 170 patients without any documented genetics related care (defined as genetic testing, genetic referrals, or genetic diagnosis), 35 (20%) had at least one congenital and/or neurodevelopmental comorbidity. Factors associated with individuals having received genetics related care included younger age (<40), male sex, and presence of extracardiac comorbidities. Our results indicate important gaps in genetics-related care for adults living with CHD. The subset of our cohort with congenital and/or neurodevelopmental comorbidities who received no genetic-related care, represent a population of adults with CHD who may have unrecognized genetic diagnoses.

Outcomes in adult congenital heart disease patients with Down syndrome undergoing cardiac surgery.

BACKGROUNDAs the life expectancy of patients with Down syndrome (DS) improves, the number of older DS patients requiring cardiac surgery for congenital heart disease will increase. Perioperative risk factors and outcomes in these patients are unknown. METHODSIn a multicenter retrospective study, teenage and adult DS patients undergoing cardiac surgery between 2008-2018 were matched by age and surgical procedure with non-DS patients. Demographic, medical, and surgical characteristics were compared. Outcome measures were length of stay (LOS), duration of mechanical ventilation, need for non-invasive positive pressure ventilation (NIPPV) and reintubation, additional cardiac interventions, postoperative infections, and early postoperative mortality. Risk factors for extended hospital LOS (>10 days) were explored using multivariable logistic regression. RESULTSWe compared 121 DS patients to 121 non-DS patients. DS patients had a longer median LOS (7 vs. 5 days, p<0.001), longer duration of mechanical ventilation (12.5 vs. 6.7 hours, p<0.001), greater need for NIPPV or reintubation (26% vs. 4%, p<0.001), and higher likelihood of postoperative infections (10% vs. 2%, p=0.035). There was no early mortality. Pre-operative risk factors for extended LOS for DS patients included pulmonary medication use (OR 4.0, p=0.046), history of immunodeficiency (OR 10.4, p=0.004), or ≥moderate tricuspid regurgitation (OR 12.7, p<0.001). CONCLUSIONSTeenage and adult DS patients undergoing congenital cardiac surgery had a longer hospital LOS and more postoperative respiratory and infection complications compared to non-DS patients without increased mortality. Cardiac surgery can be performed safely in older DS patients. Management of pulmonary disease, immunodeficiency, and tricuspid regurgitation may mitigate risk.

Factors Associated with Depressive Syndrome in Adult Congenital Heart Disease Patients at RSUP Haji Adam Malik Medan

Besides being one of the causes of death in the world, adult congenital heart disease sufferers seem to be forgotten and neglected from what is called depression and anxiety. Not much has been described about depressive psychopathology in adult congenital heart disease patients. The purpose of this study was to analyze the factors associated with depressive syndrome in congenital heart disease patients. This cross-sectional study was conducted by 97 adult congenital heart disease patients recruited sequentially, consisting of 8 independent variables and 1 dependent variable. Depression scores were assessed using the BDI-II measuring instrument. Most sexes were female, with as many as 59 subjects (60.2 %). The most marital status was 66 subjects (67.3 %). The most occupations were those who worked as many as 70 subjects (71.4 %), the most previous history of surgery/intervention were those who did not have a history of prior surgery/intervention as many as 56 subjects (57.1 %), the most comorbid diseases were those who did not have a history of comorbid diseases as many as 86 subjects (87.8 %), age with a median value of 33 (18 - 45), the median value of education for adult CHD patients was 15 (12 - 18), the median NYHA grade value of adult CHD patients is 2 (1 - 4). The characteristics of patients who are highly educated, NYHA classmates, and who have jobs should be a concern because they will be more likely to experience depressive syndrome. HIGHLIGHTS Relationship between NYHA class and depression syndrome: Patients with higher NYHA grades are prone to depression. This indicates that the more severe the heart condition, the greater the level of depression. The role of education in depression: The higher the education of heart patients, the more likely they are to develop depression. Effect of work on depression: Patients without work are more prone to depression than patients who work, demonstrating the importance of job stability for inner well-being. Use of BDI-II tool: Depression measurement is carried out using BDI-II, a screening tool that effectively measures depression. GRAPHICAL ABSTRACT

Collaborative care models in adult congenital heart disease transplant.

While multidisciplinary collaboration is a tenant of quality heart failure care and critical to the success of transplant programs, this essay challenges the temptation to shoehorn adult congenital heart disease (ACHD) patients into preexisting processes and paradigms. We explore the development of more relevant models, purposefully designed to improve ACHD transplant volumes and outcomes. Globally, the rapid acceleration of ACHD patients living with and dying from HF stands in stark contrast to their access to transplant. Inferior early outcomes after ACHD transplant remain an undeniable barrier. And yet while all large registry datasets attest to this statistic, a few centers have achieved results comparable to those in acquired heart disease. This despite increases in both ACHD candidate complexity and referrals for Fontan Circulatory Failure. Perhaps something in their approach to care delivery is key?. Alone, neither ACHD nor transplant programs can provide optimal management of HF in ACHD. A siloed approach is similarly inadequate. Building new ACHD-HF-Transplant teams, centered on the patient and supplemented by ad hoc expert partnerships, is an exciting approach that can improve outcomes, create a high-quality training environment, and in our experience, is a truly rewarding way of working together.

Patient comfort with sexual orientation and gender identity questions in adult congenital cardiology clinics.

Lack of sexual orientation and gender identity (SOGI) data creates barriers for lesbian, gay, bisexual, transgender, and queer (LGBTQ+) people in health care. Barriers to SOGI data collection include physician misperception that patients do not want to answer these questions and discomfort asking SOGI questions. This study aimed to assess patient comfort towards SOGI questions across five quaternary care adult congenital heart disease (ACHD) centres. A survey administered to ACHD patients (≥18 years) asked (1) two-step gender identity and birth sex, (2) acceptance of SOGI data, and (3) the importance for ACHD physicians to know SOGI data. Chi-square tests were used to analyse differences among demographic groups and logistic regression modelled agreement with statement of patient disclosure of SOGI improving patient-physician communication. Among 322 ACHD patients, 82% identified as heterosexual and 16% identified as LGBTQ+, across the age ranges 18-29 years (39.4%), 30-49 years (47.8%), 50-64 years (8.7%), and > 65 years (4.0%). Respondents (90.4%) felt comfortable answering SOGI questions. Respondents with bachelor's/higher education were more likely to "agree" that disclosure of SOGI improves patient-physician communication compared to those with less than bachelor's education (OR = 2.45; 95% CI 1.41, 4.25; p = .0015). These findings suggest that in this largely heterosexual population, SOGI data collection is unlikely to cause patient discomfort. Respondents with higher education were twice as likely to agree that SOGI disclosure improves patient-physician communication. The inclusion of SOGI data in future studies will provide larger samples of underrepresented minorities (e.g. LGBTQ+ population), thereby reducing healthcare disparities within the field of cardiovascular research.

Pharmacological management of heart failure in adults with congenital heart disease.

Congenital heart disease (CHD) is the most common global congenital defect affecting over 2.4 million individuals in the United States. Ongoing medical and surgical advancements have improved the survival of children with CHD leading to a shift where, as of 2010, adults constitute two-thirds of the CHD patient population. The increasing number and aging of adult congenital heart disease (ACHD) patients present a clinical challenge due to heightened complexity, morbidity, and mortality. Studies indicate that 1 in 13 ACHD patients will develop heart failure (HF) in their lifetime. ACHD-HF patients experience more frequent emergency department visits, higher hospitalization rates, longer hospital stays, and higher mortality compared to non-ACHD patients with heart failure (non-ACHD-HF). Despite HF being the leading cause of death in ACHD patients, there is a notable gap inevidence regarding treatment. While guideline-directed medical therapy (GDMT) has been extensively studied in non-ACHD-HF, research specific to ACHD-HF individuals is limited. This article aims to comprehensively review available literature addressing the pharmacological treatment of ACHD-HF.

Innovations in transplant techniques for complex anomalies.

With advances in the field of congenital cardiac surgery and in the management of congenital heart defects in early life, the population of adult congenital heart disease (ACHD) patients is increasing. End-stage heart failure is currently the main cause of cardiovascular mortality and is expected to increase in the coming years. This review summarizes recent innovations in transplant techniques, with special attention to what is known in the population of ACHD recipients. The use of machine perfusion for heart preservation enables longer preservation times. Normothermic (organ care system - OCS) and hypothermic (hypothermic oxygenated perfusion - HOPE) machine perfusion will alleviate the time pressure associated with heart transplantation in the ACHD population, may allow for expansion of the geographical range in which donors can be matched and may improve graft quality. Donation after circulatory death (DCD) heart transplantation, either through direct procurement-machine perfusion (DP-MP) or thoraco-abdominal normothermic regional perfusion (TA-NRP) is a viable strategy to further expand the donor pool. The use of machine perfusion and DCD donors in ACHD is feasible and shows promise. Time pressure and shortage of donors is even more critical in ACHD than in other patient populations, making these innovations particularly relevant. Further clinical experience and research is needed to elucidate their impact.

Integrating risks and benefits: pretransplant assessment and patient selection for heart transplantation in adult congenital heart disease.

The number of adult congenital heart disease (ACHD) patients presenting for consideration of heart transplantation continues to grow. Comprehensive pretransplant assessment and thoughtful patient selection are of critical importance to mitigate perioperative and posttransplant morbidity and mortality in this population. There is increasing evidence that patient outcomes after the onset of heart failure in the ACHD population are poor while overall transplant outcomes for ACHD patients have improved over time. Delineation of factors associated with better versus worse posttransplant outcomes is an area of ongoing research. Several studies have found that delayed patient referral, anatomic complexity and the presence of noncardiac organ dysfunction may increase peri-transplant and posttransplant risk. Pretransplant assessment and patient selection in ACHD patients should focus on mitigating perioperative and early posttransplant risk. Anatomic complexity, noncardiac organ dysfunction, and referral timing after the onset of heart failure can contribute to poor posttransplant outcomes and should inform patient selection.

Haemodynamic contributors to pulmonary hypertension across the spectrum of adult congenital heart disease.

Adult congenital heart disease (ACHD) includes multiple disease states that predispose to pulmonary hypertension (PH). Haemodynamically, PH depends on abnormalities in three components: pulmonary blood flow (Qp), pulmonary vascular resistance (PVR) and pulmonary venous pressure (PVP). We sought to evaluate the prevalence and prognostic impact of individual haemodynamic abnormalities in ACHD. Retrospective study of ACHD patients undergoing cardiac catheterization at Mayo Clinic between 1999 and 2022 who were followed for the combined endpoint of death/heart transplantation. Among 1005 patients, 37% had mean pulmonary artery pressure (mPAP) ≥25 mmHg with more systemic ventricular disease, cyanotic disease and shunt lesions, highest N-terminal pro-B-type natriuretic peptide and worse right heart remodelling/dysfunction. Among those with biventricular circulation, elevated PVP, PVR and mPAP were associated with prognosis, but not increased Qp >8 L/min. However, risk of death/transplant increased for PVR only at ≥3 Wood units (hazard ratio [HR] 3.00, 95% confidence interval [CI] 2.17-4.15; p < 0.0001) and for mPAP only at ≥25 mmHg (HR 3.15, 95% CI 2.17-4.58; p < 0.0001), not at current recommended lower cutpoints. Combined abnormalities in PVP and PVR were associated with worst outcome (HR 5.20, 95% CI 3.55-7.63; p < 0.0001) with intermediate risk with either abnormality (HR 2.11, 95% CI 1.46-3.04; p < 0.0001). Findings were consistent across type of biventricular ACHD. Only with the Fontan (univentricular) circulation was a lower mPAP threshold (20 mmHg) associated with adverse outcomes. Elevation of mPAP ≥25 mmHg in ACHD with a biventricular circulation is prognostically important regardless of disease phenotype, but milder PH of 21-25 mmHg is not associated with adverse outcome unless associated with Fontan circulation. Elevation in PVP >15 mmHg and PVR ≥3 Wood units were each individually associated with mortality with combined abnormalities associated with greatest risk. Categorizing PH in ACHD by haemodynamic mechanism (PVR, PVP or Qp) allows meaningful prognostication, and may allow more unified study of targeted therapies across heterogeneous disease states in ACHD.

The impact of donor-recipient age difference on graft survival after heart transplant in adults with congenital heart disease

BackgroundThe impact of donor-recipient age difference in adult congenital heart disease (ACHD) patients undergoing heart transplant (HT) is unknown. MethodsACHD patients (≥18 years old) who underwent HT (2000-2020) were identified using the United Network for Organ Sharing database. Graft survival through 10 years based on donor-recipient age difference was evaluated by comparing outcomes of donors >5 years older than recipient (Older), donors within 5 years of recipient age (Equal Age), and donors >5 years younger than recipient (Younger, reference group). Cox multivariable analysis was performed to evaluate the effect of donor-recipient age difference on early and late graft survival. ResultsA total of 1,275 ACHD patients underwent HT (60% male, median 35 years-old (interquartile range 24-46) with median graft survival of 13.7 years (95% confidence interval [CI] 11.7-16.0). Compared to Younger donors (n=306 [24%]), graft survival was similar with Equal Age donors (n=698 [55%]; Log rank p=0.61), though significantly reduced with Older donors (n=271 [21%]; Log rank p=0.03). In multivariable analysis, late graft survival was similar with Equal Age donors but lower with Older donors (adjusted hazard ratio [aHR] 1.63, 95% CI 1.16-2.28, p=0.005), with a trend of reduced survival with Older donors in recipients <30 or ≥40 years-old. ConclusionsGraft survival in ACHD patients undergoing HT was similar for those with Younger and Equal Age donors. Recipients with Older donors had reduced 10-year survival, possibly related to increased risk in those <30 and ≥40 years-old. These data help inform optimal donor age for ACHD patients requiring HT.

Update on Transcatheter Interventions in Adults with Congenital Heart Disease.

The field of adult congenital interventions is unique in the world of cardiac catheterization, combining the structural concepts commonly employed in pediatric heart disease and applying them to adult patients, who are more amenable to device intervention as they no longer experience somatic growth. Rapid advances in the field have been made to match the growing population of adult patients with congenital heart disease, which currently surpasses the number of pediatric patients born with congenital heart disease. Many congenital defects, which once required surgical intervention or reoperation, can now be addressed via the transcatheter approach, minimizing the morbidity and mortality often encountered within adult congenital surgeries. In this paper, we aim to provide a focused review of the more common procedures that are utilized for the treatment of adult congenital heart disease patients in the catheterization laboratory, as examples of current practices in the United States, as well as emerging concepts and devices awaiting approval in the future.