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Design: The androgen receptor (AR) mediates peripheral effects of testosterone. Previous data suggests an association between the number of CAG repeats in exon‐1 of the AR gene and AR transcriptional activity. The aim of this analysis was to determine the association between the number of AR CAG repeats and all‐cause mortality in men and the influence of testosterone level on the association. Patients and Measurements: Follow‐up data to 27 January 2018 were available for men aged 40–79 years recruited across six countries of the European Male Aging Study between 2003 and 2005. Cox proportional hazards modelling was used to determine the association between CAG repeat number/mortality. Results were expressed as hazard ratios (HR)/95% confidence intervals (CI). Results: One thousand nine hundred and seventy‐seven men were followed up. Mean baseline age was 60 ± 11.1 years. Mean duration of follow‐up was 12.2 years. At follow up 25.1% of men had died. CAG repeat length ranged from 6 to 39, with the highest proportion of CAG repeat number at 21 repeats (16.4%). In a multivariable model, compared to men with 22–23 AR CAG repeats: for men with <22 and >23 AR CAG HR, 95% CI for mortality were, <22 CAG repeats 1.17 (0.93–1.49) and >23 CAG repeats 1.14 (0.88–1.47). In a post‐hoc analysis, the association was significant for men in the lowest tertile of baseline testosterone (<14.2 nmol/L) with >23 CAG repeats: in the adjusted model for <22 and >23 CAG repeats, respectively, 1.49 (0.97–2.27) and 1.68 (1.06–2.67) versus 22–23 repeats. Conclusions: Our European‐wide cohort data overall found no association of androgen receptor CAG repeat number and mortality in men. However, post hoc analysis suggested that an association might be present in men with lower baseline testosterone concentrations, which merits further investigation. [ABSTRACT FROM AUTHOR]

Outpatient cardiologists provide longitudinal care for Fontan patients. As these patients age, they face mounting morbidities, necessitating challenging conversations about prognosis and goals of care. We created a novel survey to evaluate cardiologists’ attitudes surrounding risk counseling for patients/caregivers. Cardiologists were recruited during concomitant outpatient enrollment of individuals with Fontan operation > age 10. Physician demographic data, expectations of timing in discussing adverse event risk, and perceived barriers were collected. Barriers were analyzed using a thematic approach. 40 cardiologists (9 institutions) responded regarding 155 patients (mean age 21.2 years, SD 7.7). Physicians were mostly male (58%) with mean practice of 21 years post-fellowship (SD 12). Most felt the time was right to have a conversation with patient (55%) and family (62%), and majority thought patient (53%) and family (75%) were ready for such a conversation. Most had previously discussed prognosis with patient (72%) and family (75%). Providers were inclined to discuss risk with caregivers earlier (mean patient age 9 years, SD 11) than patients (mean patient age 17 years, SD 6.4). Nevertheless, 42% of physicians perceived significant barriers and provided 58 narrative comments categorized into 4 major themes: (1) Patient-related (53.4%), including cognitive limitations and mental health; (2) Provider-related (16.4%), including lack of familiarity, preservation of happiness, and discomfort; (3) Family related (12.3%), including protection/denial and psychosocial stressors; (4) Other (26%), including social barriers. Experienced cardiologists are willing to have difficult conversations; nearly half reported largely patient-related barriers. Facilitating these conversations is critical for the adolescent/young adult with Fontan physiology. [ABSTRACT FROM AUTHOR]

Background: It is unknown how well cardiologists predict which Fontan patients are at risk for major adverse events (MAEs)., Objectives: The purpose of this study was to examine the accuracy of cardiologists' ability to identify the "good Fontan" patient, free from MAE within the following year, and compare that predicted risk cohort to patients who experienced MAE., Methods: This prospective, multicenter study included patients ≥10 years with lateral tunnel or extracardiac Fontan. The cardiologist was asked the yes/no "surprise" question: would you be surprised if your patient has a MAE in the next year? After 12 months, the cardiologist was surveyed to assess MAE. Agreement between cardiologist predictions of MAE and observed MAE was determined using the simple kappa coefficient. Multivariable generalized linear mixed effects models were performed to identify factors associated with MAE., Results: Overall, 146 patients were enrolled, and 99/146 (68%) patients w`ere predicted to be a "good Fontan." After 12 months, 17 (12%) experienced a MAE. The simple kappa coefficient of cardiologists' prediction was 0.17 (95% CI: 0.02-0.32), suggesting prediction of MAE was 17% better than random chance. In the multivariable cardiologist-predicted MAE (N = 47) model, diuretic/beta-blocker use ( P  ≤ 0.001) and systolic dysfunction ( P  = 0.005) were associated with MAE. In the observed multivariable MAE (N = 17) model, prior unplanned cardiac admission ( P  = 0.006), diuretic/beta-blocker use ( P  = 0.028), and ≥moderate atrioventricular valve regurgitation ( P  = 0.049) were associated with MAE., Conclusions: Cardiologists are marginally able to predict which Fontan patients are at risk for MAE over a year. There was overlap between factors associated with a cardiologist's prediction of risk and observed MAE, namely the use of diuretic/beta-blocker., Competing Interests: This project was supported by a grant from the New England Congenital Cardiology Research Foundation, Boston, Massachusetts, USA. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Patients with congenital heart disease (CHD) have a higher incidence of arrhythmias during pregnancy, yet the utility of mobile cardiac telemetry (MCT) to predict adverse outcomes is unknown., Objectives: The purpose of this study is to determine whether arrhythmias on screening MCT correlate with adverse pregnancy outcomes., Methods: Patients with CHD prospectively enrolled in the Standardized Outcomes in Reproductive Cardiovascular Care initiative underwent 24-hour MCT (within 18 months prior to pregnancy). Positive findings on MCT were defined as episodes of bradyarrhythmia, symptomatic atrioventricular block, ectopic atrial or ventricular activity, and supraventricular or ventricular tachycardia. Clinically significant arrhythmia events (CSAEs) were those requiring medical or device intervention or an emergency room visit. Clinical events during the antepartum, intrapartum, and postpartum periods were compared using Fisher's exact test. Analyses were performed using Stata version 16., Results: In 141 pregnancies in 118 patients with CHD, MCT detected positive findings in 17%. Adverse cardiac outcomes occurred in 11% of pregnancies, of which CSAE occurred in 3.5%. Positive MCT was significantly associated with subsequent CSAE (21% vs 0%, P  < 0.001) and cumulative adverse maternal cardiac outcomes (33% vs 7%, P  = 0.001) but did not correlate with obstetric (46% vs 41%, P  = 0.660) or neonatal outcomes (33% vs 31%, P  = 0.810). Of the patients with CSAE, 75% had ≥moderate CHD complexity., Conclusions: Patients with CHD had a high rate of positive MCT findings. This was associated with CSAE and adverse maternal cardiac outcomes. Patients with ≥moderate CHD complexity may benefit from screening MCT to improve preconceptual counseling and planning., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

Background: Atherosclerotic disease is an important cause of morbidity among adults with congenital heart disease (CHD). Prevalence of dyslipidemia in this group is poorly described., Objectives: This study aimed to describe the prevalence of dyslipidemia among adults with CHD., Methods: A prospective, outpatient screening study was conducted among adults aged ≥18 years at 4 New England ambulatory congenital cardiology centers. Participants were surveyed regarding cardiovascular risk factors. Nonfasting fingerstick samples were obtained for analysis using a point-of-care lipid analyzer., Results: Lipid screening was completed on 186 participants (median age 30 [range 18-71] years, 50% female). Eighteen (10%) had simple CHD anatomy, and 63 (34%) had complex anatomy. Only 15% of 169 respondents reported history of high cholesterol. Eighty-five (46%) participants met National Cholesterol Education Program definition of dyslipidemia with 60 (32%), 62 (34%), and 37 (20%) having low high-density lipoprotein cholesterol (HDL-C <40 mg/dL), high non-HDL-C (≥130 mg/dL), and high total cholesterol (TC ≥200 mg/dL), respectively. TC was higher among participants with simple CHD than among those with moderate and complex lesions (mean 178.4 ± 48.7 vs 170.1 ± 35.0 vs 157.6 ± 34.5 mg/dL; P  = 0.03). HDL-C was lower among participants with complex CHD than among those with simple and moderate lesions (mean 44.1 ± 13.5 vs 46.9 ± 12.5 vs 49.8 ± 15.3 mg/dL; P  = 0.05)., Conclusions: Dyslipidemia is highly prevalent among our cohort of adults with CHD, despite <15% reporting a prior diagnosis. Low HDL-C was more common in complex CHD, and high TC was more common in simple or moderate CHD. Lipid screening should be part of preventive health maintenance for all adults with CHD., Competing Interests: This study was supported in part by the Boston Adult Congenital Heart Disease Program Dunlevie Fund and by the New England Congenital Cardiology Research Foundation. Dr. Mendelson became an employee of Novartis after completion of study data collection. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.PERSPECTIVESCOMPETENCY IN MEDICAL KNOWLEDGE: Dyslipidemia was present in nearly one-half of the ACHD patients screened despite <15% of the cohort having a prior diagnosis of such. The pattern of lipid abnormalities differed, with low HDL-C being the predominant pattern among patients with complex CHD and high non–HDL-C being the predominant pattern among patients with simple and moderate CHD. TRANSLATIONAL OUTLOOK: These data strongly support the recommendation for regular screening for cardiovascular risk factors, particularly dyslipidemia, as part of routine ACHD care., (© 2022 The Authors.)

Background: Transvenous permanent pacemaker (PPM) implantation is an available option for Fontan patients with sinus node dysfunction. However, the thrombogenic potential of leads within the Fontan baffle is unknown., Objective: The purpose of this study was to compare the clot burden in Fontan patients with a transvenous atrial PPM to those without a PPM and those with an epicardial PPM., Methods: This was a retrospective cohort study of all transvenous PPM implantations in Fontan patients followed at our institution (2000-2018). We performed frequency matching on Fontan type and age group. Primary outcome was identification of intracardiac clot, pulmonary embolus, or embolic stroke., Results: Of 1920 Fontan patients, 58 patients (median age 23 years; interquartile range [25th-75th percentiles] 14-33) at the time of transvenous PPM implantation and 174 matched subjects formed our cohort. The type of Fontan performed in case subjects was right atrium-pulmonary artery or right atrium-right ventricle conduit (54%), lateral tunnel (43%), and extracardiac (3%). The cumulative incidence of clot was highest in patients with transvenous PPM, followed by patients with epicardial PPM and no PPM (1.2 vs 0.87 vs 0.67 per 100 person-years of follow-up, respectively). In multivariable analysis, anticoagulation and/or antiplatelet therapy were protective against clot and resulted in reduction of clot risk by 3-fold (incidence rate ratio 0.33; 95% confidence interval 0.21-0.53; P <.001)., Conclusion: In a large cohort of Fontan patients matched for age and Fontan type, patients with transvenous PPM had a higher but not statistically significant incidence of clot compared to those with no PPM and epicardial PPM. Patients treated with warfarin/aspirin had lower clot risk., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)