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The use of an implantable loop recorder (ILR) increased the diagnostic yield of arrhythmia in patients with congenital heart disease (CHD) and children who presented with unexplained syncope and palpitations. The objective of the study was to report the diagnostic utility and complication data of the new miniaturized ILR in patients with CHD and children. A total of 18 patients underwent successful implantation of a miniaturized ILR. Median age was 15 years (4 - 44) and median follow-up was 20 months (2 - 29). Diagnostic yield was attained in 12 patients (67%) with detection of arrhythmia in eight of those patients (67%) and exclusion in four of the 12 (33%). There were no complications. Five devices were explanted successfully. In conclusion, implantation of a miniaturized ILR in patients with CHD and children was safe and well-tolerated. It offered a good diagnostic option to diagnose or exclude cardiac arrhythmias in patients with intermittent and unexplained syncope and/or palpitations. [ABSTRACT FROM AUTHOR]

Aims: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood., Methods and Results: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2 mm, MV prolapse (MVP) as atrial displacement ≥2 mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICCs) assessed inter-rater and inter-modality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, P < 0.001) and a 0.32 mm estimate bias (95% CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95% CI 0.93, 0.98), 0.92 (95% CI 0.79, 0.97), and 0.91 (95% CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (P < 0.001). MAD was found in children of all ages, increased +0.18 mm/year (95% CI +0.14, +0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002 mm/m/year (95% CI -0.0002, +0.0005 mm/m/year)., Conclusion: MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objective: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB)., Methods: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB., Results: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal., Conclusion: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms., (© 2023 American College of Rheumatology.)

Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aims: We set out to design a reliable, semi-automated, and quantitative imaging tool using cardiac magnetic resonance (CMR) imaging that captures LV trabeculations in relation to the morphologic endocardial and epicardial surface, or perimeter-derived ratios, and assess its diagnostic and prognostic utility., Methods and Results: We queried our institutional database between January 2008 and December 2018. Non-compacted (NC)-to-compacted (C) (NC/C) myocardium ratios were calculated and our tool was used to calculate fractal dimension (FD), total mass ratio (TMR), and composite surface ratios (SRcomp). NC/C, FD, TMR, and SRcomp were assessed in relation to LVNC diagnosis and outcomes. Univariate hazard ratios with cut-offs were performed using clinically significant variables to find 'at-risk' patients and imaging parameters were compared in 'at-risk' patients missed by Petersen Index (PI). Ninety-six patients were included. The average time to complete the semi-automated measurements was 3.90 min (SEM: 0.06). TMR, SRcomp, and NC/C were negatively correlated with LV ejection fraction (LVEF) and positively correlated with indexed LV end-systolic volumes (iLVESVs), with TMR showing the strongest correlation with LVEF (-0.287; P = 0.005) and SRcomp with iLVESV (0.260; P = 0.011). We found 29 'at-risk' patients who were classified as non-LVNC by PI and hence, were missed. When compared with non-LVNC and 'low-risk' patients, only SRcomp differentiated between both groups (1.91 SEM 0.03 vs. 1.80 SEM 0.03; P = 0.019)., Conclusion: This method of semi-automatic calculation of SRcomp captured changes in at-risk patients missed by standard methods, was strongly correlated with LVEF and LV systolic volumes and may better capture outcome events., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: Instantaneous wave-free ratio (iFR) has been proven to correlate with coronary flow reserve better than fractional flow reserve (FFR) and is non-inferior to FFR in guiding coronary revascularization in ischemic heart disease. There has been no study validating the utility of iFR in children., Methods: We performed a retrospective review of clinically indicated cases in which both FFR and iFR were obtained at Texas Children's Hospital from July, 2016 to March, 2019. FFR and iFR were obtained at baseline. Adenosine FFR (FFRa) was used for assessment of coronary artery (CA) stenoses and diastolic dobutamine FFR (dFFRd) for myocardial bridges (MBs). FFRa or dFFRd ≤0.8 and iFR ≤0.89 indicated significant flow impairment., Results: A total of 22 coronary arteries (9 CA stenoses and 13 MBs) were assessed in 20 patients with median age of 13 years (range, 4-21 years) and median weight of 60 kg (range, 19-110 kg). iFR correlated with FFRa (Spearman's rho, 0.87; P<.01) in CA stenoses and with dFFRd (Spearman's rho, 0.74; P<.01) in MBs and agreed with FFR in 20/22 cases (90.9%). In 1 patient with CA stenosis and 1 MB with normal FFR, iFR was positive and both patients underwent coronary revascularization., Conclusions: iFR correlated with FFR in the assessment of CA stenoses in children. iFR does not require administration of pharmacological agents; thus, it may reduce procedural time, cost, and complications, and result in more widespread adoption of invasive assessment of CA lesions in young patients.

The recent deaths of three hardwood floor finishers in the Boston area have highlighted the urgency of addressing hazards in this industry. Among other dangers to health and safety, fire is a constant threat in a work setting that combines highly flammable solvents, large quantities of airborne wood dust, electrical equipment, heat, and friction inside old homes. Immigrant workers, who perform a large proportion of this work, are at special risk. An Environmental Justice partnership of community-based organizations, community health centers, and environmental health researchers funded by the National Institute of Environmental Health Sciences (the "Dorchester Occupational Health Initiative") had been studying the occupational health of hardwood floor finishing when these workers died. This preparation enabled community, health, labor, business, and political leaders to mobilize a response and release recommendations within weeks of the second fatal fire. Their report, adapted below, contains important information for health and labor activists in all areas where wood flooring is common. Most notably, the use of less flammable (higher flash point) products can help reduce the risk of more fatal fires. For further information, please contact the Massachusetts Coalition for Occupational Safety and Health, masscosh.org.

The deposition of extracellular beta-amyloid peptide (A beta) in the brain is a pathologic feature of Alzheimer's disease. The beta-site amyloid precursor protein cleaving enzyme 1 (BACE1), an integral membrane aspartyl protease responsible for cleavage of amyloid precursor protein (APP) at the beta-site, promotes A beta production. A second integral membrane aspartyl protease related to BACE1, referred to as beta-site amyloid precursor protein cleaving enzyme 2 (BACE2) has also been demonstrated to cleave APP at the beta-cleavage site in transfected cells. The role of endogenous BACE2 in A beta production remains unresolved. We investigated the role of endogenous BACE2 in A beta production in cells by selective inactivation of its transcripts using RNA interference. We are able to reduce steady state levels for mRNA for each enzyme by >85%, and protein amounts by 88-94% in cells. Selective inactivation of BACE1 by RNA interference results in decreased beta-cleaved secreted APP and A beta peptide secretion from cells, as expected. Selective inactivation of BACE2 by RNAi results in increased beta-cleaved secreted APP and A beta peptide secretion from cells. Simultaneous targeting of both enzymes by RNA interference does not have any net effect on A beta released from cells. Our observations of changes in APP metabolism and A beta are consistent with a role of BACE2 in suppressing A beta production in cells that co-express both enzymes.