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Background: With improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes., Methods: CHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least one intervention for CHD at one of 11 participating U.S. centers in the Pediatric Cardiac Care Consortium. Participants with CHD were asked to complete a 99-question survey on a variety of topics including: demographics, surgeries, health insurance, health care, heart doctors, general health, height and weight, education and work history, reproductive health (for women only), and COVID-19. To construct a control group for the study, siblings of survey respondents were invited to complete a similar survey. Descriptive statistics for demographics, disease severity, center, and method of survey completion were computed for participants and controls. Comparisons were made between participants and non-participants to assess for response bias and between CHD participants and sibling controls to assess for baseline differences., Results: Among the 14,322 eligible participants, there were 3133 respondents (21.9%) from 48 U.S. states with surveys returned for inclusion in the study. Sibling contact information was provided by 691 respondents, with surveys returned by 326 siblings (47.2%). The median age of participants was 32.8 years at time of survey completion, with an interquartile range of 27.2 years to 39.7 years and an overall range of 20.1 to 82.9 years. Participants were predominantly female (55.1%) and of non-Hispanic White race/ethnicity (87.1%). There were no differences between participants and non-participants regarding severity of CHD. Compared to non-participants, participants were more likely to be female, of older age, and be of non-Hispanic White race/ethnicity. Enrolled siblings were more likely to be female and slightly younger than participants., Conclusions: With surveys from 3133 participants from across the U.S., CHD PULSE is poised to provide keen insights into the lifelong journey of those living with CHD, extending beyond mere survival. These insights will offer opportunities for informing strategies to enhance and improve future outcomes for this population of patients., (Copyright © 2024. Published by Elsevier Inc.)

Background: Recently developed fundus microperimetry (MP-1; Nidek Technologies, Srl. Vigonza PD, Italy) was used to evaluate fixation patterns and retinal sensitivity in patients who had clinically significant macular edema with a diffuse pattern and type 2 diabetes mellitus., Methods: In a prospective, observational case series study, 84 consecutive patients with type 2 diabetes (84 eyes) who had clinically significant macular edema with a diffuse pattern were studied by means of MP-1. Main outcome measures were fixation stability and position and mean retinal sensitivities within the central 2 degrees and 8 degrees areas. Logarithm of the minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA) was evaluated by means of an Early Treatment of Diabetic Retinopathy Study chart. Foveal thickness was measured by Stratus optical coherence tomography (OCT; Carl Zeiss Meditec, Inc., Dublin, CA). Age, duration of diabetes, hemoglobin A1c (HbA1c) levels, duration of symptoms, history of panretinal photocoagulation, vitreomacular adhesion, and cystoid macular changes were documented. Patients were grouped according to both position of fixation (central or eccentric) and stability of fixation (stable or unstable). Statistical analyses included independent samples t-test, binomial test, Bonferroni correction for multiple comparisons, and generalized linear model univariate analysis., Results: Of 84 eyes, 44 (52.4%) had central fixation, and 40 (47.6%) had eccentric fixation. Of 84 eyes, 34 (40.5%) had stable fixation, and 50 (59.5%) had unstable fixation. In the whole sample, mean central 2 degrees retinal sensitivity +/- SD was 6.26 +/- 3.19 dB (range, 0-14.80 dB), and mean central 8 degrees retinal sensitivity +/- SD was 7.62 +/- 2.55 (range, 3.85-14.90 dB); logMAR BCVA ranged from 0.1 to 1.3 (mean +/- SD, 0.6 +/- 0.29). Mean OCT foveal thickness +/- SD was 300.77 +/- 66.93 m (range, 220-525 m). Both comparisons between groups (stable vs. unstable and central vs. eccentric) showed statistically significant differences in mean logMAR BCVA, mean central 2 degrees and 8 degrees retinal sensitivities, and mean OCT foveal thickness. Duration of symptoms was significantly longer and HbA1c levels were higher in the groups with deterioration of stability or centrality of fixation. The prevalence of cystoid macular changes was significantly higher in the groups with eccentric or unstable fixation (P < 0.001). Generalized linear model statistical analysis showed that logMAR BCVA was significantly associated with stability of fixation (P = 0.020), mean central 8 degrees retinal sensitivity (P < 0.001), and mean OCT foveal thickness (P < 0.001). The interaction between stability of fixation and mean OCT foveal thickness showed a statistically significant association with logMAR BCVA (P = 0.003). Some eyes with eccentric fixation presented with stable fixation, especially in the presence of long-lasting symptoms, and had better visual acuity than eyes with central, but unstable fixation., Conclusion: Visual acuity, central retinal sensitivity, foveal thickness, duration of symptoms, HbA1c levels, and presence of cystoid macular changes were strongly associated with fixation impairment in type 2 diabetic patients who had clinically significant macular edema with a diffuse pattern. Stability of fixation and foveal thickness play a major role in conditioning BCVA.

It is now well established that cells from the cardiac neural crest (CNC) are essential for normal conotruncal septation. The truncal septation complex consists of the aorticopulmonary (AP) septum and the myocardial sheath of the truncus. The principal role of the CNC cells during septation appears to be their differentiation into the elastogenic smooth muscle that forms the AP septum proper. The objective of this study was to integrate serial reconstruction and specific histochemical markers in order to provide a unified analysis of the relationships between the CNC and the other components of the truncal septation complex. The development of the septation complex was compared normal embryos vs. embryos from which the CNC had been surgically ablated. Embryos from each group were harvested after incubation periods of 4-8 days (Hamburger-Hamilton stages 23-34). Histochemical procedures were performed for positive identification of the elastic matrix and smooth muscle alpha-actin; the presence of these proteins was used as the criterion for "septal cells" and to define the boundaries of the septum. The results indicate that the shape, components, boundaries, and degree of organization of the septation complex may be different from previous descriptions. Furthermore, all of the components of the truncal septation complex are dysgenic in the absence of the CNC. Of special significance in the absence of CNC. Of special significance in the absence of CNC are: 1) the failure of the myocardial sheath to retract; 2) the apparently random distribution of surrogate ectomesenchyme; and 3) the impairment of truncal elastogenesis. These results indicate that the cells of neural crest origin interact with the surrounding mesenchyme during septation and that the entire septation complex depends upon the presence of the neural crest cells for normal development.