Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

: Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

488 Candidacy for heart transplantation in adult congenital heart disease patients: a single-centre, retrospective, cohort study

Abstract Aims End-stage heart failure (HF) is the leading cause of death in adult congenital heart disease (ACHD) population. Heart transplantation (HTx) improves prognosis in ACHD end-stage HF but candidacy evaluation, referral pattern, and correct listing timing are not fully elucidated in this population. To evaluate factors associated to refusal from Htx in ACHD patients with end-stage HF referred for HTx evaluation. Methods and results This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients undergone HTx between 2000 and 2013. Refusal from HTx served as primary study endpoint. Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013. The main indication to HTx in patients with single ventricle physiology was Fontan failure, while in patients with systemic left ventricle and systemic right ventricle physiology, it was systemic ventricular dysfunction. We compared clinical, anatomical and demographic data of 41 patients accepted for transplantation with 15 patients refused after screening. Risk factors for refusal were: coexistence of multiple high risk features [odds ratio (OR): 3.6; 95% confidence interval (CI): 1.1–12.9; P 0.048]; anatomical factors (OR: 14.5; 95% CI: 3.1–68.4; P 0.001), out-of-centre ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). Survival in patients accepted for HTx was significantly higher than survival in patients declined from HTx with landmark comparison at 20, 40 and 60 months of 87%, 78%, and 72% vs. 70%, 59%, and 20%, respectively. HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1–8.3; P 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, coexistence of multiple conventional HTx high risk factors and out-of-centre referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy and to reduce referral delay in tertiary centre are strongly necessary for this growing population

Working memory training in old-age: an examination of transfer and maintenance effects.

The present study examined the efficacy of a verbal working memory (WM) training program in old-old individuals (over 75 years of age). Thirty-six adults aged 75\u201387 took part in the study: 18 were randomly assigned to receive training and the remainder served as active controls. Specific training gains in a verbal WM task (criterion task), and transfer effects on measures of visuospatial WM, short-term memory, inhibition, processing speed, and fluid intelligence were examined. The trained old-old adults performed better than the controls in the criterion task, and this benefit persisted after 8 months; they also showed an increase in the efficiency of inhibitory mechanisms at follow-up compared with pretest. The results of this study suggest that the present WM training program produces benefits maintained over time even in old-old adults. These findings confirm that there is still room for plasticity in the basic mechanisms of cognition in advance old age

Taurine grafting and collagen adsorption on PLLA films improve human primary chondrocyte adhesion and growth

Biocompatible and degradable poly(α-hydroxy acids) are one of the most widely used materials in scaffolds for tissue engineering. Nevertheless, they often need surface modification to improve interaction with cells. Aminolysis is a common method to increase the polymer hydrophilicity and to introduce surface functional groups, able to covalently link or absorb, through electrostatic interaction, bioactive molecules or macromolecules. For this purpose, multi-functional amines, such as diethylenediamine or hexamethylenediamine are used. However, common drawbacks are their toxicity and the introduction of positive charges on the surface. Thus, these kind of modified surfaces are unable to link directly proteins, such as collagens, a promising substrate for many cell types, in particular chondrocytes and osteoblasts. In this work, poly(L-lactide) (PLLA) film surface was labelled with negatively charged sulfonate groups by grafting taurine (TAU) through an aminolysis reaction. The novel modified PLLA film (PLLA-TAU) was able to interact directly with collagen. The reaction was carried out in mild conditions by using a solution of tetrabutylammonium salt of TAU in methanol. ATR-FTIR, XPS and contact angle measurements were used to verify the outcome of the reaction. After the exchange of tetrabutylamonium cation with Na+, collagen was absorbed on the TAU grafted PLLA film (PLLA-TAU-COLL). In vitro biological tests with human primary chondrocytes showed that PLLA-TAU and PLLA-TAU-COLL improved cell viability and adhesion, compared to the unmodified polymer, suggesting that these modifications make PLLA substrate suitable for cartilage repair

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: −0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (−0,14 ± 1.24 vs. –0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

Candidacy for heart transplantation in adult congenital heart disease patients: A cohort study

Objective The object of the present study is to evaluate factors precluding heart transplantation (HTx) in adult congenital heart disease patients (ACHD) with end-stage heart failure (HF) referred for HTx evaluation. Methods This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients receiving HTx between 2001 and 2013. HTx refusal due to poor candidacy status for excess risk of mortality after transplantation served as the main study outcome. Results Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013 (final sample size 60 patients). We compared clinical, anatomical and demographic data of 41 patients suitable for transplantation with 15 patients refused after screening (excluding 4 patients with ongoing screening). Risk factors for refusal were: multiple high risk features (odds ratio [OR]: 3.6; 95% confidence interval [CI]: 1.1 to 12.9; p 0.048); anatomical factors (OR: 14.5; 95% CI: 3.1 to 68.4; p 0.001), out-of-center ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1 to 8.3; p 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, multiple conventional HTx high risk factors and out-of-center referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy are strongly necessary for this growing population

Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy

Objectives To evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM). Methods In this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed. Results Short PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) >= 1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow chi(2) 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78. Conclusions Standard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL >= 1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis