Ethiopia has a high acute rheumatic fever (ARF) and rheumatic heart disease (RHD) prevalence, and to our knowledge, there are no data on the status of secondary prevention in children with RHD. This ...study describes the status of secondary RHD prevention.
A multicenter, prospective study was performed on children aged 5-17 years with RHD in Ethiopia. Good adherence was defined as at least 80% completion of benzathine penicillin (BPG) or oral Amoxicillin within the previous year. The primary outcome measure was adherence to prophylaxis, expressed as a proportion. Socio-demographics, severity of RHD, and ARF recurrence were evaluated.
A total of 337 children with a mean age of 12.9 ± 2.6 years were included. The majority (73%) had severe aortic/mitral disease. Participants were on BPG (80%) or Amoxicillin (20%) prophylaxis. Female sex (P = 0.04) use of BPG (0.03) and shorter mean duration of prophylaxis in months (48.5 ± 31.5 vs. 60.7 ± 33, respectively, P < 0.008) predicted good adherence. Running out of medications (35%), interrupted follow-up (27%), and the COVID-19 pandemic (26%) were the most common reasons for missing prophylaxis. Recurrence of ARF was higher in participants on Amoxicillin compared with BPG (40% vs. 16%, P < 0.001) and in those with poor adherence compared with good adherence (36.8% vs. 17.9%, respectively, P = 0.005). Type and duration of prophylaxis (OR 0.5, CI = 0.24, 0.9, P = 0.02; OR = 1.1, CI = 1.1, 1.2, P = 0.04, respectively), and sex (OR = 1.9, CI = 1.1, 3.4, P = 0.03) were independent predictors of poor adherence.
Poor adherence is prevalent in Ethiopian children living with RHD. Amoxicillin is a suboptimal option for prophylaxis as its use is associated with lower adherence and a higher rate of ARF recurrence.
IntroductionLimited data exists describing impact of hypertrophic cardiomyopathy (HCM) on quality of life (QOL) or exercise performance. The Valsartan for Attenuating Disease in Early Sarcomeric ...Hypertrophic Cardiomyopathy (VANISH) trial provides an ideal cohort to investigate whether QOL or exercise performance is influenced by genotype + HCM. Our study aims to compare QOL and exercise in preclinical to early HCM disease.MethodsBaseline demographic, PedsQL, exercise stress test and physical activity data were compared in the VANISH cohort between those with early (G+/LVH+) or no left ventricular hypertrophy (G+/LVH-). Standard statistical analysis was performed.ResultsAnalysis included 212 subjects; 34 (16%) were G+/LVH- and were younger (16 years, IQR 12-20) than G+/LVH+ group (n=178; 20 years, IQR 15-31); p-value <0.001. G+/LVH+ had trend towards lower overall QOL, lower emotional and school functioning status (Table). G+/LVH+ had decreased daily physical activity (p=0.02) and overall lower aerobic exercise capacity (p<0.01) that worsened with age (< vs. > 18 yrs). Only 8% G+/LVH+ reported refraining from vigorous or moderate intensity physical activity.ConclusionIn children and young adults with genotype positive HCM, those with LVH+ phenotype had lower emotional and academic QOL. They had a trend towards lower perceived physical functional status and significantly lower aerobic exercise capacity, potentially driven either by their underlying disease or medical exercise restrictions. Future study is needed to determine the contributions of lower exercise capacity and to encourage physical activity in patients with HCM.
IntroductionDifferent allometric methods have resulted in variable Z scores for an echo measurement. The Pediatric Heart Network (PHN) recently established a database of echo measurements from 3215 ...healthy children. This study compared frequently used Z score models when applied to the PHN database.MethodsZ-scores were derived for the echo measurements using 4 models (PHN, Boston, Italy, Detroit). Model comparisons were performed for each measurement by evaluating 1) graphs of measurement vs body surface area (BSA) for each model with lines at Z = -2, 0, +2; 2) correlation coefficients for PHN vs other Z scores; and 3) median Z scores for each model. Because interobserver variability for echo measurements has been reported as ≥5% difference, median Z score differences between models representing <5% difference in measurement value were considered clinically insignificant.ResultsModel characteristics are listed in the Table. For most measurements, the PHN Z score lines were similar to the Boston and Italian lines but diverged from the Detroit lines at high BSAs (Figure). From the Table, correlation coefficients were high for all 3 models vs the PHN model, but they were best for the Boston and worst for the Detroit model. Compared to the PHN model, the percentage of measurements with clinically significant differences in median Z scores was lowest for the Boston and highest for the Detroit model.ConclusionsZ scores from the multicenter PHN model correlated well with single center models. Best correlation was seen with the Boston Z scores, likely related to the large sample size and similar methodology. At upper BSA quartiles, the Detroit Z scores diverged from the PHN Z scores, possibly related to more subjects with high BSAs in the PHN database. Clinically significant differences in Z scores between the PHN model and others were seen for many measurements, which is an important consideration for clinical care and when comparing publications using the different models.
The mechanisms regulating endothelial cell response to hemodynamic forces required for heart valve development, especially valve remodeling, remain elusive. Tie1, an endothelial specific receptor ...tyrosine kinase, is up-regulated by oscillating shear stress and is required for lymphatic valve development. In this study, we demonstrate that valvular endothelial Tie1 is differentially expressed in a dynamic pattern predicted by disturbed flow during valve remodeling. Following valvular endocardial specific deletion of Tie1 in mice, we observed enlarged aortic valve leaflets, decreased valve stiffness and valvular insufficiency. Valve abnormalities were only detected in late gestation and early postnatal mutant animals and worsened with age. The mutant mice developed perturbed extracellular matrix (ECM) deposition and remodeling characterized by increased glycosaminoglycan and decreased collagen content, as well as increased valve interstitial cell expression of Sox9, a transcription factor essential for normal ECM maturation during heart valve development. This study provides the first evidence that Tie1 is involved in modulation of late valve remodeling and suggests that an important Tie1-Sox9 signaling axis exists through which disturbed flows are converted by endocardial cells to paracrine Sox9 signals to modulate normal matrix remodeling of the aortic valve.
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•Valvular endothelial Tie1 is expressed in an accentuated pattern predicted by disturbed shear stress.•Tie1 deficiency leads to enlarged aortic valves and aortic insufficiency in perinatal and postnatal mice.•Valvular endothelial Tie1 deficiency results in accentuated expression of Sox9 in valve interstitial cells.
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