Measurement of Upper Extremity-Specific Comfort and Capability.

Patient-reported outcome measures (PROMs) quantify symptom intensity and magnitude of capability. Upper extremity PROMs were developed shortly after the advent of general health PROMs. PROMs are still primarily research tools, and their use with individual patients is still evolving. When PROMs were developed, the initial and intuitive expectation was a strong correlation of comfort and capability with pathophysiology severity. In other words, people with greater radiographic arthritis or larger degenerative tendon defects were expected to feel worse and do less. After more than 20 years of research using PROMs, it is clear that mindset and circumstances account for more of the variation in PROMs than pathophysiology severity. Mounting research establishes upper extremity PROMs and PROMs in general as useful tools for anchoring and developing comprehensive, biopsychosocial approaches to care.

Multicentre comparative analysis of long-term outcomes after aortic valve replacement in children.

OBJECTIVE: The ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10-15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre. Our hypothesis was that patients with pulmonic valve autograft would have better survival than mechanical prosthetic. METHODS: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional US-based registry of paediatric cardiac interventions, linked with the National Death Index and United Network for Organ Sharing through 2019. Children (0-20 years old) receiving aortic valve replacement (AVR) from 1982 to 2003 were identified. Kaplan-Meier transplant-free survival was calculated, and Cox proportional hazard models estimated hazard ratios for mechanical AVR (M-AVR) versus pulmonic valve autograft. RESULTS: Among 911 children, the median age at AVR was 13.4 years (IQR=8.4-16.5) and 73% were male. There were 10 cardiac transplants and 153 deaths, 5 after transplant. The 25-year transplant-free survival post AVR was 87.1% for autograft vs 76.2% for M-AVR and 72.0% for tissue (bioprosthetic or homograft). After adjustment, M-AVR remained related to increased mortality/transplant versus autograft (HR=1.9, 95% CI=1.1 to 3.4). Surprisingly, survival for patients with M-AVR, but not autograft, was lower for those treated in centres with higher in-hospital mortality. CONCLUSION: Pulmonic valve autograft provides the best long-term outcomes for children with aortic valve disease, but AVR results may depend on a centre's experience or patient selection.

Modifiable Clinical Correlates of Vascular Health in Children and Adolescents with Dyslipidemia.

Atherosclerosis promoting cardiovascular disease risk factors (CVDrf) are highly prevalent among youth in the U.S. Determining which standard modifiable clinical measures (SMCMs) has the greatest impact on vascular structure and function is valuable for the health care provider to help identify children at highest risk. The aim of this study was to determine modifiable outpatient clinical predictors of vascular health in youth with CVDrf. Children and adolescents with CVDrf (n = 120, 13.1 ± 1.9 years, 49% female) were recruited from a pediatric preventive cardiology clinic. The SMCMs included BMI z-score, waist-to-height ratio (WTHR), lipid panel, hemoglobin A1c, blood pressure (BP), presence of tobacco smoke exposure, and presence of hypertriglyceridemic waist (HTW) phenotype (triglycerides ≥ 110 mg/dL and waist circumference ≥ 90 percentile). Vascular function and structure were measured with pulse wave velocity (PWV), central systolic BP (CSP), augmentation index (AIx), and carotid artery intima-media thickness (cIMT). Sex and height specific z-scores for PWV, CSP, and cIMT were used. Multiple linear regression with backwards selection identified SMCMs which strongly predicted vascular function and structure. Among SMCMs, WTHR and HTW were the most frequent predictors of vascular function (PWV: R2 = 0.32; CSP: R2 = 0.35; AIx R2 = 0.13). Other predictors of vascular function included hemoglobin A1C, BP, and BMI z-score. Systolic BP and LDL cholesterol were predictors of vascular structure (cIMT: R2 = 0.14). The strongest predictors of vascular health in youth with CVDrf were related to measures of central obesity. Targeting these SMCM in lieu of vascular testing in outpatient clinic setting may be practical to identify children and adolescents at greatest risk for CVD.

Outcomes of Mechanical Mitral Valve Replacement in Children.

BACKGROUND: Mitral valve anomalies in children are rare but frequently severe, recalcitrant, and not often amenable to primary repair, necessitating mechanical mitral valve replacement (M-MVR). This study examined outcomes of a cohort undergoing a first M-MVR at age younger than 21 years. METHODS: We queried the Pediatric Cardiac Care Consortium, a multi-institutional United States-based cardiac intervention registry, for patients undergoing first M-MVR for 2-ventricle congenital heart disease. Survival and transplant status through 2014 were obtained from Pediatric Cardiac Care Consortium and linkage with the National Death Index and the Organ Procurement and Transplantation Network. RESULTS: We identified 441 patients (median age, 4.3 years; interquartile range, 1.3 to 10.1 years) meeting study criteria. The commonest disease necessitating M-MVR was atrioventricular canal (44.3%). Early mortality (death <90 days after M-MVR) was 11.1%; there was increased risk of early death if age at M-MVR was younger than 2 years (odds ratio, 7.8; 95% confidence interval [CI], 1.1 to 56.6) and with concurrent other mechanical valve placement (odds ratio, 8.5; 95% CI, 2.0 to 35.6). In those surviving more than 90 days after M-MVR, transplant-free survival was 76% at 20 years of follow-up (median follow-up, 16.6 years; interquartile range, 11.9 to 21.3 years). Adjusted analysis in those who survived more than 90 days showed elevated risk of death/transplant for boys (hazard ratio, 1.5; 95% CI, 1.0 to 2.3), age at M-MVR younger than 2 years (10-year survival: hazard ratio, 4.3; 95% CI, 1.2 to 15.1), and nonbileaflet prosthesis placement (hazard ratio, 2.4; 95% CI, 1.3 to 4.3). CONCLUSIONS: M-MVR is a viable strategy in children with unrepairable mitral valve disease. Age younger than 2 years at the first M-MVR is associated with significant early risk of death and poorer long-term survival.

Lipoprotein (a): Examination of Cardiovascular Risk in a Pediatric Referral Population.

Atherosclerotic cardiovascular disease (CVD), a leading cause of death globally, has origins in childhood. Major risk factors include family history of premature CVD, dyslipidemia, diabetes mellitus, and hypertension. Lipoprotein (a) [Lp(a)], an inherited lipoprotein, is associated with premature CVD, but its impact on cardiovascular health during childhood is less understood. The objective of the study was to examine the relationship between Lp(a), family history of premature CVD, dyslipidemia, and vascular function and structure in a high-risk pediatric population. This is a single-center, cross-sectional study of 257 children referred to a preventive cardiology clinic. The independent variable, Lp(a), separated children into high-Lp(a) [Lp(a) ≥ 30 mg/dL] and normal-Lp(a) groups [Lp(a) < 30 mg/dL]. Dependent variables included family history of premature CVD; dyslipidemia, defined as low-density lipoprotein cholesterol > 130 mg/dL, high-density lipoprotein cholesterol (HDL-C) < 45 mg/dL, triglycerides (TG) > 100 mg/dL; and vascular changes suggesting early atherosclerosis, as measured by carotid-femoral pulse wave velocity (PWV) and carotid artery intima-media thickness (CIMT). Of the 257 children, 110 (42.8%) had high Lp(a) and 147 (57.2%) had normal Lp(a). There was a higher prevalence of African-American children in the high-Lp(a) group (19.3%) compared to the normal-Lp(a) group (2.1%) (p < 0.001). High Lp(a) was associated with positive family history of premature CVD (p = 0.03), higher-than-optimal HDL-C (p = 0.02), and lower TG (p < 0.001). There was no difference in PWV or CIMT between groups. High Lp(a) in children is associated with family history of premature CVD and is prevalent in African-American children. In children with high Lp(a), promotion of intensive lifestyle modifications is prudent to decrease premature CVD-related morbidity.