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The efficacy of lipid-lowering therapy in reducing cardiovascular disease in adults is well-established. Unfortunately, it is also well-established that adults have inadequate adherence to lipid-lowering therapy, which is associated with increased costs and mortality. However, the adherence patterns of youth prescribed lipid-lowering therapy is not well-described. We analyzed data that was prospectively collected from patients <27 years-old who were referred to a large regional preventive cardiology clinic from 2010 to 2017. Adherence to lipid-lowering therapy was self-reported at the patient's most recent clinic visit and categorized as either adequate adherence (≥80%) or inadequate adherence (<80%). We compared adherence rates by demographic factors, class of lipid-lowering therapy, length of time on lipid-lowering therapy, family history, lipid parameters, and laboratory measures of adverse effects. In our cohort, we had 318 patients prescribed a lipid-lowering medication over a seven-year period. Of those, 235 (75%) had adequate adherence. Those with adequate adherence had an improved LDL-C (123 mg/dL [standard deviation (SD) 32.3] vs. 167 mg/dL [SD 50.4], p < 0.05), total cholesterol (198 mg/dL [49.5] vs. 239 mg/dL [SD 53.2]), and non-HDL-C (148 mg/dL [SD 38.7] vs. 193 mg/dL [SD 43.9]). In addition, patients with adequate adherence were more likely to reach goal LDL-C of <130 mg/dL than those with inadequate adherence (130 vs. 25, p < 0.01). The relationship between LDL-C and adherence remained statistically significant after controlling for age, gender, and the length of time on therapy (β = −0.66, p < 0.01). Adherence level did not differ by gender, class of lipid-lowering therapy, length of time on lipid-lowering therapy, or presence of a family history of an atherosclerotic event. The findings were similar when we only analyzed those prescribed a statin. Self-reported adherence to lipid-lowering therapy in youth is excellent and was associated with achieving goal LDL-C goals. Obtaining adherence data from patients may help more patients reach LDL-C goals. • In youth, self-reported adherence to lipid-lowering therapy, in particular statins, is high (75%). • n - Addressing low adherence may lead to more effective cardiovascular disease reduction. • Adequate adherence (i.e., >80%) was associated with reaching low-density lipoprotein cholesterol level goals. [ABSTRACT FROM AUTHOR]

Objective: To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry.Study Design: This is a cross-sectional analysis of 493 participants aged <18 years with heterozygous familial hypercholesterolemia recruited from US lipid clinics (n = 20) between April 1, 2014, and January 12, 2018. At enrollment, some were new patients and some were already in care. Clinical characteristics are described, including lipid levels and lipid-lowering treatments.Results: Mean age at diagnosis was 9.4 (4.0) years; 47% female, 68% white and 12% Hispanic. Average (SD) highest Low-density lipoprotein cholesterol (LDL-C) was 238 (61) mg/dL before treatment. Lipid-lowering therapy was used by 64% of participants; 56% were treated with statin. LDL-C declined 84 mg/dL (33%) among those treated with lipid-lowering therapy; statins produced the greatest decline, 100 mg/dL (39% reduction). At enrollment, 39% had reached an LDL-C goal, either <130 mg/dL or ≥50% decrease from pre-treatment; 20% of those on lipid-lowering therapy reached both goals.Conclusions: Among youth enrolled in the Cascade Screening for Awareness and Detection of FH Registry, diagnosis occurred relatively late, only 77% of children eligible for lipid-lowering therapy were receiving treatment, and only 39% of those treated met their LDL-C goal. Opportunities exist for earlier diagnosis, broader use of lipid-lowering therapy, and greater reduction of LDL-C levels. [ABSTRACT FROM AUTHOR]

Heart disease is the number one cause of death in women. Little is known about how adolescent and young adult women perceive their risk of heart disease. We conducted eight online, semistructured focus groups with 35 young women aged 15–24 years recruited from two primary care practices in Boston, MA. Focus group discussion topics built upon data from a larger sample of women who completed the American Heart Association Women's Health Study survey. Topics included health concerns salient to young women, perceived susceptibility to heart disease, and barriers to heart-healthy behaviors. We used qualitative coding and thematic analyses to synthesize data. Participants were surprised to learn that heart disease is the leading cause of death in women. Young women discussed age ("I feel like those are things I associate with older people like 40"), gender ("I usually hear more about men suffering from heart problems than women"), and social norms ("we're so pressured just to grow up and [be] more focused on pregnancies or depression or our weight") as reasons for their low perceived risk for heart disease. Participants noted several barriers to adopting heart-healthy behaviors including stress, lack of time, and low perceived risk. "We just don't have time to worry about hearts. Especially if our hearts aren't bothering us to begin with and we can't see it." Perceptions of age, gender, and social norms contribute to low heart disease awareness among young women, which in turn may limit heart-healthy behaviors. [ABSTRACT FROM AUTHOR]

To optimize treatment and prevent cardiovascular disease in subjects with type 1 diabetes, it is important to determine how cholesterol metabolism changes with type 1 diabetes. The objective of the study was to compare plasma levels of campesterol and β-sitosterol, markers of cholesterol absorption, as well as lathosterol, a marker of cholesterol synthesis, in youth with and without type 1 diabetes. Serum samples were obtained from adolescent subjects with type 1 diabetes (n = 175, mean age 15.2 years, mean duration of diabetes 8.2 years) and without diabetes (n = 74, mean age 15.4 years). Campesterol, β-sitosterol, and lathosterol, were measured using targeted liquid chromatography tandem mass spectrometry, compared between groups, and correlated with the available cardiometabolic variables. Campesterol and β-sitosterol levels were 30% higher in subjects with type 1 diabetes and positively correlated with hemoglobin A1c levels. In contrast, lathosterol levels were 20% lower in subjects with type 1 diabetes and positively correlated with triglycerides, body mass index, and systolic blood pressure. Plasma markers suggest that cholesterol absorption is increased, whereas cholesterol synthesis is decreased in adolescent subjects with type 1 diabetes. Further studies to address the impact of these changes on the relative efficacy of cholesterol absorption and synthesis inhibitors in subjects with type 1 diabetes are urgently needed. • Cholesterol absorption markers are increased in type 1 diabetes. • Cholesterol synthesis markers are decreased in type 1 diabetes. • Cholesterol absorption markers correlate with HbA1c in type 1 diabetes. • Cholesterol synthesis markers correlate with BMI, TG, and BP in type 1 diabetes. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease.Study Design: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 2017. Neighborhood SES was measured using a US census-based score derived from 6 measures related to income, education, and occupation. Linear and logistic regression were used to examine the association of SES with number of days of fever at time of treatment, LOS, and CAA.Results: Patients in the lowest SES quartile were treated later than patients with greater SES (7 [IQR 5, 9] vs 6 [IQR 5, 8] days, P = .01). Patients in the lowest SES quartile were more likely to be treated after 10 days of illness, with an OR 1.9 (95% CI 1.3-2.8). In multivariable analysis, SES remained an independent predictor of the number of days of fever at time of treatment (P = .01). Patients in the lowest SES quartile had longer LOS than patients with greater SES (3 [IQR 2, 5] vs 3 [IQR 2, 4], P = .007). In subgroup analysis of white children, those in the lowest SES quartile vs quartiles 2-4 were more likely to develop large/giant CAA 17 (12%) vs 30 (6%), P = .03.Conclusions: Lower SES is associated with delayed treatment, prolonged LOS, and increased risk of large/giant CAA. Novel approaches to diagnosis and education are needed for children living in low-SES neighborhoods. [ABSTRACT FROM AUTHOR]

Objective: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications.Study Design: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1.5.Results: In 9 patients (5 male), median age 14.4 years (range 7.1-22.8 years), INR testing was prescribed weekly to monthly and was done by home monitor (n = 5) or laboratory (n = 3) or combined (1). Median length of warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR >6 occurred in 3 patients (4 events); INRs ≥5 <6 in 7 patients (12 events); and INR <1.5 in 5 patients (28 events). The incidence of major bleeding events and clinically relevant nonmajor bleeding events were each 4.3 per 100 patient-years (95% CI 0.9-12.6). New asymptomatic coronary thrombosis was detected by imaging in 2 patients.Conclusions: Bleeding and clotting complications are common in patients with Kawasaki disease on warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin. [ABSTRACT FROM AUTHOR]

Background Young adults with familial hypercholesterolemia (FH) are at a critical period for establishing behaviors to promote future cardiovascular health. Objective To examine challenges transitioning to adult care for young adults with FH and parents of FH-affected young adults in the context of 2 developmental tasks, transitioning from childhood to early adulthood and assuming responsibility for self-management of a chronic disorder. Methods Semistructured, qualitative interviews were conducted with 12 young adults with FH and 12 parents of affected young adults from a pediatric subspecialty preventive cardiology program in a northeastern academic medical center. Analyses were conducted using a modified grounded theory framework. Results Respondents identified 5 challenges: (1) recognizing oneself as a decision maker, (2) navigating emerging independence, (3) prioritizing treatment for a chronic disorder with limited signs and symptoms, (4) managing social implications of FH, and (5) finding credible resources for guidance. Both young adults and parents proposed similar recommendations for addressing these challenges, including the need for family and peer involvement to establish and maintain diet and exercise routines and to provide medication reminders. Systems-level recommendations included early engagement of adolescents in shared decision-making with health care team; providing credible, educational resources regarding FH; and using blood tests to track treatment efficacy. Conclusion Young adults with FH transitioning to adult care may benefit from explicit interventions to address challenges to establishing healthy lifestyle behaviors and medication adherence as they move toward being responsible for their medical care. Further research should explore the efficacy of recommended interventions. [ABSTRACT FROM AUTHOR]

This study shows that only 12.5% of laboratory reports (2/16) included age-appropriate pediatric reference ranges for all lipid and lipoproteins. The use of erroneous reference range(s) could lead to missed alerts of dyslipidemia in up to 97.3% (total cholesterol), 93.6% (high-density lipoprotein cholesterol), 94.8% (low-density lipoprotein cholesterol), and 87.8% (triglycerides) of youth in the population-based National Health and Nutrition Examination Survey cohort. These findings highlight the potential missed opportunities for reinforcing lifestyle counseling for dyslipidemia in addition to obesity in youth. [ABSTRACT FROM AUTHOR]

Purpose Guidelines recommend cholesterol screening for all adolescents and young adults (AYAs) ages 17–21 years. Little is known about how screening results impact perceptions of AYA health. Methods We recruited 37 AYAs and 35 parents of AYAs with differing risk for abnormal cholesterol results: (1) familial hypercholesterolemia; (2) obesity; and (3) generally healthy. Participants completed quantitative health status ratings using visual analog scales (VASs) and semistructured interviews regarding three hypothetical cholesterol screening scenarios: (1) high likelihood of cardiovascular disease (CVD) before age 40 years (“high risk”); (2) some risk of CVD before age 70 years (“moderate risk”); and (3) low risk for CVD despite a strong family history of CVD (“low risk”). We analyzed VAS data with logistic regression and qualitative data with a priori and emergent coding using multiple coders. Results Each group perceived all three cholesterol screening scenarios as comparatively less than perfect health; the high-risk result fell furthest from perfect health. Although there was no significant difference between AYAs and parents in VAS ratings, qualitative analyses revealed AYAs were more likely than parents to discount the impact of moderate-risk results because of longer length of time before predicted CVD. Conclusions AYAs' and parents' perceptions of the impact of cholesterol screening results on AYA health varied by presented scenario, ranging from mild to significant decreases in perceptions of AYA health. As universal cholesterol screening continues to be adopted in this age group, further studies of the real-life impact on AYA risk perceptions and subsequent behavior are warranted. [ABSTRACT FROM AUTHOR]

Abstract: The familial hypercholesterolemias (FH) are a group of genetic defects resulting in severe elevations of blood cholesterol levels and increased risk of premature coronary heart disease. FH is among the most commonly occurring congenital metabolic disorders. FH is a treatable disease. Aggressive lipid lowering is necessary to achieve the target LDL cholesterol reduction of at least 50% or more. Even greater target LDL cholesterol reductions may be necessary for FH patients who have other CHD risk factors. Despite the prevalence of this disease and the availability of effective treatment options, FH is both underdiagnosed and undertreated, particularly among children. Deficiencies in the diagnosis and treatment of FH indicate the need for greatly increased awareness and understanding of this disease, both on the part of the public and of healthcare practitioners. This document provides recommendations for the screening, diagnosis and treatment of FH in pediatric and adult patients developed by the National Lipid Association Expert Panel on Familial Hypercholesterolemia. This report goes beyond previously published guidelines by providing specific clinical guidance for the primary care clinician and lipid specialist with the goal of improving care of patients with FH and reducing their elevated risk for CHD. [Copyright &y& Elsevier]

Objectives: To test prospectively the reproducibility and feasibility of endothelial pulse amplitude testing (Endo-PAT), a novel Food and Drug Administration-approved technology, in healthy adolescents. Study design: We performed Endo-PAT testing on 2 different days separated by no more than 7 days in 30 healthy fasting adolescents, ages 13 to 19 years, to assess reproducibility and feasibility. The reported level of discomfort, as measured on a pain scale of 1 to 5, was documented. Results: The mean difference in paired Endo-PAT indices was 0.12 (95% CI, –0.09-0.33; P = .24; intraclass correlation coefficient, 0.78), and the within-subject variation of Endo-PAT index was 0.16. The Endo-PAT index on test days 1 and 2 were 1.91 ± 0.57 and 1.78 ± 0.51 (mean plus or minus SD), respectively. All attempted studies (100%) were completed (95% CI, 88%-100%), and all completed studies (100%) could be analyzed (95% CI, 88%-100%). The median pain score was 1 on both days. Conclusion: In healthy adolescents, Endo-PAT is feasible and has excellent reproducibility. This technology may provide an easy and reliable means of assessing endothelial function in the pediatric population. [Copyright &y& Elsevier]

Abstract: Cardiovascular disease (CVD) is multifactorial in etiology. Traditional cardiovascular risk factors, such as increased cholesterol concentrations and blood pressure, are used to assess CVD risk. Recently, better understanding of the role of inflammation in atherosclerosis has prompted many to propose the measurement of various inflammatory markers to better identify those who are at increased risk. C-reactive protein (CRP) is found in endothelial atherosclerotic lesions, and evidence suggests that it may play a role in atherogenesis. Of candidate serum markers that might add information to clinical risk assessment, high-sensitivity C-reactive protein (hsCRP) measurement has the most potential for clinical use for multiple reasons: (a) high hsCRP is associated with a twofold to a threefold increase in the prevalence of myocardial infarction, stroke, and peripheral vascular disease, and it predicts incident cardiovascular events in those with and without preexisting CVD; (b) the increased risk associated with high hsCRP is independent of other established risk factors; (c) hsCRP augments the predictive capacity of the Framingham Risk Score; (d) hsCRP assays are standardized, and this analyte is biologically stable over time; (e) various risk-reducing interventions also reduce hsCRP, and research is underway to assess whether specifically targeting hsCRP reduces CVD risk. National guidelines regarding the clinical utility of hsCRP in primary and secondary prevention settings have been recently issued. [Copyright &y& Elsevier]

Poor childhood cardiovascular health translates into poor adult cardiovascular health. We hypothesized care in a preventive cardiology clinic would improve cardiovascular health after lifestyle counseling. Over a median of 3.9 months, mean cardiovascular health score (range 0-11) improved from 5.8 ± 2.2 to 6.3 ± 2.1 (P < .001) in 767 children. [ABSTRACT FROM AUTHOR]

Objectives: To determine predictors of >1 emergency department (ED) visit for a Kawasaki disease diagnosis in a quaternary care pediatric hospital and compare outcomes between patients with 1 vs >1 visit for Kawasaki disease diagnosis.Study Design: Medical records of patients evaluated for Kawasaki disease between January 2006 and August 2018 at Boston Children's Hospital were abstracted for demographic and clinical data. Predictors of >1 visit were explored using logistic regression and classification and regression tree analysis.Results: Of 530 patients diagnosed with Kawasaki disease, 117 (22%) required multiple ED visits for Kawasaki disease diagnosis. Multivariable regression and classification and regression tree analysis identified ≤2 Kawasaki disease criteria (OR 33.9; 95% CI 18.1-63.6), <3 days of fever at the first visit (OR 3.47; 95% CI 1.77-6.84), and non-White race (OR 2.15; 95% CI 1.18-3.95) as predictors of >1 visit. There were no significant differences in duration of hospitalization, day of illness at initial Kawasaki disease treatment, intravenous immunoglobulin resistance, need for adjunctive therapies, or coronary artery outcomes between patients diagnosed with Kawasaki disease at initial visit vs subsequent visits.Conclusions: Incomplete Kawasaki disease criteria, fewer days of fever, and non-White race were significant predictors of multiple ED visits for Kawasaki disease diagnosis in this single institution study. Our findings underscore the importance of maintaining a high index of suspicion for Kawasaki disease in patients with <4 Kawasaki disease criteria. Further research is needed to determine causes for increased healthcare use in non-White patients to receive a Kawasaki disease diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: To describe muscle-related statin adverse effects in real-world pediatric practice.Study Design: Using prospectively collected quality improvement data from a pediatric preventive cardiology practice, we compared serum creatine kinase (CK) levels among patients prescribed and not prescribed statins, and pre-/poststatin initiation. Multivariable mixed-effect models were constructed accounting for repeated measures, examining the effect of statins on log-transformed CK (lnCK) levels adjusted for age, sex, weight, season, insurance type, and race/ethnicity.Results: Among 1501 patients seen over 3.5 years, 474 patients (14?±?4 years, 47% female) had at least 1 serum CK measured. Median (IQR) CK levels of patients prescribed (n?=?188 patients, 768 CK measurements) and not prescribed statins (n?=?351 patients, 682 CK measurements) were 107 (83) IU/L and 113 (81) IU/L, respectively. In multivariable-adjusted models, lnCK levels did not differ based on statin use (??=?0.02 [SE 0.05], P?=?.7). Among patients started on statins (n?=?86, 130 prestatin and 292 poststatin CK measurements), median CK levels did not differ in adjusted models (? for statin use on lnCK?=?.08 [SE .07], P?=?.2). There was a clinically insignificant increase in CK over time (??=?.08 [SE .04], P?=?.04 per year). No muscle symptoms or rhabdomyolysis were reported among patients with high CK levels.Conclusions: In a real-world practice, pediatric patients using statins did not experience higher CK levels, nor was there a meaningful CK increase with statin initiation. These data suggest the limited utility to checking CK in the absence of symptoms, supporting current guidelines. [ABSTRACT FROM AUTHOR]

Objectives: To determine pediatricians' practices, attitudes, and barriers regarding screening for and treatment of pediatric dyslipidemias in 9- to 11-year-olds and 17- to 21-year-olds.Study Design: American Academy of Pediatrics (AAP) 2013-2014 Periodic Survey of a national, randomly selected sample of 1627 practicing AAP physicians. Pediatricians' responses were described and modeled.Results: Of 614 (38%) respondents who met eligibility criteria, less than half (46%) were moderately/very knowledgeable about the 2008 AAP cholesterol statement; fewer were well-informed about 2011 National Heart, Lung, and Blood Institute Guidelines or 2007 US Preventive Service Task Force review (both 26%). Despite published recommendations, universal screening was not routine: 68% reported they never/rarely/sometimes screened healthy 9- to 11-year-olds. In contrast, more providers usually/most/all of the time screened based on family cardiovascular history (61%) and obesity (82%). Screening 17- to 21-year-olds was more common in all categories (P?Conclusions: US pediatricians report lipid screening and treatment practices that are largely at odds with existing recommendations, likely because of lack of knowledge and conflicts among national guidelines, and concern about treatment efficacy and harms. Education regarding pediatric lipid disorders could promote guideline implementation. [ABSTRACT FROM AUTHOR]

Objective: To determine the real-world effectiveness of statins and impact of baseline factors on low-density lipoprotein cholesterol (LDL-C) reduction among children and adolescents.Study Design: We analyzed data prospectively collected from a quality improvement initiative in the Boston Children's Hospital Preventive Cardiology Program. We included patients ≤21 years of age initiated on statins between September 2010 and March 2014. The primary outcome was first achieving goal LDL-C, defined as <130 mg/dL, or <100 mg/dL with high-level risk factors (eg, diabetes, etc). Cox proportional hazards models were used to assess the impact of baseline clinical and lifestyle factors.Results: Among the 1521 pediatric patients evaluated in 3813 clinical encounters over 3.5 years, 97 patients (6.3%) were started on statin therapy and had follow-up data (median age 14 [IQR 7] years, 54% were female, and 24% obese, 62% with at least one lifestyle risk factor). The median baseline LDL-C was 215 (IQR 78) mg/dL, and median follow-up after starting statin was 1 (IQR 1.3) year. The cumulative probability of achieving LDL-C goal within 1 year was 60% (95% CI 47-69). A lower probability of achieving LDL-C goals was associated with male sex (HR 0.5 [95% CI 0.3-0.8]) and higher baseline LDL-C (HR 0.92 [95% CI 0.87-0.98] per 10 mg/dL), but not age, body mass index percentile, lifestyle factors, or family history.Conclusions: The majority of pediatric patients started on statins reached LDL-C treatment goals within 1 year. Male patients and those with greater baseline LDL-C were less likely to be successful and may require increased support. [ABSTRACT FROM AUTHOR]

Familial hypercholesterolemia (FH) is an autosomal dominant disorder of low-density lipoprotein (LDL) metabolism leading to high LDL cholesterol (LDL-C) and accelerated atherosclerosis. The rare homozygous form is associated with physical examination findings and coronary heart disease during childhood. The more common heterozygous form (hetFH) is asymptomatic until adulthood, when those affected develop premature cardiovascular disease (CVD) events, often in early adulthood. Identification of hetFH is key because of the relatively high prevalence, 1 in 200 to 500, and the opportunity to lower LDL-C and reduce CVD outcomes. Selective screening based on family history can identify affected individuals, but many with hetFH are missed by relying on this strategy and go undiagnosed during childhood, leading to the recommendation by the National Heart, Lung, and Blood Institute Expert Panel for universal lipid screening between ages 9 and 11 y and again at ages 17 to 21 y. Diagnosis should lead to treatment with lifestyle modification and pharmacotherapy when appropriate because lowering LDL-C in youth has beneficial effects on subclinical atherosclerosis and likely reduces premature CVD events. This article reviews what is known about the epidemiology and pathophysiology of FH as it relates to the care of children and adolescents. Approaches to identification and treatment of FH during childhood are presented, including both recommendations from published guidelines and clinical experience. A clinical case is used to illustrate various points. [ABSTRACT FROM AUTHOR]

Background Adolescents and young adults (AYAs) with familial hypercholesterolemia (FH) are at high risk for underdiagnosis and inadequate treatment. Yet, little is known about the factors that influence the medical decision making of AYAs with FH and their families. Objective This study explores how family medical history, family narratives of medical experiences, and AYA medical experiences together function as “experiential evidence” and influence screening and treatment decisions. Methods Twenty-four parents and AYAs affected by FH from a pediatric preventive cardiology practice responded to a survey and a semistructured qualitative interview. Transcribed interviews were analyzed using a modified grounded theory approach. Study design, instruments, and interpretation of results were informed by a 20-member stakeholder panel. Results AYAs and parents reported extensive personal and family experiences with cholesterol and cardiovascular conditions and treatments, sometimes distinct from FH, which were used as evidence to inform their own perceptions of FH risk and treatment. This experiential evidence impacted perceptions of: (1) hereditary risk for FH diagnoses, (2) risk for future cardiovascular disease, (3) risks associated with treatments, and (4) capacity to comply with recommended treatments. Although experiences of family members initially informed screening and treatment decisions, the subsequent personal experiences of AYAs led to new experiential evidence that informed future decisions. Conclusions Family cardiovascular history related to and distinct from FH influenced screening and treatment decisions of AYAs and parents affected by FH. Additional clinical assessment of personal and family medical experiences may enhance understanding of the decision-making processes among AYAs and ultimately improve adherence to screening and treatment recommendations. [ABSTRACT FROM AUTHOR]

Introduction: Ideal cardiovascular health is present in <50% of children and <1% of adults, yet its prevalence from adolescence through adulthood has not been fully evaluated. This study characterizes the association of age with ideal cardiovascular health and compares these associations across sex, race/ethnicity, and SES subgroups.Methods: This study, conducted in 2020, analyzed adolescents and adults aged 12-79 years from the cross-sectional National Health and Nutrition Examination Survey 2005-2016 (N=38,706). Polynomial models were used to model the association of age with ideal cardiovascular health, defined using the American Heart Association's Life's Simple 7 criteria (scales 0-14, with higher values indicating better cardiovascular health).Results: Mean cardiovascular health was lower with increasing age, starting in early adolescence and dropping to a nadir by age 60 years before stabilizing. At age 20 years, only 45% of adults had ideal cardiovascular health (≥5 ideal cardiovascular health metrics), and >50% of adults had poor cardiovascular health (≤2 ideal cardiovascular health metrics) at age 53 years. Women had higher mean cardiovascular health than men in early life but lower mean cardiovascular health from age 60 years onward. Mean cardiovascular health scores were highest for non-Hispanic White and higher-income adults and lowest for non-Hispanic Black and low-income adults across all ages. Mean cardiovascular health scores fell from intermediate to poor levels approximately 30 years earlier for non-Hispanic Black than for non-Hispanic White adults and approximately 35 years earlier for low-income adults than in higher-income adults.Conclusions: Cardiovascular health scores are lower with increasing age from early adolescence through adulthood. Race/ethnicity and income disparities in cardiovascular health are observed at young ages and are more profound at older ages. [ABSTRACT FROM AUTHOR]

Patients with coronary artery aneurysms (CAA) after Kawasaki disease (KD) are at risk of thrombosis, which can lead to myocardial infarction or sudden death. Clinical practice guidelines recommend anticoagulation for high-risk patients. Web-based worldwide survey of physicians completed between 2016 and 2017 investigating anti-thrombotic management after KD. We compared management of patients by geographic location, Human Development Index (HDI) tier, and medical specialty. The survey was completed by 603 physicians from 63 countries. In patients with normal coronaries, 95 (25%) of physicians recommended low-dose aspirin during long-term follow-up (>3 months after diagnosis). In patients with non-giant CAA, dual antiplatelet (e.g. aspirin and clopidogrel) was used by 121 (32%) of physicians, and anticoagulation by 72 (19%) of physicians. In patients with giant CAA, dual antiplatelet was used by 39 (10%) of physicians and anticoagulation by 285 (74%). In multivariable analysis, cardiology (OR 6.4 [95% CI 2.7, 16.1]) and rheumatology (OR 4.3 [95% CI 1.6, 12.6]) specialty (versus general pediatrics) were the only independent predictors of anticoagulant use in patients with giant CAA. There is significant variation in anti-thrombosis management of patients with CAA after KD, with 26% of physicians not recommending anticoagulation of patients with giant CAA. Further studies are needed to evaluate the drivers of this practice variation to inform educational initiatives and to ascertain impact on long-term outcomes. • Significant variation in antithrombosis treatment after Kawasaki disease. • Variation was associated with geographic location and clinical expertise. • Country economic development level did not predict anticoagulation practice. • Anticoagulation was recommended by 75% physicians for patients with giant coronary aneurysm. • Aspirin was well-accepted for treatment of patients with coronary artery aneurysm. [ABSTRACT FROM AUTHOR]

Background: Blood pressure (BP) and cholesterol are major modifiable risk factors for cardiovascular disease (CVD), but effects of exposures during young adulthood on later life CVD risk have not been well quantified.Objective: The authors sought to evaluate the independent associations between young adult exposures to risk factors and later life CVD risk, accounting for later life exposures.Methods: The authors pooled data from 6 U.S. cohorts with observations spanning the life course from young adulthood to later life, and imputed risk factor trajectories for low-density lipoprotein (LDL) and high-density lipoprotein cholesterols, systolic and diastolic BP starting from age 18 years for every participant. Time-weighted average exposures to each risk factor during young (age 18 to 39 years) and later adulthood (age ≥40 years) were calculated and linked to subsequent risks of coronary heart disease (CHD), heart failure (HF), or stroke.Results: A total of 36,030 participants were included. During a median follow-up of 17 years, there were 4,570 CHD, 5,119 HF, and 2,862 stroke events. When young and later adult risk factors were considered jointly in the model, young adult LDL ≥100 mg/dl (compared with <100 mg/dl) was associated with a 64% increased risk for CHD, independent of later adult exposures. Similarly, young adult SBP ≥130 mm Hg (compared with <120 mm Hg) was associated with a 37% increased risk for HF, and young adult DBP ≥80 mm Hg (compared with <80 mm Hg) was associated with a 21% increased risk.Conclusions: Cumulative young adult exposures to elevated systolic BP, diastolic BP and LDL were associated with increased CVD risks in later life, independent of later adult exposures. [ABSTRACT FROM AUTHOR]

Background: There are limited data on the prevalence and treatment of familial hypercholesterolemia (FH) among U.S. adults who experience a myocardial infarction (MI) at a young age.Objectives: This study aimed to evaluate the prevalence of clinically defined FH and examine the rates of statin utilization and low-density lipoprotein cholesterol (LDL-C) achieved 1-year post MI.Methods: The YOUNG-MI registry is a retrospective cohort study that includes patients who experience an MI at or below age 50 years between 2000 and 2016 at 2 academic centers. Probable or definite FH was defined by the Dutch Lipid Clinic criteria. Outcomes included the proportion of patients classified as probable or definite FH, use of lipid-lowering therapy, and LDL-C achieved 1-year post MI.Results: The cohort consisted of 1,996 adults with a median age of 45 years; 19% were women, and 54% had ST-segment elevation MI. Probable/definite FH was present in 180 (9%) of whom 42.8% were not on statins prior to their MI. Of the 1,966 patients surviving until hospital discharge, 89.4% of FH patients and 89.9% of non-FH patients were discharged on statin therapy (p = 0.82). Among FH patients, 63.3% were discharged on high-intensity statin compared with 48.4% for non-FH patients (p < 0.001). At 1-year follow-up, the percent reduction in LDL-C among FH patients was -44.4% compared with -34.5% (p = 0.006) in non-FH patients. The proportion of patients with LDL-C ≥70 mg/dl was higher among FH patients (82.2%) compared with non-FH patients (64.5%; p < 0.001).Conclusions: Clinically defined FH was present in nearly 1 of 10 patients with MI at a young age. Only two-thirds of FH patients were discharged on high-intensity statin therapy, and the vast majority had elevated LDL-C at 1 year. These findings reinforce the need for more aggressive lipid-lowering therapy in young FH and non-FH patients post-MI. [ABSTRACT FROM AUTHOR]

Introduction: Young adults with hyperlipidemia, hypertension, and diabetes are at increased risk of developing heart disease later in life. Despite emphasis on early screening, little is known about awareness of these risk factors in young adulthood.Methods: Data from the nationally representative cross-sectional National Health and Nutrition Examination Survey 2011-2014 were analyzed in 2017 to estimate the prevalence of self-reported awareness of hypercholesterolemia, hypertension, and diabetes in U.S. young adults aged 18-39 years (n=11,083). Prevalence estimates were weighted to population estimates using survey procedures, and predictors of awareness were identified using weighted logistic regression.Results: Among U.S. young adults, the prevalence of hypercholesterolemia, hypertension, and diabetes was 8.8% (SE=0.4%); 7.3% (SE=0.3%); and 2.6% (SE=0.2%), respectively. The prevalence of borderline high cholesterol, blood pressure, and blood glucose were substantially higher (21.6% [SE= 0.6%]; 26.9% [SE=0.7%]; and 18.9% [SE=0.6%], respectively). Awareness was low for hypercholesterolemia (56.9% [SE=2.4%]) and moderate for hypertension and diabetes (62.7% [SE=2.4%] and 70.0% [SE=2.7%]); <25% of young adults with borderline levels of these risk factors were aware of their risk. Correlates of risk factor awareness included older age, insurance status, family income above the poverty line, U.S. origin, having a usual source of health care, and the presence of comorbid conditions.Conclusions: Despite the high prevalence of cardiovascular risk factors in U.S. young adults, awareness remains less than ideal. Interventions that target access may increase awareness and facilitate achieving treatment goals in young adults. [ABSTRACT FROM AUTHOR]

Although awareness of familial hypercholesterolemia (FH) is increasing, this common, potentially fatal, treatable condition remains underdiagnosed. Despite FH being a genetic disorder, genetic testing is rarely used. The Familial Hypercholesterolemia Foundation convened an international expert panel to assess the utility of FH genetic testing. The rationale includes the following: 1) facilitation of definitive diagnosis; 2) pathogenic variants indicate higher cardiovascular risk, which indicates the potential need for more aggressive lipid lowering; 3) increase in initiation of and adherence to therapy; and 4) cascade testing of at-risk relatives. The Expert Consensus Panel recommends that FH genetic testing become the standard of care for patients with definite or probable FH, as well as for their at-risk relatives. Testing should include the genes encoding the low-density lipoprotein receptor (LDLR), apolipoprotein B (APOB), and proprotein convertase subtilisin/kexin 9 (PCSK9); other genes may also need to be considered for analysis based on patient phenotype. Expected outcomes include greater diagnoses, more effective cascade testing, initiation of therapies at earlier ages, and more accurate risk stratification. [ABSTRACT FROM AUTHOR]

Background Kawasaki disease is a complex and potentially serious condition. It has been observed in temporal relation to immunisation. Methods We conducted a systematic literature review using various reference sources to review the available evidence published in the literature. Results We identified twenty seven publications reporting a temporal association between immunisation and Kawasaki disease. We present a systematic review of data drawn from randomised controlled trials, observational studies, case series and reports, and reviews. Overall there was a lack of standardised case definitions, making data interpretation and comparability challenging. Conclusions Although a temporal relationship between immunisation and Kawasaki disease is suggested, evidence for an increased risk or a causal association is lacking. Implementation of a standardised Kawasaki disease case definition would increase confidence in the findings and add value to future studies of pre- or post-licensure vaccine safety studies. [ABSTRACT FROM AUTHOR]

Objectives: The objective of our study was to compare the indices of vascular health in Kawasaki disease (KD) patients to those of control subjects. Background: The literature on peripheral vascular health after KD is conflicting. Methods: Subjects were patients 11 to 29 years of age with the onset of KD >12 months before the study visit (n = 203) and healthy control subjects (n = 50). We measured endothelial function (using the Endothelial Pulse Amplitude Testing index), intima-media thickness (IMT) of the right common carotid artery (RCCA) and the left common carotid artery (LCCA), and fasting lipid profile and C-reactive protein (CRP). KD patients were classified according to their worst-ever coronary artery (CA) status: group I, always normal CAs (n = 136, 67%); group II, CA z-scores ≥2 but <3 (n = 20, 10%); group III, CA aneurysm z-scores ≥3 but <8 mm (n = 40, 20%); and group IV, giant CA aneurysm, defined as ≥8 mm (n = 7, 3%). Results: At a median of 11.6 years (range, 1.2 to 26 years) after KD onset, compared with controls, KD patients had a higher peak velocity in the LCCA (p = 0.04) and higher pulsatility index of both the RCCA and LCCA (p = 0.006 and p = 0.05, respectively). However, there were no differences in the Endo-PAT index or carotid IMT or stiffness. The mean IMT of the LCCA tended to differ across the KD subgroups and control group (p = 0.05), with a higher mean in group IV. Otherwise the KD subgroups and control group had similar vascular health indexes. Conclusions: In contrast to some earlier reports, our study of North American children and young adults demonstrated that KD patients whose maximum CA dimensions were either always normal or mildly ectatic have normal vascular health indexes, providing reassurance regarding peripheral vascular health in this population. [Copyright &y& Elsevier]