Environmental Exposures and Pediatric Cardiology: A Scientific Statement From the American Heart Association.

Environmental toxicants and pollutants are causes of adverse health consequences, including well-established associations between environmental exposures and cardiovascular diseases. Environmental degradation is widely prevalent and has a long latency period between exposure and health outcome, potentially placing a large number of individuals at risk of these health consequences. Emerging evidence suggests that environmental exposures in early life may be key risk factors for cardiovascular conditions across the life span. Children are a particularly sensitive population for the detrimental effects of environmental toxicants and pollutants given the long-term cumulative effects of early-life exposures on health outcomes, including congenital heart disease, acquired cardiac diseases, and accumulation of cardiovascular disease risk factors. This scientific statement highlights representative examples for each of these cardiovascular disease subtypes and their determinants, focusing specifically on the associations between climate change and congenital heart disease, airborne particulate matter and Kawasaki disease, blood lead levels and blood pressure, and endocrine-disrupting chemicals with cardiometabolic risk factors. Because children are particularly dependent on their caregivers to address their health concerns, this scientific statement highlights the need for clinicians, research scientists, and policymakers to focus more on the linkages of environmental exposures with cardiovascular conditions in children and adolescents.

Food Is Medicine: A Presidential Advisory From the American Heart Association.

Unhealthy diets are a major impediment to achieving a healthier population in the United States. Although there is a relatively clear sense of what constitutes a healthy diet, most of the US population does not eat healthy food at rates consistent with the recommended clinical guidelines. An abundance of barriers, including food and nutrition insecurity, how food is marketed and advertised, access to and affordability of healthy foods, and behavioral challenges such as a focus on immediate versus delayed gratification, stand in the way of healthier dietary patterns for many Americans. Food Is Medicine may be defined as the provision of healthy food resources to prevent, manage, or treat specific clinical conditions in coordination with the health care sector. Although the field has promise, relatively few studies have been conducted with designs that provide strong evidence of associations between Food Is Medicine interventions and health outcomes or health costs. Much work needs to be done to create a stronger body of evidence that convincingly demonstrates the effectiveness and cost-effectiveness of different types of Food Is Medicine interventions. An estimated 90% of the $4.3 trillion annual cost of health care in the United States is spent on medical care for chronic disease. For many of these diseases, diet is a major risk factor, so even modest improvements in diet could have a significant impact. This presidential advisory offers an overview of the state of the field of Food Is Medicine and a road map for a new research initiative that strategically approaches the outstanding questions in the field while prioritizing a human-centered design approach to achieve high rates of patient engagement and sustained behavior change. This will ideally happen in the context of broader efforts to use a health equity-centered approach to enhance the ways in which our food system and related policies support improvements in health.

Cumulative insulin resistance and hyperglycemia with arterial stiffness and carotid IMT progression in 1,779 adolescents: a 9-yr longitudinal cohort study.

In pediatric population with diabetes and obesity, insulin resistance (HOMA-IR) has been associated with worsening vascular outcomes, however, the cumulative role of HOMA-IR, hyperglycemia, and hyperinsulinemia on repeatedly measured vascular outcomes in asymptomatic youth is unknown. We examined the longitudinal associations of fasting glucose, insulin, and HOMA-IR with carotid-femoral pulse wave velocity (cfPWV) and carotid intima-media thickness (cIMT). From the Avon Longitudinal Study of Parents and Children (ALSPAC) birth cohort, UK 1,779, 15-yr-old participants were followed up for 9 yr. Glucose, insulin, and HOMA-IR assessed at 15, 17, and 24 yr and sex-specifically dichotomized as ≥75th percentile, indicating high category and <75th percentile as reference. cfPWV and cIMT were measured at ages 17 and 24 yr. Associations were examined using linear mixed-effect models adjusted for cardiometabolic and lifestyle covariates. Among 1,779 participants [49.9% female], glucose, insulin, and HOMA-IR had a J- or U-shaped increase from ages 15 through 24 yr. The cumulative exposures to hyperinsulinemia effect estimate -0.019 mU/L; [95% CI -0.019 to -0.002; P = 0.033] and high HOMA-IR: -0.021; [-0.039 to -0.004; P = 0.019] from 15 to 24 yr of age were negatively associated with the 7-yr cfPWV progression. Only cumulative hyperinsulinemia and high HOMA-IR from ages 15 to 17 yr but not from ages 17 to 24 yr was associated with decreased cfPWV progression. There were no associations between cumulative hyperglycemia and cfPWV or cIMT progression. Hyperinsulinemia and HOMA-IR were not associated with cIMT progression. In conclusion, late adolescence may be an optimal timing for intervention targeted at sustaining the protective effect of the decline of insulin and insulin resistance on arterial stiffness progression.NEW & NOTEWORTHY Fasting plasma glucose, insulin, and insulin resistance had a J- or U-shaped increase from 15 to 24 yr with the base of the curve at age 17 yr. Cumulative high insulin and high insulin resistance from 15 to 24 yr were negatively associated with arterial stiffness progression from ages 17 to 24 yr. Age 17 yr may be an optimal timing for intervention targeted at sustaining the protective effect of the decline of insulin and insulin resistance on arterial stiffness progression.

Double-Trouble: Atherosclerotic Risk Factors and Congenital Heart Disease.

PURPOSE OF REVIEW: Youth with congenital heart disease (CHD) are uniquely vulnerable to genetic and acquired atherosclerotic cardiovascular disease (ASCVD) risk factors. With the increasingly successful management of CHD, it is important to prevent or optimally managed risk factors with the goal of improving outcomes and longevity. RECENT FINDINGS: This review summarizes guidelines for the evaluation and management of obesity, dyslipidemia, and hypertension in youth (< 18 years of age), focusing on the special vulnerabilities associated with the type of repair and the presence of residual disease in those who undergo cardiac surgery. Clinicians must focus on targeting these highly prevalent ASCVD risk factors to protect CHD survivors from preventable ASCVD morbidity and mortality by applying lifestyle, pharmacologic, or surgical therapies as needed. Future work should examine interventions to identify and treat ASCVD risk factors in CHD patients. Given the increased prevalence of ASCVD risk factors in youth and the morbidity and premature mortality associated with CHD, it is important for clinicians to assess global risk factors in these patients frequently, encourage adherence to lifestyle changes, and recommend pharmacotherapy and surgical interventions when clinically indicated. Future efforts should identify barriers and opportunities for improving risk factor assessment and timely intervention as a routine part of clinical care.

Lipid temporal trends in normal-weight youth.

Atherosclerosis begins in youth, partly driven by excess weight (EW) and abnormal lipids. Despite pediatric obesity worsening, lipids improved. Given the relation between EW and abnormal lipids, changes in normal-weight (NW) youth may be relevant. We examined the proportions and temporal trends of youth with abnormal lipids who were NW versus EW. METHODS: Analysis was done from National Health and Nutrition Examination Surveys 1988-2016. Data were extracted for 10- to 20-year-olds measured with anthropometrics and laboratory testing to determine proportions of NW versus EW with total cholesterol >190 mg/dL, high-density lipoprotein cholesterol (HDL-C) <40 mg/dL, and calculated non-HDL-C >145 mg/dL (N = 14,785). In survey-weighted regression analysis, a weight-status interaction term was used to examine effect modification in the lipid temporal trend. RESULTS: Over time, EW prevalence increased, whereas dyslipidemia decreased (trend P value < .001 for both). For the pooled sample, EW more than doubled the risk of each lipid disorder (P < .0001 for each). However, for each abnormal lipid, 26%-63% were NW. As the temporal trend in abnormal lipids declined, the proportion with abnormal lipids who were NW also declined. On regression analysis, temporal declines in NW and EW differed for HDL-C. CONCLUSIONS: NW constituted more than a quarter to half of youth with abnormal lipids. Over time, youth with abnormal lipids were less often NW. The novel observation that a high proportion of youth with abnormal lipids are NW is relevant to debates on universal lipid screening, the focus on weight reduction in youth lipid management, and conventional wisdom in cardiometabolic health.

Pediatric multisystem SARS COV2 with versus without cardiac involvement: a multicenter study from Latin America.

Latin America (LATAM) children offer special insight into Severe Acute Respiratory Syndrome Coronavirus 2 (SARS COV2) due to high-risk race/ethnicity, variability in medical resources, diverse socioeconomic background, and numerous involved organ systems. This multinational study of LATAM youth examined the distinguishing features of acute or late multisystem SARS COV2 with versus without cardiac involvement. A consecutive sample of youth 0-18 years old (N = 98;50% male) presenting with multisystem SARS COV2 to 32 centers in 10 Latin American countries participating in a pediatric cardiac multi-imaging society were grouped as with versus without cardiac involvement, defined as abnormal echocardiographic findings or arrhythmia. Collected clinical data were analyzed by Student's t-test or Fisher's exact test. Cardiac (N = 48, 50% male) versus no cardiac (N = 50, 50% male) were similar in age; weight; nonrespiratory symptoms; and medical history. The cardiac group had 1 death and symptoms including coronary artery dilation, ejection fraction <50%, pericardial effusion, peripheral edema, arrhythmia, and pulmonary artery thrombus. The cardiac group had higher risk of ICU admission (77% vs 54%, p = 0.02); invasive ventilation (23% vs 4%,p = 0.007); vasoactive infusions (27% vs 4%, p = 0.002); prominent respiratory symptoms (60% vs 36%, p < 0.03); abnormal chest imaging (69% vs 34%, p = 0.001); troponin (33% vs 12%, p = 0.01); alanine aminotransferase (33% vs 12%, p = 0.02); and thrombocytopenia (46% vs 22%, p = 0.02). Receiver operating curve analysis showed that abnormal laboratories had 94% sensitivity and 98% negative predictive value on the need for ICU interventions.Conclusion: In LATAM children with multisystem SARS COV2, cardiac involvement was prevalent. Cardiac involvement was more likely to require ICU interventions, certain abnormal labs, and respiratory involvement. What is Known: • SARS COV2 can be asymptomatic in children but in some cases can have serious multisystemic involvement. • Hispanic ethnicity is purportedly at high risk of SARS COV2 in nations where they are often disadvantaged minority populations. What is New: • Latin American children presenting with multisystem SARS COV2 frequently have cardiac involvement which was associated with ICU interventions; prominent respiratory symptoms; abnormal chest X-ray; elevated troponin, ALT, and thrombocytopenia. • Elevated troponin, ALT or thrombocytopenia had high sensitivity and negative predictive value on the need for intensive care interventions.

Cardiovascular Risk Reduction in High-Risk Pediatric Patients: A Scientific Statement From the American Heart Association.

This scientific statement presents considerations for clinical management regarding the assessment and risk reduction of select pediatric populations at high risk for premature cardiovascular disease, including acquired arteriosclerosis or atherosclerosis. For each topic, the evidence for accelerated acquired coronary artery disease and stroke in childhood and adolescence and the evidence for benefit of interventions in youth will be reviewed. Children and adolescents may be at higher risk for cardiovascular disease because of significant atherosclerotic or arteriosclerotic risk factors, high-risk conditions that promote atherosclerosis, or coronary artery or other cardiac or vascular abnormalities that make the individual more vulnerable to the adverse effects of traditional cardiovascular risk factors. Existing scientific statements and guidelines will be referenced when applicable, and suggestions for risk identification and reduction specific to each setting will be described. This statement is directed toward pediatric cardiologists, primary care providers, and subspecialists who provide clinical care for these young patients. The focus will be on management and justification for management, minimizing information on pathophysiology and epidemiology.

Childhood and Adolescent Adversity and Cardiometabolic Outcomes: A Scientific Statement From the American Heart Association.

Adverse experiences in childhood and adolescence, defined as subjectively perceived threats to the safety or security of the child's bodily integrity, family, or social structures, are known to be associated with cardiometabolic outcomes over the life course into adulthood. This American Heart Association scientific statement reviews the scientific literature on the influence of childhood adversity on cardiometabolic outcomes that constitute the greatest public health burden in the United States, including obesity, hypertension, type 2 diabetes mellitus, and cardiovascular disease. This statement also conceptually outlines pathways linking adversity to cardiometabolic health, identifies evidence gaps, and provides suggestions for future research to inform practice and policy. We note that, despite a lack of objective agreement on what subjectively qualifies as exposure to childhood adversity and a dearth of prospective studies, substantial evidence documents an association between childhood adversity and cardiometabolic outcomes across the life course. Future studies that focus on mechanisms, resiliency, and vulnerability factors would further strengthen the evidence and provide much-needed information on targets for effective interventions. Given that childhood adversities affect cardiometabolic health and multiple health domains across the life course, interventions that ameliorate these initial upstream exposures may be more appropriate than interventions remediating downstream cardiovascular disease risk factor effects later in life.

Predictors and Consequences of Pediatric Hypertension: Have Advanced Echocardiography and Vascular Testing Arrived?

PURPOSE OF REVIEW: Pediatric hypertension is relatively common and associated with future adult hypertension. Elevated blood pressure in youth predicts future adult cardiovascular disease and blood pressure control can prevent progression of pediatric kidney disease. However, pediatric blood pressure is highly variable within a given child and among children in a population. RECENT FINDINGS: Therefore, modalities to index aggregate and cumulative blood pressure status are of potential benefit in identifying youth in danger of progression from a risk factor of subclinical phenotypic alteration to clinically apparent event. In this review, we advocate for the health risk stratification roles of echocardiographically assessed cardiac remodeling, arterial stiffness assessment, and assessment by ultrasound of arterial thickening in children and adolescents with hypertension.

Hepatotoxicity of Statins as Determined by Serum Alanine Aminotransferase in a Pediatric Cohort With Dyslipidemia.

OBJECTIVE: The aim of the study was to evaluate the hepatotoxicity of statins, as determined by serum alanine aminotransferase (ALT), in children and adolescents with dyslipidemia in real-world clinical practice. STUDY DESIGN: Clinical and laboratory data were prospectively collected between September 2010 and March 2014. We compared ALT levels between patients prescribed versus not prescribed 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (statins), and then compared ALT before and after initiation of statins. RESULTS: Over the 3.5-year observation period, there were 2704 ALT measurements among 943 patients. The mean age was 14 years; 54% were boys, 47% obese, and 208 patients were treated with statins. Median follow-up after first ALT was 18 months. The mean (SD) ALT in statin and non-statin users was 23 (20) U/L and 28 (28) U/L, respectively. In models adjusted for age, sex, and race, ALT was 2.1 U/L (95% CI 0.1 to 4.4; P = 0.04) lower among statin users, which was attenuated after adjustment for weight category. Patients started on statins during the observation period did not demonstrate an increase in ALT over time (ALT 0.9 U/L [95% confidence interval -5.2 to 3.4] increase per year; P = 0.7). CONCLUSIONS: In our study population, we did not observe a higher burden of ALT elevations among pediatric patients on statins as compared to those with dyslipidemia who are not on statins, supporting the hepatic safety of statin use in childhood.

The relationship between payer type and lipid outcomes in response to clinical lifestyle interventions in youth with dyslipidemia.

BACKGROUND: Payer-type (government-sponsored health coverage versus private health insurance) has been shown to influence a variety of cardiovascular disease outcomes in adults. However, it is unclear if the payer-type impacts the response to a lifestyle intervention in children with dyslipidemia. METHODS: We analyzed data prospectively collected from patients under the age of 25 years who were referred to a large regional preventive cardiology clinic from 2010 to 2016 in Massachusetts. We compared baseline high density lipoprotein cholesterol (HDL-C), triglycerides (TG), non-HDL-C, and low density lipoprotein cholesterol (LDL-C) by payer-type. Further, we analyzed the change in lipid values in response to a clinic-based multidisciplinary intervention over a nearly six-year period by payer-type with multi-variable adjusted linear regression models. We also tested for effect modifications by age, sex, race, and body mass index (BMI) category. RESULTS: Of the 1739 eligible patients (mean age 13 years, 52% female, 60% overweight and obese, 59% White), we found that patients with government-sponsored coverage (n = 354, 20%) presented to referral lipid clinic with lower HDL-C (- 3.5 mg/dL [1.0], p < 0.001) and higher natural log-transformed TG (+ 0.14 [0.04], p < 0.001) as compared to those with private insurance; however, the association was attenuated to the null after additionally adjusting for BMI category (- 1.1 [0.9], p = 0.13, and + 0.05 [0.04], p = 0.2 for HDL-C and natural log-transformed TG, respectively). We found no difference in baseline LDL-C between payer-types (+ 3.4 mg/dL [3.0], p = 0.3). However, longitudinally, we found patients with private insurance and a self-reported race of White to have a clinically meaningful additional improvement in LDL-C, decreasing 12.8 (5.5) mg/dL (p = 0.02) between baseline and first follow-up, as compared to White patients with government-sponsored health coverage, after adjusting for age, sex, time between visits, and baseline LDL-C. CONCLUSIONS: Our results suggest that youth with government-sponsored coverage are referred with poorer lipid profiles than those with private insurance, although this is largely explained by higher rates of overweight and obesity in the government-sponsored health coverage group. White patients with private insurance had substantially better improvement in LDL-C longitudinally, suggesting that higher socioeconomic status facilitates improvement in LDL-C, but is less beneficial for HDL-C and triglyceride levels.

Cardiovascular Consequences of Childhood Secondhand Tobacco Smoke Exposure: Prevailing Evidence, Burden, and Racial and Socioeconomic Disparities: A Scientific Statement From the American Heart Association.

BACKGROUND: Although public health programs have led to a substantial decrease in the prevalence of tobacco smoking, the adverse health effects of tobacco smoke exposure are by no means a thing of the past. In the United States, 4 of 10 school-aged children and 1 of 3 adolescents are involuntarily exposed to secondhand tobacco smoke (SHS), with children of minority ethnic backgrounds and those living in low-socioeconomic-status households being disproportionately affected (68% and 43%, respectively). Children are particularly vulnerable, with little control over home and social environment, and lack the understanding, agency, and ability to avoid SHS exposure on their own volition; they also have physiological or behavioral characteristics that render them especially susceptible to effects of SHS. Side-stream smoke (the smoke emanating from the burning end of the cigarette), a major component of SHS, contains a higher concentration of some toxins than mainstream smoke (inhaled by the smoker directly), making SHS potentially as dangerous as or even more dangerous than direct smoking. Compelling animal and human evidence shows that SHS exposure during childhood is detrimental to arterial function and structure, resulting in premature atherosclerosis and its cardiovascular consequences. Childhood SHS exposure is also related to impaired cardiac autonomic function and changes in heart rate variability. In addition, childhood SHS exposure is associated with clustering of cardiometabolic risk factors such as obesity, dyslipidemia, and insulin resistance. Individualized interventions to reduce childhood exposure to SHS are shown to be at least modestly effective, as are broader-based policy initiatives such as community smoking bans and increased taxation. PURPOSE: The purpose of this statement is to summarize the available evidence on the cardiovascular health consequences of childhood SHS exposure; this will support ongoing efforts to further reduce and eliminate SHS exposure in this vulnerable population. This statement reviews relevant data from epidemiological studies, laboratory-based experiments, and controlled behavioral trials concerning SHS and cardiovascular disease risk in children. Information on the effects of SHS exposure on the cardiovascular system in animal and pediatric studies, including vascular disruption and platelet activation, oxidation and inflammation, endothelial dysfunction, increased vascular stiffness, changes in vascular structure, and autonomic dysfunction, is examined. CONCLUSIONS: The epidemiological, observational, and experimental evidence accumulated to date demonstrates the detrimental cardiovascular consequences of SHS exposure in children. IMPLICATIONS: Increased awareness of the adverse, lifetime cardiovascular consequences of childhood SHS may facilitate the development of innovative individual, family-centered, and community health interventions to reduce and ideally eliminate SHS exposure in the vulnerable pediatric population. This evidence calls for a robust public health policy that embraces zero tolerance of childhood SHS exposure.

Cardiovascular Health Promotion in Children: Challenges and Opportunities for 2020 and Beyond: A Scientific Statement From the American Heart Association.

This document provides a pediatric-focused companion to "Defining and Setting National Goals for Cardiovascular Health Promotion and Disease Reduction: The American Heart Association's Strategic Impact Goal Through 2020 and Beyond," focused on cardiovascular health promotion and disease reduction in adults and children. The principles detailed in the document reflect the American Heart Association's new dynamic and proactive goal to promote cardiovascular health throughout the life course. The primary focus is on adult cardiovascular health and disease prevention, but critical to achievement of this goal is maintenance of ideal cardiovascular health from birth through childhood to young adulthood and beyond. Emphasis is placed on the fundamental principles and metrics that define cardiovascular health in children for the clinical or research setting, and a balanced and critical appraisal of the strengths and weaknesses of the cardiovascular health construct in children and adolescents is provided. Specifically, this document discusses 2 important factors: the promotion of ideal cardiovascular health in all children and the improvement of cardiovascular health metric scores in children currently classified as having poor or intermediate cardiovascular health. Other topics include the current status of cardiovascular health in US children, opportunities for the refinement of health metrics, improvement of health metric scores, and possibilities for promoting ideal cardiovascular health. Importantly, concerns about the suitability of using single thresholds to identify elevated cardiovascular risk throughout the childhood years and the limits of our current knowledge are noted, and suggestions for future directions and research are provided.

Statin-Associated Myopathy in a Pediatric Preventive Cardiology Practice.

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.

Correlates of Achieving Statin Therapy Goals in Children and Adolescents with Dyslipidemia.

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.

Aortic Stiffness in Youth with Hypertrophic Cardiomyopathy Genotype.

Clinical events in hypertrophic cardiomyopathy (HCM) patients are related to the degree of hypertrophy. Aortic stiffness in adult HCM patients has been reported to be higher than control patients. Increased stiffness may cause more LV hypertrophy and thus lead to more clinical events. We sought to (a) noninvasively compare aortic structure and function between youth with sarcomeric HCM genotype versus control youth and (b) explore the relation between aortic function and degree of left ventricular (LV) hypertrophy. In a prospective study from a single referral center, clinical, anthropometric, and hemodynamic data were acquired on 28 consecutive pathogenic HCM gene mutation carriers and 26 unrelated controls (mean age 16.3, 50 % girls). Hemodynamic data included applanation tonometry measured central pulse pressure, carotid-femoral pulse wave velocity (CFPWV), reflected wave augmentation index (AIx). In the HCM gene carriers, LV mass-to-volume ratio was extracted from clinically indicated echocardiograms as an index of hypertrophy. Associations were assessed using multivariable adjusted linear regression. The HCM group was comprised of 14 myosin binding protein C3 carriers, 13 myosin heavy chain 7 carriers, and 1 child with both. HCM and control groups did not differ by age, sex, height, body mass index, heart rate, or blood pressure. HCM carriers had significantly lower CFPWV than controls (4.46 ± 0.88 vs. 4.97 ± 0.44 m/s, p = 0.01) and higher AIx magnitude (27 ± 19 vs. 18 ± 7 %, p = 0.04). These associations persisted after adjustment for age, sex, height, heart rate, mean pressure, and medication use. Within the HCM group, LV hypertrophy was related to AIx but not CFPWV. CFPWV nor AIx differed by genotype. Aortic stiffness appears lower, but wave reflection appears higher in youth carrying HCM gene mutations. The degree of wave reflection appears correlated with LV hypertrophy in this high-risk cohort, suggesting that mitigation of wave reflection may possibly attenuate LV hypertrophy.

Patterns of Lipid Lowering Therapy among Children Ages 8-20 Years.

OBJECTIVES: Pediatric guidelines in 2008 and 2011 recommended lipid lowering therapy in children ≥ 8 years of age with high-risk cardiovascular conditions, such as familial hypercholesterolemia (FH). Our objective was to describe the patterns and predictors of lipid lowering therapy initiation in commercially insured children between 2005 and 2010. STUDY DESIGN: Using commercial health plan data on children ages 8-20 years from 2004-2010, we estimated rates of lipid lowering therapy initiation overall and stratified by age. Using a nested case-control design, we used multivariable logistic regression to identify temporal, demographic, clinical, and health utilization characteristics associated with lipid lowering therapy initiation. RESULTS: Among >13 million children, 665 initiated lipid lowering therapy for an incidence rate 2.6/100,000 person-years (PY). Incidence rates were highest in 2005 (4.1/100,000 PY) and 2008 (3.9/100,000 PY), with no discernable secular trend. Rates of lipid lowering therapy initiation were significantly greater in children ≥ 15 years of age (OR 2.9 [95% CI 5.2-13.0]), males (2.1 [1.7-2.4]), and those with a diagnosis of FH (165.2 [129.0-211.6]), other dyslipidemia (175.5 [143.2-215.3]), diabetes type I (7.7 [4.7-12.4]), diabetes type II (13.6 [8.5-21.7]), hypertension (8.1 [4.9-13.3]), obesity (7.8 [4.7-12.7]), and ≥ 5 outpatient visits (1.5 [1.2-1.7]), and children with dispensing of ≥ 2 nonlipid lowering therapy prescriptions were less likely to initiate lipid lowering therapy (0.2 [0.2-0.3]). CONCLUSIONS: Despite new guidelines, lipid lowering therapy initiation in children is low and has not increased through 2010. Although diagnosis of FH and other dyslipidemias was associated with higher probability of lipid lowering therapy initiation, our findings suggest lipid lowering therapy is underutilized in this population given the prevalence of these disorders.

Pediatric High Blood Pressure Recognition Associated With Electronic Decision Support: A Cohort Analysis.

BACKGROUND: Pediatric high blood pressure (BP) predicts future cardiovascular disease events. High BP is improperly measured, underrecognized, and undermanaged especially in disadvantaged populations. In a large, diverse, academic pediatric practice, we detail the associations of a comprehensive initiative with high BP provider recognition. METHODS: A comprehensive BP initiative was promulgated including (1) retraining providers and staff on BP management; (2) deploying equipment at 55+ sites; and (3) electronic decision support tool alerting staff and clinicians and suggesting management. During the 14-month preintervention and 14-month postintervention periods, data on BP and patient characteristics were collected. The outcome was incident BP recognition defined as any of the following: BP-specific International Classification of Diseases, Tenth Revision, diagnosis; problem list entry; specialty referral; diagnostic testing; repeat visit; or antihypertensives. Dichotomized as underpre versus at or over 13 years of age, analyses utilized interrupted time series and multivariable-adjusted logistic regression. RESULTS: From preintervention (children, n=105 674; adolescents, n=54 365) to postintervention (children, n=87 917; adolescents, n=56 470), the proportion measured with high BP declined in children and adolescents (30% versus 14% and 30% versus 15%, respectively, each P<0.001). Post-intervention, high BP provider recognition was 58% higher in children (4.6%-7.3%) and 43% higher in adolescents (7.9% versus 11.3%; P<0.001 for both). The improvement was not different in disadvantaged race, ethnicity, or zip codes. CONCLUSIONS: A comprehensive BP initiative was associated with more appropriate high BP measurement and recognition including in disadvantaged populations. Future work may address the low overall and nonsustained recognition and consideration of electronic decision support for pediatric BP management and mitigating disparities.

Multiple accessory pathways in the young: the impact of structural heart disease.

BACKGROUND: The presence of multiple accessory pathways (MultAP) is described in structural heart disease (SHD) such as Ebstein's anomaly and cardiomyopathies. Structural defects can impact the tolerability of tachyarrhythmia and can complicate both medical management and ablation. In a large cohort of pediatric patients with and without SHD undergoing invasive electrophysiology study, we examined the prevalence of MultAP and the effect of both MultAP and SHD on ablation outcomes. METHODS: Accessory pathway number and location, presence of SHD, ablation success, and recurrence were analyzed in consecutive patients from our center over a 16-year period. RESULTS: In 1088 patients, 1228 pathways (36% retrograde only) were mapped to the right side (TV) in 18%, septum (S) in 39%, and left side (MV) in 43%. MultAP were present in 111 pts (10%), involving 250 distinct pathways. SHD tripled the risk of MultAP (26% SHD vs 8% no SHD, P < .001). Multivariable adjusted risk factors for MultAP included Ebstein's (OR 8.7[4.4-17.5], P < .001) and cardiomyopathy (OR 13.3[5.1-34.5], P < .001). Of 1306 ablation attempts, 94% were acutely successful with an 8% recurrence rate. Ablation success was affected by SHD (85% vs 95% for no SHD, P < .01) but not by MultAP (91% vs 94% for single, P = .24). Recurrence rate was higher for SHD (17% SHD vs 8% no SHD, P < .05) and MultAP (19% MultAP vs 8% single, P < .001). CONCLUSIONS: MultAP are found in 10% of pediatric patients, and are more common in SHD compared to those with normal hearts. Both the presence of MultAP and SHD negatively influence ablation outcomes.

Coarctation of the Aorta: Modern Paradigms Across the Lifespan.

While coarctation of the aorta varies greatly in both severity and age at presentation, all patients are at increased risk of hypertension both before and after repair. Despite advances in knowledge about genetic etiologies, pathophysiologic mechanisms, and optimal repair strategies, patients with repaired coarctation of the aorta remain at increased risk of acquired cardiovascular disease. The aims of this review are to describe the management of coarctation of the aorta at all ages before and after repair, highlight pathophysiologic mechanisms of hypertension, and review long-term follow-up considerations.