Obstructive sleep apnea (OSA) is a known risk factor for cardiovascular disease in adults. It is associated with incident systemic hypertension, arrhythmia, stroke, coronary artery disease, and heart failure. OSA is common in children and adolescents, but there has been less focus on OSA as a primary risk factor for cardiovascular disease in children and adolescents. This scientific statement summarizes what is known regarding the impact of sleep‐disordered breathing and, in particular, OSA on the cardiovascular health of children and adolescents. This statement highlights what is known regarding the impact of OSA on the risk for hypertension, arrhythmia, abnormal ventricular morphology, impaired ventricular contractility, and elevated right heart pressure among children and adolescents. This scientific statement also summarizes current best practices for the diagnosis and evaluation of cardiovascular disease–related complications of OSA in children and adolescents with sleep apnea and highlights potential future research in the area of sleep‐disordered breathing and cardiovascular health during childhood and adolescence.

BACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare genetic disorder characterized by severely elevated low-density lipoprotein cholesterol (LDL-C) levels due to profoundly defective LDL receptor (LDLR) function. Given that severely elevated LDL-C starts in utero, atherosclerosis often presents during childhood or adolescence, creating a largely unmet need for aggressive LDLR-independent lipid-lowering therapies in young patients with HoFH. Here we present the first evaluation of the efficacy and safety of evinacumab, a novel LDLR-independent lipid-lowering therapy, in pediatric patients with HoFH from parts A and B of a 3-part study. METHODS: The phase 3, part B, open-label study treated 14 patients 5 to 11 years of age with genetically proven HoFH (true homozygotes and compound heterozygotes) with LDL-C >130 mg/dL, despite optimized lipid-lowering therapy (including LDLR-independent apheresis and lomitapide), with intravenous evinacumab 15 mg/kg every 4 weeks. RESULTS: Evinacumab treatment rapidly and durably (through week 24) decreased LDL-C with profound reduction in the first week, with a mean (SE) LDL-C reduction of −48.3% (10.4%) from baseline to week 24. ApoB (mean [SE], –41.3% [9.0%]), non–high-density lipoprotein cholesterol (–48.9% [9.8%]), and total cholesterol (–49.1% [8.1%]) were similarly decreased. Treatment-emergent adverse events were reported in 10 (71.4%) patients; however, only 2 (14.3%) reported events that were considered to be treatment-related (nausea and abdominal pain). One serious treatment-emergent adverse event of tonsillitis occurred (n=1), but this was not considered treatment-related. CONCLUSIONS: Evinacumab constitutes a new treatment for pediatric patients with HoFH and inadequately controlled LDL-C despite optimized lipid-lowering therapy, lowering LDL-C levels by nearly half in these extremely high-risk and difficult-to-treat individuals. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT04233918.

Systemic hypertension is a major cause of morbidity and mortality in adulthood. High blood pressure (HBP) and repeated measures of HBP, hypertension (HTN), begin in youth. Knowledge of how best to diagnose, manage, and treat systemic HTN in children and adolescents is important for primary and subspecialty care providers. OBJECTIVES: To provide a technical summary of the methodology used to generate the 2017 “Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents,” an update to the 2004 “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.” DATA SOURCES: Medline, Cochrane Central Register of Controlled Trials, and Excerpta Medica Database references published between January 2003 and July 2015 followed by an additional search between August 2015 and July 2016. STUDY SELECTION: English-language observational studies and randomized trials. METHODS: Key action statements (KASs) and additional recommendations regarding the diagnosis, management, and treatment of HBP in youth were the product of a detailed systematic review of the literature. A content outline establishing the breadth and depth was followed by the generation of 4 patient, intervention, comparison, outcome, time questions. Key questions addressed: (1) diagnosis of systemic HTN, (2) recommended work-up of systemic HTN, (3) optimal blood pressure (BP) goals, and (4) impact of high BP on indirect markers of cardiovascular disease in youth. Once selected, references were subjected to a 2-person review of the abstract and title followed by a separate 2-person full-text review. Full citation information, population data, findings, benefits and harms of the findings, as well as other key reference information were archived. Selected primary references were then used for KAS generation. Level of evidence (LOE) scoring was assigned for each reference and then in aggregate. Appropriate language was used to generate each KAS based on the LOE and the balance of benefit versus harm of the findings. Topics that could not be researched via the stated approach were (1) definition of HTN in youth, and (2) definition of left ventricular hypertrophy. KASs related to these stated topics were generated via expert opinion. RESULTS: Nearly 15 000 references were identified during an initial literature search. After a deduplication process, 14 382 references were available for title and abstract review, and 1379 underwent full text review. One hundred twenty-four experimental and observational studies published between 2003 and 2016 were selected as primary references for KAS generation, followed by an additional 269 primary references selected between August 2015 and July 2016. The LOE for the majority of references was C. In total, 30 KASs and 27 additional recommendations were generated; 12 were related to the diagnosis of HTN, 13 were related to management and additional diagnostic testing, 3 to treatment goals, and 2 to treatment options. Finally, special additions to the clinical practice guideline included creation of new BP tables based on BP values obtained solely from children with normal weight, creation of a simplified table to enhance screening and recognition of abnormal BP, and a revision of the criteria for diagnosing left ventricular hypertrophy. CONCLUSIONS: An extensive and detailed systematic approach was used to generate evidence-based guidelines for the diagnosis, management, and treatment of youth with systemic HTN.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common. Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.

Addressing the need for more equitable cardio-oncology care requires attention to existing disparities in cardio-oncologic disease prevention and outcomes. This is particularly important among those affected by adverse social determinants of health (SDOH). The intricate relationship of SDOH, cancer diagnosis, and outcomes from cardiotoxicities associated with oncologic therapies is influenced by sociopolitical, economic, and cultural factors. Furthermore, mechanisms in cell signaling and epigenetic effects on gene expression link adverse SDOH to cancer and the CVD-related complications of oncologic therapies. To mitigate these disparities, a multifaceted strategy is needed that includes attention to health care access, policy, and community engagement for improved disease screening and management. Interdisciplinary teams must also promote cultural humility and competency and leverage new health technology to foster collaboration in addressing the impact of adverse SDOH in cardio-oncologic outcomes.