95 results on '"de Ferranti, Sarah"'
Search Results
2. Familial Hypercholesterolemia Variant and Cardiovascular Risk in Individuals With Elevated Cholesterol
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Zhang, Yiyi, Dron, Jacqueline S., Bellows, Brandon K., Khera, Amit V., Liu, Junxiu, Balte, Pallavi P., Oelsner, Elizabeth C., Amr, Sami Samir, Lebo, Matthew S., Nagy, Anna, Peloso, Gina M., Natarajan, Pradeep, Rotter, Jerome I., Willer, Cristen, Boerwinkle, Eric, Ballantyne, Christie M., Lutsey, Pamela L., Fornage, Myriam, Lloyd-Jones, Donald M., Hou, Lifang, Psaty, Bruce M., Bis, Joshua C., Floyd, James S., Vasan, Ramachandran S., Heard-Costa, Nancy L., Carson, April P., Hall, Michael E., Rich, Stephen S., Guo, Xiuqing, Kazi, Dhruv S., de Ferranti, Sarah D., and Moran, Andrew E.
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IMPORTANCE: Familial hypercholesterolemia (FH) is a genetic disorder that often results in severely high low-density lipoprotein cholesterol (LDL-C) and high risk of premature coronary heart disease (CHD). However, the impact of FH variants on CHD risk among individuals with moderately elevated LDL-C is not well quantified. OBJECTIVE: To assess CHD risk associated with FH variants among individuals with moderately (130-189 mg/dL) and severely (≥190 mg/dL) elevated LDL-C and to quantify excess CHD deaths attributable to FH variants in US adults. DESIGN, SETTING, AND PARTICIPANTS: A total of 21 426 individuals without preexisting CHD from 6 US cohort studies (Atherosclerosis Risk in Communities study, Coronary Artery Risk Development in Young Adults study, Cardiovascular Health Study, Framingham Heart Study Offspring cohort, Jackson Heart Study, and Multi-Ethnic Study of Atherosclerosis) were included, 63 of whom had an FH variant. Data were collected from 1971 to 2018, and the median (IQR) follow-up was 18 (13-28) years. Data were analyzed from March to May 2023. EXPOSURES: LDL-C, cumulative past LDL-C, FH variant status. MAIN OUTCOMES AND MEASURES: Cox proportional hazards models estimated associations between FH variants and incident CHD. The Cardiovascular Disease Policy Model projected excess CHD deaths associated with FH variants in US adults. RESULTS: Of the 21 426 individuals without preexisting CHD (mean [SD] age 52.1 [15.5] years; 12 041 [56.2%] female), an FH variant was found in 22 individuals with moderately elevated LDL-C (0.3%) and in 33 individuals with severely elevated LDL-C (2.5%). The adjusted hazard ratios for incident CHD comparing those with and without FH variants were 2.9 (95% CI, 1.4-6.0) and 2.6 (95% CI, 1.4-4.9) among individuals with moderately and severely elevated LDL-C, respectively. The association between FH variants and CHD was slightly attenuated when further adjusting for baseline LDL-C level, whereas the association was no longer statistically significant after adjusting for cumulative past LDL-C exposure. Among US adults 20 years and older with no history of CHD and LDL-C 130 mg/dL or higher, more than 417 000 carry an FH variant and were projected to experience more than 12 000 excess CHD deaths in those with moderately elevated LDL-C and 15 000 in those with severely elevated LDL-C compared with individuals without an FH variant. CONCLUSIONS AND RELEVANCE: In this pooled cohort study, the presence of FH variants was associated with a 2-fold higher CHD risk, even when LDL-C was only moderately elevated. The increased CHD risk appeared to be largely explained by the higher cumulative LDL-C exposure in individuals with an FH variant compared to those without. Further research is needed to assess the value of adding genetic testing to traditional phenotypic FH screening.
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- 2024
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3. Association of Severe Hypercholesterolemia and Familial Hypercholesterolemia Genotype With Risk of Coronary Heart Disease
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Zhang, Yiyi, Dron, Jacqueline S., Bellows, Brandon K., Khera, Amit V., Liu, Junxiu, Balte, Pallavi P., Oelsner, Elizabeth C., Amr, Sami Samir, Lebo, Matthew S., Nagy, Anna, Peloso, Gina M., Natarajan, Pradeep, Rotter, Jerome I., Willer, Cristen, Boerwinkle, Eric, Ballantyne, Christie M., Lutsey, Pamela L., Fornage, Myriam, Lloyd-Jones, Donald M., Hou, Lifang, Psaty, Bruce M., Bis, Joshua C., Floyd, James S., Vasan, Ramachandran S., Heard-Costa, Nancy L., Carson, April P., Hall, Michael E., Rich, Stephen S., Guo, Xiuqing, Kazi, Dhruv S., de Ferranti, Sarah D., and Moran, Andrew E.
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- 2023
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4. Multi-disciplinary collaborative consensus guidance statement on the assessment and treatment of postacute sequelae of SARS-CoV-2 infection (PASC) in children and adolescents.
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Malone, Laura A., Morrow, Amanda, Yuxi Chen, Curtis, Donna, de Ferranti, Sarah D., Desai, Monika, Fleming, Talya K., Giglia, Therese M., Hall, Trevor A., Henning, Ellen, Jadhav, Sneha, Johnston, Alicia M., Kathirithamby, Dona Rani C., Kokorelis, Christina, Lachenauer, Catherine, Lilun Li, Lin, Henry C., Locke, Tran, MacArthur, Carol, and Mann, Michelle
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- 2022
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5. International Atherosclerosis Society guidance for implementing best practice in the care of familial hypercholesterolaemia
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Watts, Gerald F., Gidding, Samuel S., Hegele, Robert A., Raal, Frederick J., Sturm, Amy C., Jones, Laney K., Sarkies, Mitchell N., Al-Rasadi, Khalid, Blom, Dirk J., Daccord, Magdalena, de Ferranti, Sarah D., Folco, Emanuela, Libby, Peter, Mata, Pedro, Nawawi, Hapizah M., Ramaswami, Uma, Ray, Kausik K., Stefanutti, Claudia, Yamashita, Shizuya, Pang, Jing, Thompson, Gilbert R., and Santos, Raul D.
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This contemporary, international, evidence-informed guidance aims to achieve the greatest good for the greatest number of people with familial hypercholesterolaemia (FH) across different countries. FH, a family of monogenic defects in the hepatic LDL clearance pathway, is a preventable cause of premature coronary artery disease and death. Worldwide, 35 million people have FH, but most remain undiagnosed or undertreated. Current FH care is guided by a useful and diverse group of evidence-based guidelines, with some primarily directed at cholesterol management and some that are country-specific. However, none of these guidelines provides a comprehensive overview of FH care that includes both the lifelong components of clinical practice and strategies for implementation. Therefore, a group of international experts systematically developed this guidance to compile clinical strategies from existing evidence-based guidelines for the detection (screening, diagnosis, genetic testing and counselling) and management (risk stratification, treatment of adults or children with heterozygous or homozygous FH, therapy during pregnancy and use of apheresis) of patients with FH, update evidence-informed clinical recommendations, and develop and integrate consensus-based implementation strategies at the patient, provider and health-care system levels, with the aim of maximizing the potential benefit for at-risk patients and their families worldwide.
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- 2023
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6. Using Adult Ambulatory Blood Pressure Thresholds in Youth: Impact of Nocturnal Hypertension.
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Lyon, Shannon M., Hartz, Jacob, Huang, Yisong, Chen, Ming Hui, and de Ferranti, Sarah D.
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Keywords: adolescents; blood pressure; blood pressure monitoring, ambulatory; child; hypertension EN adolescents blood pressure blood pressure monitoring, ambulatory child hypertension e68 e70 3 03/16/23 20230401 NES 230401 The recently published Ambulatory Blood Pressure Monitoring in Children and Adolescents: 2022 Update: A Scientific Statement from the American Heart Association provides new cut points for the diagnosis of hypertension using an ambulatory blood pressure (BP) monitor (ABPM) in those >=13 years old and updates ABPM diagnostic criteria.[1] The new cut points align with adult criteria and are generally lower than pediatric cut points published in 2014, particularly for adolescent males.[1],[2] The updated ABPM criteria will result in some adolescents being reclassified from normotensive to hypertensive. In analyzing the BP characteristics of group 2, the group reclassified as hypertensive by 2022 Guideline criteria, 44 studies (35%) had combined daytime and nocturnal hypertension, 4 (3%) had isolated daytime hypertension, and 77 (62%) had isolated nocturnal hypertension. Prevalence of hypertension (HTN) shown as combined day/nocturnal HTN, isolated daytime HTN, and isolated nocturnal HTN in group 2 (normotensive by 2014 criteria and reclassified as hypertensive by 2022 criteria) and group 3 (hypertensive by 2014 and 2022 criteria). [Extracted from the article]
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- 2023
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7. Adherence to lipid-lowering therapy and reaching treatment goals in youth seen in a preventive cardiology clinic.
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Hartz, Jacob, Ryan, Heather, Huang, Yisong, Baker, Annette, Bachman, Jennifer, McAuliffe, Ellen, and de Ferranti, Sarah
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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]
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- 2023
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8. Association of Acute Anti-inflammatory Treatment With Medium-term Outcomes for Coronary Artery Aneurysms in Kawasaki Disease
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Friedman, Kevin G., McCrindle, Brian W., Runeckles, Kyle, Dahdah, Nagib, Harahsheh, Ashraf S., Khoury, Michael, Lang, Sean, Manlhiot, Cedric, Tremoulet, Adriana H., Raghuveer, Geetha, Selamet Tierney, Elif Seda, Jone, Pei-Ni, Li, Jennifer S., Szmuszkovicz, Jacqueline R., Norozi, Kambiz, Jain, Supriya S., Yetman, Angela T., Newburger, Jane W., Altman, Carolyn A., Anderson, Brett R., Beckley, Mikayla, Braunlin, Elizabeth, Burns, Jane C., Carr, Michael R., Choueiter, Nadine F., Colyer, Jessica H., Dallaire, Frederic, De Ferranti, Sarah D., Desjardins, Laurent, Elias, Matthew D., Ferris, Anne, Gewitz, Michael, Giglia, Therese M., Greenway, Steven C., Harris, Kevin C., Hill, Kevin D., Hite, Michelle, Kimball, Thomas R., Kutty, Shelby, Lai, Lillian, Lee, Simon, Lin, Ming-Tai, Low, Tisiana, Mackie, Andrew S., Mawad, Wadi, McHugh, Kimberly E., Mondal, Tapas, Myers, Kimberly, Portman, Michael A., Renaud, Claudia, Scuccimarri, Rosie, Sexson Tejitel, S. Kristen, Texter, Karen M., Thacker, Deepika, Wagner-Lees, Sharon, Wong, Kenny, Wu, Mei-Hwan, and Zadokar, Varsha
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The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.
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- 2022
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9. SARS-CoV-2 Infection and Associated Cardiovascular Manifestations and Complications in Children and Young Adults: A Scientific Statement From the American Heart Association
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Jone, Pei-Ni, John, Anitha, Oster, Matthew E., Allen, Kiona, Tremoulet, Adrianna H., Saarel, Elizabeth V., Lambert, Linda M., Miyamoto, Shelley D., and de Ferranti, Sarah D.
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Coronavirus disease 2019 (COVID-19) resulted in a global pandemic and has overwhelmed health care systems worldwide. In this scientific statement, we describe the epidemiology, pathophysiology, clinical presentations, treatment, and outcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and multisystem inflammatory syndrome in children and young adults with a focus on cardiovascular manifestations and complications. We review current knowledge about the health consequences of this illness in children and young adults with congenital and acquired heart disease, the public health burden and health disparities of this infection in these populations, and vaccine-associated myocarditis.
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- 2022
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10. Insulin Prevents Hypercholesterolemia by Suppressing 12α-Hydroxylated Bile Acids
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Semova, Ivana, Levenson, Amy E., Krawczyk, Joanna, Bullock, Kevin, Gearing, Mary E., Ling, Alisha V., Williams, Kathryn A., Miao, Ji, Adamson, Stuart S., Shin, Dong-Ju, Chahar, Satyapal, Graham, Mark J., Crooke, Rosanne M., Hagey, Lee R., Vicent, David, de Ferranti, Sarah D., Kidambi, Srividya, Clish, Clary B., and Biddinger, Sudha B.
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- 2022
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11. Special Considerations for Healthy Lifestyle Promotion Across the Life Span in Clinical Settings: A Science Advisory From the American Heart Association
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Kris-Etherton, Penny M., Petersen, Kristina S., Després, Jean-Pierre, Braun, Lynne, de Ferranti, Sarah D., Furie, Karen L., Lear, Scott A., Lobelo, Felipe, Morris, Pamela B., and Sacks, Frank M.
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At a population level, engagement in healthy lifestyle behaviors is suboptimal in the United States. Moreover, marked disparities exist in healthy lifestyle behaviors and cardiovascular risk factors as a result of social determinants of health. In addition, there are specific challenges to engaging in healthy lifestyle behaviors related to age, developmental stage, or major life circumstances. Key components of a healthy lifestyle are consuming a healthy dietary pattern, engaging in regular physical activity, avoiding use of tobacco products, habitually attaining adequate sleep, and managing stress. For these health behaviors, there are guidelines and recommendations; however, promotion in clinical settings can be challenging, particularly in certain population groups. These challenges must be overcome to facilitate greater promotion of healthy lifestyle practices in clinical settings. The 5A Model (assess, advise, agree, assist, and arrange) was developed to provide a framework for clinical counseling with consideration for the demands of clinical settings. In this science advisory, we summarize specific considerations for lifestyle-related behavior change counseling using the 5A Model for patients across the life span. In all life stages, social determinants of health and unmet social-related health needs, as well as overweight and obesity, are associated with increased risk of cardiovascular disease, and there is the potential to modify this risk with lifestyle-related behavior changes. In addition, specific considerations for lifestyle-related behavior change counseling in life stages in which lifestyle behaviors significantly affect cardiovascular disease risk are outlined. Greater attention to healthy lifestyle behaviors during every clinician visit will contribute to improved cardiovascular health.
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- 2021
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12. Infliximab versus second intravenous immunoglobulin for treatment of resistant Kawasaki disease in the USA (KIDCARE): a randomised, multicentre comparative effectiveness trial
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Burns, Jane C, Roberts, Samantha C, Tremoulet, Adriana H, He, Feng, Printz, Beth F, Ashouri, Negar, Jain, Supriya S, Michalik, David E, Sharma, Kavita, Truong, Dongngan T, Wood, James B, Kim, Katherine K, Jain, Sonia, Anand, Vikram, Anderson, Marsha, Ang, Jocelyn, Ansusinha, Emily, Arditi, Moshe, Bartlett, Allison, Baker, Annette, Chatterjee, Archana, DeBiasi, Roberta, De Ferranti, Sarah, Dekker, Cornelia, DeZure, Chandani, Dominguez, Samuel, Erdem, Guliz, Halasa, Natasha, Harahsheh, Ashraf S., Hite, Michelle, Jaggi, Preeti, Jone, Pei-Ni, Jones, Jessica, Kaushik, Neeru, Kumar, Madan, Kurio, Gregory, Lloyd, David, Manaloor, John, McNelis, Amy, Nadipuram, Santhosh, Newburger, Jane, Newcomer, Charles, Perkins, Tiffany, Portman, Michael, Romero, José R, Rometo, Allison, Ronis, Tova, Rosenkranz, Margalit, Rowley, Anne, Samuy, Nichole, Scalici, Paul, Schuster, Jennifer, Sexson Tejtel, S. Kristen, Simonsen, Kari, Szmuszkovicz, Jacqueline, and Yeh, Sylvia
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Although intravenous immunoglobulin (IVIG) is effective therapy for Kawasaki disease, 10–20% of patients have recrudescent fever as a sign of persistent inflammation and require additional treatment. We aimed to compare infliximab with a second infusion of IVIG for treatment of resistant Kawasaki disease.
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- 2021
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13. Association Between Cumulative Low-Density Lipoprotein Cholesterol Exposure During Young Adulthood and Middle Age and Risk of Cardiovascular Events
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Zhang, Yiyi, Pletcher, Mark J., Vittinghoff, Eric, Clemons, Autumn M., Jacobs, David R., Allen, Norrina B., Alonso, Alvaro, Bellows, Brandon K., Oelsner, Elizabeth C., Zeki Al Hazzouri, Adina, Kazi, Dhruv S., de Ferranti, Sarah D., and Moran, Andrew E.
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IMPORTANCE: Low-density lipoprotein cholesterol (LDL-C) is a major risk factor for cardiovascular disease (CVD). Most observational studies on the association between LDL-C and CVD have focused on LDL-C level at a single time point (usually in middle or older age), and few studies have characterized long-term exposures to LDL-C and their role in CVD risk. OBJECTIVE: To evaluate the associations of cumulative exposure to LDL-C, time-weighted average (TWA) LDL-C, and the LDL-C slope change during young adulthood and middle age with incident CVD later in life. DESIGN, SETTING, AND PARTICIPANTS: This cohort study analyzed pooled data from 4 prospective cohort studies in the US (Atherosclerosis Risk in Communities Study, Coronary Artery Risk Development in Young Adults Study, Framingham Heart Study Offspring Cohort, and Multi-Ethnic Study of Atherosclerosis). Participants were included if they had 2 or more LDL-C measures that were at least 2 years apart between ages 18 and 60 years, with at least 1 of the LDL-C measures occurring during middle age at 40 to 60 years. Data from 1971 to 2017 were collected and analyzed from September 25, 2020, to January 10, 2021. EXPOSURES: Cumulative exposure to LDL-C, TWA LDL-C, and LDL-C slope from age 18 to 60 years. MAIN OUTCOMES AND MEASURES: Incident coronary heart disease (CHD), ischemic stroke, and heart failure (HF). RESULTS: A total of 18 288 participants were included in this study. These participants had a mean (SD) age of 56.4 (3.7) years and consisted of 10 309 women (56.4%). During a median follow-up of 16 years, 1165 CHD, 599 ischemic stroke, and 1145 HF events occurred. In multivariable Cox proportional hazards regression models that adjusted for the most recent LDL-C level measured during middle age and for other CVD risk factors, the hazard ratios for CHD were as follows: 1.57 (95% CI, 1.10-2.23; P for trend = .01) for cumulative LDL-C level, 1.69 (95% CI, 1.23-2.31; P for trend <.001) for TWA LDL-C level, and 0.88 (95% CI, 0.69-1.12; P for trend = .28) for LDL-C slope. No association was found between any of the LDL-C variables and ischemic stroke or HF. CONCLUSIONS AND RELEVANCE: This cohort study showed that cumulative LDL-C and TWA LDL-C during young adulthood and middle age were associated with the risk of incident CHD, independent of midlife LDL-C level. These findings suggest that past levels of LDL-C may inform strategies for primary prevention of CHD and that maintaining optimal LDL-C levels at an earlier age may reduce the lifetime risk of developing atherosclerotic CVD.
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- 2021
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14. Association of Myocarditis With BNT162b2 Messenger RNA COVID-19 Vaccine in a Case Series of Children
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Dionne, Audrey, Sperotto, Francesca, Chamberlain, Stephanie, Baker, Annette L., Powell, Andrew J., Prakash, Ashwin, Castellanos, Daniel A., Saleeb, Susan F., de Ferranti, Sarah D., Newburger, Jane W., and Friedman, Kevin G.
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IMPORTANCE: The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. OBJECTIVE: To review results of comprehensive cardiac imaging in children with myocarditis after COVID-19 vaccine. DESIGN, SETTING, AND PARTICIPANTS: This study was a case series of children younger than 19 years hospitalized with myocarditis within 30 days of BNT162b2 messenger RNA COVID-19 vaccine. The setting was a single-center pediatric referral facility, and admissions occurred between May 1 and July 15, 2021. MAIN OUTCOMES AND MEASURES: All patients underwent cardiac evaluation including an electrocardiogram, echocardiogram, and cardiac magnetic resonance imaging. RESULTS: Fifteen patients (14 male patients [93%]; median age, 15 years [range, 12-18 years]) were hospitalized for management of myocarditis after receiving the BNT162b2 (Pfizer) vaccine. Symptoms started 1 to 6 days after receipt of the vaccine and included chest pain in 15 patients (100%), fever in 10 patients (67%), myalgia in 8 patients (53%), and headache in 6 patients (40%). Troponin levels were elevated in all patients at admission (median, 0.25 ng/mL [range, 0.08-3.15 ng/mL]) and peaked 0.1 to 2.3 days after admission. By echocardiographic examination, decreased left ventricular (LV) ejection fraction (EF) was present in 3 patients (20%), and abnormal global longitudinal or circumferential strain was present in 5 patients (33%). No patient had a pericardial effusion. Cardiac magnetic resonance imaging findings were consistent with myocarditis in 13 patients (87%) including late gadolinium enhancement in 12 patients (80%), regional hyperintensity on T2-weighted imaging in 2 patients (13%), elevated extracellular volume fraction in 3 patients (20%), and elevated LV global native T1 in 2 patients (20%). No patient required intensive care unit admission, and median hospital length of stay was 2 days (range 1-5). At follow-up 1 to 13 days after hospital discharge, 11 patients (73%) had resolution of symptoms. One patient (7%) had persistent borderline low LV systolic function on echocardiogram (EF 54%). Troponin levels remained mildly elevated in 3 patients (20%). One patient (7%) had nonsustained ventricular tachycardia on ambulatory monitor. CONCLUSIONS AND RELEVANCE: In this small case series study, myocarditis was diagnosed in children after COVID-19 vaccination, most commonly in boys after the second dose. In this case series, in short-term follow-up, patients were mildly affected. The long-term risks associated with postvaccination myocarditis remain unknown. Larger studies with longer follow-up are needed to inform recommendations for COVID-19 vaccination in this population.
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- 2021
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15. Low-Density Lipoprotein Cholesterol Trajectories and Prevalence of High Low-Density Lipoprotein Cholesterol Consistent With Heterozygous Familial Hypercholesterolemia in US Children
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Zhang, Yiyi, Woo, Jessica G., Urbina, Elaine M., Jacobs, David R., Moran, Andrew E., and de Ferranti, Sarah D.
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- 2021
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16. Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry.
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de Ferranti, Sarah D., Shrader, Peter, Linton, MacRae F., Knowles, Joshua W., Hudgins, Lisa C., Benuck, Irwin, Kindt, Iris, O'Brien, Emily C., Peterson, Amy L., Ahmad, Zahid S., Clauss, Sarah, Duell, P. Barton, Shapiro, Michael D., Wilemon, Katherine, Gidding, Samuel S., and Neal, William
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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]- Published
- 2021
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17. Young Women's Perceptions of Heart Disease Risk.
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Gooding, Holly C., Brown, Courtney A., Revette, Anna C., Vaccarino, Viola, Liu, Jingyi, Patterson, Sierra, Stamoulis, Catherine, and de Ferranti, Sarah D.
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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]
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- 2020
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18. Society for Cardiovascular Magnetic Resonance 2023 Cases of SCMR Case Series
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Johnson, Jason N., Hoke, Cara, Chamis, Anna Lisa, Campbell, Michael Jay, Gearhart, Addison, de Ferranti, Sarah D, Beroukhim, Rebecca, Mozumdar, Namrita, Cartoski, Mark, Nees, Shannon, Hudson, Jonathan, Kakhi, Sorayya, Daryani, Yousef, Pasan Botheju, W. Savindu, Shah, Keyur B., Makkiya, Mohammed, Dimza, Michelle, Moguillansky, Diego, Al-Ani, Mohammad, Andreae, Andrew, Kim, Han, Ahamed, Hisham, Kannan, Rajesh, Joji, Chris Ann, Baritussio, Anna, Dendy, Jeffrey M., Bhagirath, Pranav, Ganigara, Madhusudan, Hulten, Edward, Tunks, Robert, Kozor, Rebecca, and Chen, Sylvia S.M.
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“Cases of SCMR” is a case series on the SCMR website (https://www.scmr.org) for the purpose of education. The cases reflect the clinical presentation, and the use of cardiovascular magnetic resonance (CMR) in the diagnosis and management of cardiovascular disease. The 2023 digital collection of cases are presented in this manuscript.
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- 2024
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19. Type 1 diabetes is associated with an increase in cholesterol absorption markers but a decrease in cholesterol synthesis markers in a young adult population.
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Semova, Ivana, Levenson, Amy E., Krawczyk, Joanna, Bullock, Kevin, Williams, Kathryn A., Wadwa, R. Paul, Shah, Amy S., Khoury, Philip R., Kimball, Thomas R., Urbina, Elaine M., de Ferranti, Sarah D., Bishop, Franziska K., Maahs, David M., Dolan, Lawrence M., Clish, Clary B., and Biddinger, Sudha B.
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CARDIOVASCULAR disease prevention ,CHOLESTEROL metabolism ,BIOMARKERS ,CARDIOVASCULAR diseases risk factors ,CHOLESTEROL ,GLYCOSYLATED hemoglobin ,TYPE 1 diabetes ,MASS spectrometry ,RESEARCH funding ,TRIGLYCERIDES ,BODY mass index ,ADOLESCENCE - Abstract
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]
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- 2019
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20. Primary adjunctive corticosteroid therapy is associated with improved outcomes for patients with Kawasaki disease with coronary artery aneurysms at diagnosis
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Friedman, Kevin G, Gauvreau, Kimberlee, Baker, Annette, Son, Mary Beth, Sundel, Robert, Dionne, Audrey, Giorgio, Thomas, De Ferranti, Sarah, and Newburger, Jane W
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ObjectivePatients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis.DesignSingle-centre, retrospective review.PatientsPatients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5.InterventionsPrimary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48).Main outcome measuresTreatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate.ResultsOf the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002).ConclusionPrimary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.
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- 2021
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21. Update on Preventive Cardiology
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Clauss, Sarah B. and de Ferranti, Sarah D.
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Although progress had been made in reducing cardiovascular disease (CVD) mortality, the positive trend has reversed in recent years, and CVD remains the most common cause of mortality in US women and men. Youth represent the future of CVD prevention; emerging evidence suggests exposure to risk factors in children contributes to atherosclerosis and results in vascular changes and increased CVD events. The contributors to CVD include those commonly seen in adults. This article reviews hypercholesterolemia, hypertension, obesity, diabetes, and smoking. It discusses the prevalence of each disease, diagnosis, treatment, and cardiovascular complications.
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- 2020
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22. Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial
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Manlhiot, Cedric, Newburger, Jane W., Low, Tisiana, Dahdah, Nagib, Mackie, Andrew S., Raghuveer, Geetha, Giglia, Therese M., Dallaire, Frederic, Mathew, Mathew, Runeckles, Kyle, Pahl, Elfriede, Harahsheh, Ashraf S., Norozi, Kambiz, de Ferranti, Sarah D., Friedman, Kevin, Yetman, Anji T., Kutty, Shelby, Mondal, Tapas, McCrindle, Brian W., Altman, Carolyn A., Anderson, Brett R., Braunlin, Elizabeth, Burns, Jane C., Carr, Michael R., Choueiter, Nadine F., Colyer, Jessica H., Crean, Andrew, Dempsey, Adam, Desjardins, Laurent, Dillenburg, Rejane, Dionne, Audrey, Ferris, Anna, Gewitz, Michael, Grcic, Michelle M., Greenway, Steven C., Hamel-Perrault, Elodie, Harris, Kevin C., Hayden-Rush, Christina, Hill, Kevin D., Jain, Supriya, Jone, Pei-Ni, Kimball, Thomas R., Lang, Sean M., Li, Jennifer S., Lin, Ming-Tai, Mahle, William T., McHugh, Kimberly E., Portman, Michael A., Renaud, Claudia, Sexson Tejitel, S. Kristen, Szmuszkovicz, Jacqueline R., Texter, Karen M., Thacker, Deepika, Selamet Tierney, Elif Seda, Thomas, Thomas, Tremoulet, Adriana H., Wagner-Lees, Sharon, and Warren, Andrew
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The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs.
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- 2020
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23. Cardiovascular Risk and Cardiovascular Health Behaviours in the Transition From Childhood to Adulthood
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Chung, Richard J., Mackie, Andrew S., Baker, Annette, and de Ferranti, Sarah D.
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The prevention and management of cardiovascular risk factors during the transition from childhood to adulthood is critically important in defining cardiovascular health trajectories. Unfortunately, many young people fall out of clinical care during this important time, leading to worsening cardiovascular risk and missed opportunities to modify future outcomes. The field of health care transition has evolved to support young people with complex health needs in developing self-management and self-advocacy skills to promote positive health outcomes despite changes in health care providers and resources. While transitional care efforts are largely focused on childhood-onset chronic illnesses such as sickle cell disease and cystic fibrosis, young people with cardiovascular risk factors such as hypertension, obesity, and dyslipidemia also stand to benefit from structured supports to ensure continuity in care and positive health behaviours. On the backdrop of the broader health care transition literature, we offer practical insights and suggestions for ensuring that young people with cardiovascular risk factors experience uninterrupted high-quality care and support as they enter the adult health care system. Starting transition preparation in early adolescence, actively engaging all key stakeholders throughout the process, and remaining mindful of the developmental underpinnings and social context of transition are keys to success.
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- 2020
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24. Pediatric Lipid Screening and Treatment in Canada: Practices, Attitudes, and Barriers
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Khoury, Michael, Rodday, Angie Mae, Mackie, Andrew S., Gill, Peter, McLaughlin, Tom, Harris, Kevin C., Wong, Peter, McCrindle, Brian W., Birken, Catherine S., and de Ferranti, Sarah D.
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The pediatric lipid screening and treatment practices, attitudes, and perceived barriers of Canadian pediatricians are not known. We sought to evaluate this in a survey of pediatricians through the Canadian Pediatric Surveillance Program (CPSP) in March 2019. The survey included an assessment of lipid screening of 9- to 11-year-old youth and a hypothetical case of persistent severe dyslipidemia to ascertain management practices. There were 759 respondents (28% response rate, 759 of 2742), of whom 236 provided outpatient primary care to 9- to 11-year-old youth as part of their routine clinical practice. Among primary care-providing pediatricians, universal lipid screening of healthy 9- to 11-year-old youth most or all of the time was reported by 3% (8 of 230). Reported screening practices most or all of the time were more common for youth with risk factors such as overweight and obesity (54%, 127 of 235) and a family history of premature cardiovascular disease (39%, 85 of 217). Most respondents would refer a child with severe persistent dyslipidemia to dieticians (69%, 152 of 220) and a lipid specialist (64%, 144 of 220) most or all of the time, whereas 7% (16 of 220) would start statin therapy themselves. A lack of Canadian pediatric lipid guidelines was reported as a major barrier for 49% (114 of 233) and minor barrier for 40% (93 of 213). The rate of routine lipid screening of healthy 9- to 11-year-old youth among Canadian primary care-providing pediatricians is low and at odds with current US guidelines. This discrepancy may be due at least in part to a lack of Canadian guidelines on pediatric dyslipidemia, the development of which may address certain perceived barriers and influence future attitudes.
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- 2020
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25. The American Heart Association 2030 Impact Goal: A Presidential Advisory From the American Heart Association
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Angell, Sonia Y., McConnell, Michael V., Anderson, Cheryl A.M., Bibbins-Domingo, Kirsten, Boyle, Douglas S., Capewell, Simon, Ezzati, Majid, de Ferranti, Sarah, Gaskin, Darrell J., Goetzel, Ron Z., Huffman, Mark D., Jones, Marsha, Khan, Yosef M., Kim, Sonia, Kumanyika, Shiriki K., McCray, Alexa T., Merritt, Robert K., Milstein, Bobby, Mozaffarian, Dariush, Norris, Tyler, Roth, Gregory A., Sacco, Ralph L., Saucedo, Jorge F., Shay, Christina M., Siedzik, David, Saha, Somava, and Warner, John J.
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Each decade, the American Heart Association (AHA) develops an Impact Goal to guide its overall strategic direction and investments in its research, quality improvement, advocacy, and public health programs. Guided by the AHA’s new Mission Statement, to be a relentless force for a world of longer, healthier lives, the 2030 Impact Goal is anchored in an understanding that to achieve cardiovascular health for all, the AHA must include a broader vision of health and well-being and emphasize health equity. In the next decade, by 2030, the AHA will strive to equitably increase healthy life expectancy beyond current projections, with global and local collaborators, from 66 years of age to at least 68 years of age across the United States and from 64 years of age to at least 67 years of age worldwide. The AHA commits to developing additional targets for equity and well-being to accompany this overarching Impact Goal. To attain the 2030 Impact Goal, we recommend a thoughtful evaluation of interventions available to the public, patients, providers, healthcare delivery systems, communities, policy makers, and legislators. This presidential advisory summarizes the task force’s main considerations in determining the 2030 Impact Goal and the metrics to monitor progress. It describes the aspiration that these goals will be achieved by working with a diverse community of volunteers, patients, scientists, healthcare professionals, and partner organizations needed to ensure success.
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- 2020
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26. Reducing the Clinical and Public Health Burden of Familial Hypercholesterolemia: A Global Call to Action
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Wilemon, Katherine A., Patel, Jasmine, Aguilar-Salinas, Carlos, Ahmed, Catherine D., Alkhnifsawi, Mutaz, Almahmeed, Wael, Alonso, Rodrigo, Al-Rasadi, Khalid, Badimon, Lina, Bernal, Luz M., Bogsrud, Martin P., Braun, Lynne T., Brunham, Liam, Catapano, Alberico L., Cillíková, Kristyna, Corral, Pablo, Cuevas, Regina, Defesche, Joep C., Descamps, Olivier S., de Ferranti, Sarah, Eiselé, Jean-Luc, Elikir, Gerardo, Folco, Emanuela, Freiberger, Tomas, Fuggetta, Francesco, Gaspar, Isabel M., Gesztes, Ákos G., Grošelj, Urh, Hamilton-Craig, Ian, Hanauer-Mader, Gabriele, Harada-Shiba, Mariko, Hastings, Gloria, Hovingh, G. Kees, Izar, Maria C., Jamison, Allison, Karlsson, Gunnar N., Kayikçioglu, Meral, Koob, Sue, Koseki, Masahiro, Lane, Stacey, Lima-Martinez, Marcos M., López, Greizy, Martinez, Tania L., Marais, David, Marion, Letrillart, Mata, Pedro, Maurina, Inese, Maxwell, Diana, Mehta, Roopa, Mensah, George A., Miserez, André R., Neely, Dermot, Nicholls, Stephen J., Nohara, Atsushi, Nordestgaard, Børge G., Ose, Leiv, Pallidis, Athanasios, Pang, Jing, Payne, Jules, Peterson, Amy L., Popescu, Monica P., Puri, Raman, Ray, Kausik K., Reda, Ashraf, Sampietro, Tiziana, Santos, Raul D., Schalkers, Inge, Schreier, Laura, Shapiro, Michael D., Sijbrands, Eric, Soffer, Daniel, Stefanutti, Claudia, Stoll, Mario, Sy, Rody G., Tamayo, Martha L., Tilney, Myra K., Tokgözoglu, Lale, Tomlinson, Brian, Vallejo-Vaz, Antonio J., Vazquez-Cárdenas, Alejandra, de Luca, Patrícia Vieira, Wald, David S., Watts, Gerald F., Wenger, Nanette K., Wolf, Michaela, Wood, David, Zegerius, Aram, Gaziano, Thomas A., and Gidding, Samuel S.
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IMPORTANCE: Familial hypercholesterolemia (FH) is an underdiagnosed and undertreated genetic disorder that leads to premature morbidity and mortality due to atherosclerotic cardiovascular disease. Familial hypercholesterolemia affects 1 in 200 to 250 people around the world of every race and ethnicity. The lack of general awareness of FH among the public and medical community has resulted in only 10% of the FH population being diagnosed and adequately treated. The World Health Organization recognized FH as a public health priority in 1998 during a consultation meeting in Geneva, Switzerland. The World Health Organization report highlighted 11 recommendations to address FH worldwide, from diagnosis and treatment to family screening and education. Research since the 1998 report has increased understanding and awareness of FH, particularly in specialty areas, such as cardiology and lipidology. However, in the past 20 years, there has been little progress in implementing the 11 recommendations to prevent premature atherosclerotic cardiovascular disease in an entire generation of families with FH. OBSERVATIONS: In 2018, the Familial Hypercholesterolemia Foundation and the World Heart Federation convened the international FH community to update the 11 recommendations. Two meetings were held: one at the 2018 FH Foundation Global Summit and the other during the 2018 World Congress of Cardiology and Cardiovascular Health. Each meeting served as a platform for the FH community to examine the original recommendations, assess the gaps, and provide commentary on the revised recommendations. The Global Call to Action on Familial Hypercholesterolemia thus represents individuals with FH, advocacy leaders, scientific experts, policy makers, and the original authors of the 1998 World Health Organization report. Attendees from 40 countries brought perspectives on FH from low-, middle-, and high-income regions. Tables listing country-specific government support for FH care, existing country-specific and international FH scientific statements and guidelines, country-specific and international FH registries, and known FH advocacy organizations around the world were created. CONCLUSIONS AND RELEVANCE: By adopting the 9 updated public policy recommendations created for this document, covering awareness; advocacy; screening, testing, and diagnosis; treatment; family-based care; registries; research; and cost and value, individual countries have the opportunity to prevent atherosclerotic heart disease in their citizens carrying a gene associated with FH and, likely, all those with severe hypercholesterolemia as well.
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- 2020
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27. Impact of Socioeconomic Status on Outcomes of Patients with Kawasaki Disease.
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Dionne, Audrey, Bucholz, Emily M., Gauvreau, Kimberlee, Gould, Patrick, Son, Mary Beth F., Baker, Annette L., de Ferranti, Sarah D., Fulton, David R., Friedman, Kevin G., and Newburger, Jane W.
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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]- Published
- 2019
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28. Safety and Efficacy of Warfarin Therapy in Kawasaki Disease.
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Baker, Annette L., Vanderpluym, Christina, Gauvreau, Kimberly A., Fulton, David R., de Ferranti, Sarah D., Friedman, Kevin G., Murray, Jenna M., Brown, Loren D., Almond, Christopher S., Evans-Langhorst, Margaret, and Newburger, Jane W.
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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]- Published
- 2017
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29. Omega-3 Fatty Acids for the Management of Hypertriglyceridemia: A Science Advisory From the American Heart Association
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Skulas-Ray, Ann C., Wilson, Peter W.F., Harris, William S., Brinton, Eliot A., Kris-Etherton, Penny M., Richter, Chesney K., Jacobson, Terry A., Engler, Mary B., Miller, Michael, Robinson, Jennifer G., Blum, Conrad B., Rodriguez-Leyva, Delfin, de Ferranti, Sarah D., and Welty, Francine K.
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Hypertriglyceridemia (triglycerides 200–499 mg/dL) is relatively common in the United States, whereas more severe triglyceride elevations (very high triglycerides, ≥500 mg/dL) are far less frequently observed. Both are becoming increasingly prevalent in the United States and elsewhere, likely driven in large part by growing rates of obesity and diabetes mellitus. In a 2002 American Heart Association scientific statement, the omega-3 fatty acids (n-3 FAs) eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) were recommended (at a dose of 2–4 g/d) for reducing triglycerides in patients with elevated triglycerides. Since 2002, prescription agents containing EPA+DHA or EPA alone have been approved by the US Food and Drug Administration for treating very high triglycerides; these agents are also widely used for hypertriglyceridemia. The purpose of this advisory is to summarize the lipid and lipoprotein effects resulting from pharmacological doses of n-3 FAs (>3 g/d total EPA+DHA) on the basis of new scientific data and availability of n-3 FA agents. In treatment of very high triglycerides with 4 g/d, EPA+DHA agents reduce triglycerides by ≥30% with concurrent increases in low-density lipoprotein cholesterol, whereas EPA-only did not raise low-density lipoprotein cholesterol in very high triglycerides. When used to treat hypertriglyceridemia, n-3 FAs with EPA+DHA or with EPA-only appear roughly comparable for triglyceride lowering and do not increase low-density lipoprotein cholesterol when used as monotherapy or in combination with a statin. In the largest trials of 4 g/d prescription n-3 FA, non–high-density lipoprotein cholesterol and apolipoprotein B were modestly decreased, indicating reductions in total atherogenic lipoproteins. The use of n-3 FA (4 g/d) for improving atherosclerotic cardiovascular disease risk in patients with hypertriglyceridemia is supported by a 25% reduction in major adverse cardiovascular events in REDUCE-IT (Reduction of Cardiovascular Events With EPA Intervention Trial), a randomized placebo-controlled trial of EPA-only in high-risk patients treated with a statin. The results of a trial of 4 g/d prescription EPA+DHA in hypertriglyceridemia are anticipated in 2020. We conclude that prescription n-3 FAs (EPA+DHA or EPA-only) at a dose of 4 g/d (>3 g/d total EPA+DHA) are an effective and safe option for reducing triglycerides as monotherapy or as an adjunct to other lipid-lowering agents.
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- 2019
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30. Experience participating in the American College of Cardiology Quality NetworkTM: paediatric and adult congenital cardiology collaborative quality improvement
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Hartwell, Lauren P., Baker, Annette L., Jenkins, Kathy J., de Ferranti, Sarah D., and Saleeb, Susan F.
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AbstractBackgroundThe American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network.MethodsQuarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances.ResultsChest pain metric performance was high compared with national means: obtaining family history (90–100% versus 51–77%), electrocardiogram (100% versus 89–99%), and echocardiogram for exertional complaints (95–100% versus 74–96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85–97%), prescribing aspirin (100% versus 86–99%), follow-up with imaging (100% versus 85–98%), and documenting no activity restriction without coronary aneurysms (83–100% versus 64–93%). Counselling for elevated body mass index was variable (25–75% versus 31–50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60–85% versus 54–68%) with improved genetics data capture.ConclusionThe Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.
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- 2019
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31. Ideal Cardiovascular Health, Cardiovascular Remodeling, and Heart Failure in Blacks.
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Spahillari, Aferdita, Talegawkar, Sameera, Correa, Adolfo, Carr, J. Jeffrey, Terry, James G., Lima, João, Freedman, Jane E., Das, Saumya, Kociol, Robb, de Ferranti, Sarah, Mohebali, Donya, Mwasongwe, Stanford, Tucker, Katherine L., Murthy, Venkatesh L., and Shah, Ravi V.
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Background--The lifetime risk of heart failure (HF) is higher in the black population than in other racial groups in the United States. Methods and Results--We measured the Life's Simple 7 ideal cardiovascular health metrics in 4195 blacks in the JHS (Jackson Heart Study; 2000-2004). We evaluated the association of Simple 7 metrics with incident HF and left ventricular structure and function by cardiac magnetic resonance (n=1188). Mean age at baseline was 54.4 years (65% women). Relative to 0 to 2 Simple 7 factors, blacks with 3 factors had 47% lower incident HF risk (hazard ratio [HR], 0.53; 95% confidence interval [CI], 0.39-0.73; P<0.0001); and those with ≥4 factors had 61% lower HF risk (HR, 0.39; 95% CI, 0.24-0.64; P=0.0002). Higher blood pressure (HR, 2.32; 95% CI, 1.28-4.20; P=0.005), physical inactivity (HR, 1.65; 95% CI, 1.07-2.55; P=0.02), smoking (HR, 2.04; 95% CI, 1.43-2.91; P<0.0001), and impaired glucose control (HR, 1.76; 95% CI, 1.34-2.29; P<0.0001) were associated with incident HF. The age-/sex-adjusted population attributable risk for these Simple 7 metrics combined was 37.1%. Achievement of ideal blood pressure, ideal body mass index, ideal glucose control, and nonsmoking was associated with less likelihood of adverse cardiac remodeling by cardiac magnetic resonance. Conclusions--Cardiovascular risk factors in midlife (specifically elevated blood pressure, physical inactivity, smoking, and poor glucose control) are associated with incident HF in blacks and represent targets for intensified HF prevention. [ABSTRACT FROM AUTHOR]
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- 2017
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32. Transitioning from pediatric to adult health care with familial hypercholesterolemia: Listening to young adult and parent voices.
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Sliwinski, Samantha K., Gooding, Holly, de Ferranti, Sarah, Mackie, Thomas I., Shah, Supriya, Saunders, Tully, and Leslie, Laurel K.
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ACADEMIC medical centers ,BLOOD testing ,CARDIOLOGY ,CONCEPTUAL structures ,DECISION making ,DIET ,DRUGS ,EXERCISE ,HEALTH care teams ,INTERVIEWING ,LISTENING ,RESEARCH methodology ,MEDICAL care ,PATIENT compliance ,PEER counseling ,HEALTH self-care ,QUALITATIVE research ,LIFESTYLES ,PARENT attitudes ,HEALTH care reminder systems ,FAMILIAL hypercholesterolemia ,PSYCHOLOGY - Abstract
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]
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- 2017
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33. Lack of age-appropriate reference intervals causing potentially missed alerts in clinical reports of dyslipidemia.
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Sena, Cecilia, Ohene-Adjei, Michael, Deng, Shuliang, Patibandla, Nandan, May, Benjamin, de Ferranti, Sarah D., Starc, Thomas J., and Thaker, Vidhu V.
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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]
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- 2023
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34. Variation in the management of Kawasaki disease
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Dionne, Audrey, Burgner, David, De Ferranti, Sarah, Singh-Grewal, Davinder, Newburger, Jane, and Dahdah, Nagib
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Intravenous immunoglobulin (IVIG) reduces coronary aneurysms in patients with Kawasaki disease (KD), but additional management options remain challenging, with no generalisable evidence-based recommendations. We performed a survey of 724 physicians from 73 countries to assess variation in practice. IVIG was the preferred initial treatment by 659 (91%) of respondents. Criteria for adjunctive primary treatment varied considerably and definitions of IVIG resistance varied markedly by geographical continent, Human Development Index tiers and medical specialty. A second dose of IVIG was used most often for patients with coronary aneurysm non-responsive to initial treatment (572, 79%), but corticosteroids (379, 52%) and tumour necrosis factor alpha inhibitors (208, 29%) were also frequently used. Our findings highlight the need for international collaborative efforts to optimise management of patients with KD worldwide.
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- 2020
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35. Prevalence and Predictors of Cholesterol Screening, Awareness, and Statin Treatment Among US Adults With Familial Hypercholesterolemia or Other Forms of Severe Dyslipidemia (1999–2014)
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Bucholz, Emily M., Rodday, Angie Mae, Kolor, Katherine, Khoury, Muin J., and de Ferranti, Sarah D.
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Supplemental Digital Content is available in the text.
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- 2018
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36. Heart Disease and Stroke Statistics—2018 Update: A Report From the American Heart Association
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Benjamin, Emelia J., Virani, Salim S., Callaway, Clifton W., Chamberlain, Alanna M., Chang, Alexander R., Cheng, Susan, Chiuve, Stephanie E., Cushman, Mary, Delling, Francesca N., Deo, Rajat, de Ferranti, Sarah D., Ferguson, Jane F., Fornage, Myriam, Gillespie, Cathleen, Isasi, Carmen R., Jiménez, Monik C., Jordan, Lori Chaffin, Judd, Suzanne E., Lackland, Daniel, Lichtman, Judith H., Lisabeth, Lynda, Liu, Simin, Longenecker, Chris T., Lutsey, Pamela L., Mackey, Jason S., Matchar, David B., Matsushita, Kunihiro, Mussolino, Michael E., Nasir, Khurram, O’Flaherty, Martin, Palaniappan, Latha P., Pandey, Ambarish, Pandey, Dilip K., Reeves, Mathew J., Ritchey, Matthew D., Rodriguez, Carlos J., Roth, Gregory A., Rosamond, Wayne D., Sampson, Uchechukwu K.A., Satou, Gary M., Shah, Svati H., Spartano, Nicole L., Tirschwell, David L., Tsao, Connie W., Voeks, Jenifer H., Willey, Joshua Z., Wilkins, John T., Wu, Jason HY., Alger, Heather M., Wong, Sally S., and Muntner, Paul
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- 2018
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37. Obesity, Hypertension, and Dyslipidemia in Childhood Are Key Modifiable Antecedents of Adult Cardiovascular Disease
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Turer, Christy B., Brady, Tammy M., and de Ferranti, Sarah D.
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- 2018
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38. Adolescent Perceptions of Cholesterol Screening Results: “Young Invincibles” or Developing Adults?
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Gooding, Holly C., Sheldrick, R. Christopher, Leslie, Laurel K., Shah, Supriya, de Ferranti, Sarah D., and Mackie, Thomas I.
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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]
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- 2016
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39. Stakeholders’ Perspectives on Stakeholder-engaged Research (SER)
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Mackie, Thomas I., Sheldrick, Radley C., de Ferranti, Sarah D., Saunders, Tully, Rojas, Erick G., and Leslie, Laurel K.
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- 2017
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40. Nutrition in Children and Adolescents
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Corkins, Mark R., Daniels, Stephen R., de Ferranti, Sarah D., Golden, Neville H., Kim, Jae H., Magge, Sheela N., and Schwarzenberg, Sarah Jane
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Nutrition is a critical factor for appropriate child and adolescent development. Appropriate nutrition changes according to age. Nutrition is an important element for prevention of disease development, especially for chronic diseases. Many children and adolescents live in environments that do not promote optimum nutrition. Families must work to provide improved food environments to encourage optimum nutrition. Early primordial prevention of risk factors for chronic disease, such as cardiovascular disease, is important, and dietary habits established early may be carried through adult life.
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- 2016
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41. Cardiovascular Consequences of Childhood Secondhand Tobacco Smoke Exposure: Prevailing Evidence, Burden, and Racial and Socioeconomic Disparities: A Scientific Statement From the American Heart Association
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Raghuveer, Geetha, White, David A., Hayman, Laura L., Woo, Jessica G., Villafane, Juan, Celermajer, David, Ward, Kenneth D., de Ferranti, Sarah D., and Zachariah, Justin
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- 2016
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42. Wearable Biosensors in Pediatric Cardiovascular Disease
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Tandon, Animesh and de Ferranti, Sarah D.
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- 2019
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43. Drug Therapy for Hypercholesterolemia and Dyslipidemia.
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Munoz, Ricardo, Schmitt, Carol G., Roth, Stephen J., da Cruz, Eduardo, and de Ferranti, Sarah D.
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The initial and primary treatment of abnormal lipid levels (Table 13-1) in children is to change diet and activity levels. Pharmacological treatment of lipid disorders is used according to guidelines published in 1992 (Table 13-2). In adults, treatment cutpoints and goals for therapy are adjusted based on high-risk populations and in the presence of other cardiovascular (CV) risk factors; a similar approach is being taken in pediatrics (Table 13-3). New pediatric lipid guidelines are being developed and will likely reflect this type of thinking. Although atherosclerosis is known to begin in childhood, extensive outcome data are lacking in pediatrics, and parental and/or patient preferences are usually included in the decisionmaking process. [ABSTRACT FROM AUTHOR]
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- 2008
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44. Chapter 60: Hyperlipidemia and Cardiovascular Disease Risk.
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de Ferranti, Sarah and Neufeld, Ellis
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Chapter 60 of the book "Nutrition in Pediatrics: Basic Science & Clinical Applications" is presented. It presents evidence supporting the early onset of atherosclerosis in chilhood, including new thinking about pathophysiology and evidence of preclinical disease by noninvasive testing. The pediatric origins of adult atherosclerosis is discussed. It explores the relation of C-reactive protein to pediatric lipid disorders and cardiac risk.
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- 2008
45. Chapter 40: Complications of Obesity: Metabolic Syndrome.
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Osganian, Stavroula K. and de Ferranti, Sarah
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Chapter 40 of the book "Nutrition in Pediatrics: Basic Science & Clinical Applications" is presented. The chapter offers information on obesity-associated metabolic abnormalities known as metabolic syndrome. Adults with metabolic syndrome have significantly increased risk for developing type 2 diabetes and morbidity and mortality from cardiovascular disease (CVD).
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- 2008
46. Abstract 10745: Return to Play After Covid-19 Infection: Cardiac Imaging Findings in Youth
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Yin, Suellen M, bachman, jennifer, Dionne, Audrey, Friedman, Kevin G, Newburger, Jane W, Gauthier, Naomi, Shafer, Keri M, Castellanos, Daniel A, Baker, Annette L, Atwood, Sarah, Burke, Patricia, Alexander, Mark E, and De Ferranti, Sarah D
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Introduction:SARS CoV2 has been associated with acute and subacute myocarditis. Screening after COVID-19 has been recommended prior to returning to physical activity. The yield of such investigations, particularly in youth, remains unknown.Methods:Retrospective chart review was performed of patients evaluated in a cardiology clinic for return to play (RTP) after COVID-19 between 01/20 - 06/21. Iteratively updated guidance for testing was provided to ambulatory providers. Patients with MIS-C were excluded. We reviewed demographics, clinical evaluation, and cardiac imaging.Results:Of 429 patients (age 16.3 ± 2.7 [range 8-21] yrs, 44% male) evaluated including 9 with pre-existing heart disease, 318 underwent echocardiograms and 23 also had cardiac MRI. Cardiac MRI was performed for abnormal screening ECG or echocardiogram or cardiovascular symptoms. New echocardiographic abnormalities were detected in 69 patients: 4 were possibly related to COVID-19 infection, 3 were new structural heart disease (2 anomalous coronary, 1 bicuspid aortic valve), and 62 were normal variants or trivial findings. New ventricular dysfunction (right or left, qualitative dysfunction or LV ejection fraction [EF] <55%) was noted in 11 patients (LV EF mean 52.7 ± 2.6%, minimum 46%). Eight underwent cardiac MRI confirming trivial to no ventricular dysfunction and none of the 23 MRIs demonstrated late gadolinium enhancement or met Lake Louise criteria for myocarditis. All patients without new diagnoses of structural heart disease were ultimately deemed safe to return to previous levels of physical activity.Conclusions:Cardiovascular evaluation for RTP of youth after COVID-19 demonstrated mild transient abnormalities and incidental findings, but no diagnoses of myocarditis. These data suggest early concerns about subclinical myocarditis may have been overestimated. Future studies should consider the costs and benefits of current society algorithms for such evaluations.
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- 2022
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47. Young adult and parent stakeholder perspectives on participation in patient-centered comparative effectiveness research
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Saunders, Tully, Mackie, Thomas I, Shah, Supriya, Gooding, Holly, de Ferranti, Sarah D, and Leslie, Laurel K
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Aim:Explore perspectives of adolescent and young adult (AYA) and parent stakeholders regarding their engagement in comparative effectiveness research (CER) evaluating cholesterol screening and treatment strategies for 17–21 year olds. Methods:All nine AYAs and parent stakeholders participating in a 20-member panel of AYAs, parents and professionals (i.e., clinicians, researchers, policy makers, payers), completed a quantitative survey and a semistructured interview at the completion of the core CER study. Results & Conclusion:AYAs and parents stakeholders emphasized the role of power differentials regarding shared knowledge, relationships and trust, and logistics. To mitigate power differentials, stakeholders recommended more materials, clearer definition of roles and in-person meetings. Perceived positive outcomes included diversity of perspectives provided, better understanding their own health and decision-making and improving CER.
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- 2016
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48. Long-Term Risk of Atherosclerotic Cardiovascular Disease in US Adults With the Familial Hypercholesterolemia Phenotype
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Perak, Amanda M., Ning, Hongyan, de Ferranti, Sarah D., Gooding, Holly C., Wilkins, John T., and Lloyd-Jones, Donald M.
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Supplemental Digital Content is available in the text.
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- 2016
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49. The Role of Proprotein Convertase Subtilisin/Kexin Type 9 in Nephrotic Syndrome-Associated Hypercholesterolemia
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Haas, Mary E., Levenson, Amy E., Sun, Xiaowei, Liao, Wan-Hui, Rutkowski, Joseph M., de Ferranti, Sarah D., Schumacher, Valerie A., Scherer, Philipp E., Salant, David J., and Biddinger, Sudha B.
- Abstract
Supplemental Digital Content is available in the text.
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- 2016
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50. Effect of Leptin Replacement on PCSK9 in ob/obMice and Female Lipodystrophic Patients
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Levenson, Amy E., Haas, Mary E., Miao, Ji, Brown, Rebecca J., de Ferranti, Sarah D., Muniyappa, Ranganath, and Biddinger, Sudha B.
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Leptin treatment has beneficial effects on plasma lipids in patients with lipodystrophy, but the underlying mechanism is unknown. Proprotein convertase subtilisin/kexin type 9 (PCSK9) decreases low-density lipoprotein (LDL) clearance, promotes hypercholesterolemia, and has recently emerged as a novel therapeutic target. To determine the effect of leptin on PCSK9, we treated male and female ob/obmice with leptin for 4 days via sc osmotic pumps (∼24 μg/d). Leptin reduced body weight and food intake in all mice, but the effects of leptin on plasma PCSK9 and lipids differed markedly between the sexes. In male mice, leptin suppressed PCSK9 but had no effect on plasma triglycerides or cholesterol. In female mice, leptin suppressed plasma triglycerides and cholesterol but had no effect on plasma PCSK9. In parallel, we treated female lipodystrophic patients (8 females, ages 5–23 y) with sc metreleptin injections (∼4.4 mg/d) for 4–6 months. In this case, leptin reduced plasma PCSK9 by 26% (298 ± 109 vs 221 ± 102 ng/mL; n = 8; P= .008), and the change in PCSK9 was correlated with a decrease in LDL cholesterol (r2= 0.564, P= .03). In summary, in leptin-deficient ob/obmice, the effects of leptin on PCSK9 and plasma lipids appeared to be independent of one another and strongly modified by sex. On the other hand, in lipodystrophic females, leptin treatment reduced plasma PCSK9 in parallel with LDL cholesterol.
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- 2016
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