Derivation and validation of SIDIAP-FHP score: A new risk model predicting cardiovascular disease in familial hypercholesterolemia phenotype

Assessment of individual cardiovascular risk, distinguishing primary and secondary prevention, would improve the clinical management of the population with familial hypercholesterolemia. We aimed to develop and validate two risk functions to predict incident and recurrent atherosclerotic cardiovascu...

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Veröffentlicht in:Atherosclerosis 2020-01, Vol.292, p.42-51
Hauptverfasser: Ramos, Rafel, Masana, Luís, Comas-Cufí, Marc, García-Gil, Maria, Martí-Lluch, Ruth, Ponjoan, Anna, Plana, Núria, Alves-Cabratosa, Lia, Marrugat, Jaume, Elosua, Roberto, Dégano, Irene R., Gomez-Marcos, Mauel A., Zamora, Alberto
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container_end_page 51
container_issue
container_start_page 42
container_title Atherosclerosis
container_volume 292
creator Ramos, Rafel
Masana, Luís
Comas-Cufí, Marc
García-Gil, Maria
Martí-Lluch, Ruth
Ponjoan, Anna
Plana, Núria
Alves-Cabratosa, Lia
Marrugat, Jaume
Elosua, Roberto
Dégano, Irene R.
Gomez-Marcos, Mauel A.
Zamora, Alberto
description Assessment of individual cardiovascular risk, distinguishing primary and secondary prevention, would improve the clinical management of the population with familial hypercholesterolemia. We aimed to develop and validate two risk functions to predict incident and recurrent atherosclerotic cardiovascular disease (ASCVD) in a primary care-based population with familial hypercholesterolemia phenotype (FHP), and to compare their predictive capacity with that of the SpAnish Familial hypErcHolEsterolemiA cohoRT (SAFEHEART) risk equation (SAFEHEART-RE). Data from the Catalan primary care system database (SIDIAP) of patients ≥18 years old with FHP in 2006–2013 were used to develop and validate two risk functions to predict incident and recurrent ASCVD. A validation dataset was also used to compare the model predictive capacity to that of SAFEHEART-RE. The new model (SIDIAP-FHP) included age, diabetes, smoking, sex (male), hypertension, and baseline low-density lipoprotein cholesterol in the primary prevention cohort and age, diabetes, smoking, and disease characteristics (progressive, recent, polyvascular, or included myocardial infarction) in the secondary prevention cohort. The models demonstrated a fair fit: C-Statistic: 0.71 (95%CI:0.68–0.75) in primary prevention and 0.65 (95%CI:0.60–0.70) in secondary prevention (higher than that of SAFEHEART-RE: 0.64 [95%CI:0.60–0.68] and 0.55 [95%CI:0.51–0.59], respectively; both p 
doi_str_mv 10.1016/j.atherosclerosis.2019.10.016
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We aimed to develop and validate two risk functions to predict incident and recurrent atherosclerotic cardiovascular disease (ASCVD) in a primary care-based population with familial hypercholesterolemia phenotype (FHP), and to compare their predictive capacity with that of the SpAnish Familial hypErcHolEsterolemiA cohoRT (SAFEHEART) risk equation (SAFEHEART-RE). Data from the Catalan primary care system database (SIDIAP) of patients ≥18 years old with FHP in 2006–2013 were used to develop and validate two risk functions to predict incident and recurrent ASCVD. A validation dataset was also used to compare the model predictive capacity to that of SAFEHEART-RE. The new model (SIDIAP-FHP) included age, diabetes, smoking, sex (male), hypertension, and baseline low-density lipoprotein cholesterol in the primary prevention cohort and age, diabetes, smoking, and disease characteristics (progressive, recent, polyvascular, or included myocardial infarction) in the secondary prevention cohort. The models demonstrated a fair fit: C-Statistic: 0.71 (95%CI:0.68–0.75) in primary prevention and 0.65 (95%CI:0.60–0.70) in secondary prevention (higher than that of SAFEHEART-RE: 0.64 [95%CI:0.60–0.68] and 0.55 [95%CI:0.51–0.59], respectively; both p &lt; 0.01). The Brier scores obtained with the SIDIAP-FHP score were significantly lower than that obtained with SAFEHEART-RE in both the primary and secondary prevention cohorts. The SIDIAP-FHP score provides accurate ASCVD risk estimates for primary and secondary prevention in the FHP population, with better predictive capacity than that of SAFEHEART-RE in this general population, especially in persons with previous ASCVD. [Display omitted] •Familial hypercholesterolemia phenotype entails high risk of atherosclerotic events.•A patient-tailored assessment of risk may help achieve optimal treatment.•The SIDIAP-FHP score is useful in general population or primary care settings.•Clinicians may use the SIDIAP-FHP to prioritize referrals to lipids-clinic specialists.</description><identifier>ISSN: 0021-9150</identifier><identifier>EISSN: 1879-1484</identifier><identifier>DOI: 10.1016/j.atherosclerosis.2019.10.016</identifier><identifier>PMID: 31759248</identifier><language>eng</language><publisher>Ireland: Elsevier B.V</publisher><subject>Aged ; Atherosclerosis ; Atherosclerosis - diagnosis ; Atherosclerosis - etiology ; Cardiovascular diseases ; Cohort Studies ; Familial hypercholesterolemia ; Female ; Humans ; Hyperlipoproteinemia Type II - complications ; Hyperlipoproteinemia Type II - genetics ; Male ; Middle Aged ; Models, Statistical ; Phenotype ; Prevention ; Retrospective Studies ; Risk assessment ; Risk Assessment - methods</subject><ispartof>Atherosclerosis, 2020-01, Vol.292, p.42-51</ispartof><rights>2019</rights><rights>Copyright © 2019. 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We aimed to develop and validate two risk functions to predict incident and recurrent atherosclerotic cardiovascular disease (ASCVD) in a primary care-based population with familial hypercholesterolemia phenotype (FHP), and to compare their predictive capacity with that of the SpAnish Familial hypErcHolEsterolemiA cohoRT (SAFEHEART) risk equation (SAFEHEART-RE). Data from the Catalan primary care system database (SIDIAP) of patients ≥18 years old with FHP in 2006–2013 were used to develop and validate two risk functions to predict incident and recurrent ASCVD. A validation dataset was also used to compare the model predictive capacity to that of SAFEHEART-RE. The new model (SIDIAP-FHP) included age, diabetes, smoking, sex (male), hypertension, and baseline low-density lipoprotein cholesterol in the primary prevention cohort and age, diabetes, smoking, and disease characteristics (progressive, recent, polyvascular, or included myocardial infarction) in the secondary prevention cohort. 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[Display omitted] •Familial hypercholesterolemia phenotype entails high risk of atherosclerotic events.•A patient-tailored assessment of risk may help achieve optimal treatment.•The SIDIAP-FHP score is useful in general population or primary care settings.•Clinicians may use the SIDIAP-FHP to prioritize referrals to lipids-clinic specialists.</description><subject>Aged</subject><subject>Atherosclerosis</subject><subject>Atherosclerosis - diagnosis</subject><subject>Atherosclerosis - etiology</subject><subject>Cardiovascular diseases</subject><subject>Cohort Studies</subject><subject>Familial hypercholesterolemia</subject><subject>Female</subject><subject>Humans</subject><subject>Hyperlipoproteinemia Type II - complications</subject><subject>Hyperlipoproteinemia Type II - genetics</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Models, Statistical</subject><subject>Phenotype</subject><subject>Prevention</subject><subject>Retrospective Studies</subject><subject>Risk assessment</subject><subject>Risk Assessment - methods</subject><issn>0021-9150</issn><issn>1879-1484</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkcFu1DAQhi0EokvhFZAvSFyy2EkcO0gcVi1tV6rUSoWz5dgTdhYnDnZ2Ud-hD12vtvTQExdb1vePZ-b_CfnE2ZIz3nzZLs28gRiS9YcT07JkvM1smekrsuBKtgWvVf2aLBgredFywU7Iu5S2jLFacvWWnFRciras1YI8nEPEvZkxjNSMju6NR3d8hp7erc_Xq9vi4uqWJhsifKUrOsJfGjH9pkNw4OkUwaGdcfxFrYkOw94ku_MmUocJTAKKI-3NgB6Np5v7CaLdBA9pzuN7GNDQaQNjmDN5T970xif48HSfkp8X33-cXRXXN5frs9V1YSvVzoXhQgimmkZ2nZIgAFgjoDOdsJIbMLVifdc62VadrCvOJWNdI5SUqqwEY646JZ-P_04x_NnlUfSAyYL3ZoSwS7rM_jTZVVlm6bej1GazU4ReTxEHE-81Z_oQiN7qF4HoQyAHnGmu__jUatcN4J6r_yWQBZdHAeSF9whRJ4sw2uxqBDtrF_A_Wz0Co0qoWg</recordid><startdate>202001</startdate><enddate>202001</enddate><creator>Ramos, Rafel</creator><creator>Masana, Luís</creator><creator>Comas-Cufí, Marc</creator><creator>García-Gil, Maria</creator><creator>Martí-Lluch, Ruth</creator><creator>Ponjoan, Anna</creator><creator>Plana, Núria</creator><creator>Alves-Cabratosa, Lia</creator><creator>Marrugat, Jaume</creator><creator>Elosua, Roberto</creator><creator>Dégano, Irene R.</creator><creator>Gomez-Marcos, Mauel A.</creator><creator>Zamora, Alberto</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-7970-5537</orcidid><orcidid>https://orcid.org/0000-0002-0789-4954</orcidid><orcidid>https://orcid.org/0000-0001-8146-5288</orcidid><orcidid>https://orcid.org/0000-0001-6907-0654</orcidid><orcidid>https://orcid.org/0000-0001-8235-0095</orcidid><orcidid>https://orcid.org/0000-0003-3320-554X</orcidid></search><sort><creationdate>202001</creationdate><title>Derivation and validation of SIDIAP-FHP score: A new risk model predicting cardiovascular disease in familial hypercholesterolemia phenotype</title><author>Ramos, Rafel ; 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We aimed to develop and validate two risk functions to predict incident and recurrent atherosclerotic cardiovascular disease (ASCVD) in a primary care-based population with familial hypercholesterolemia phenotype (FHP), and to compare their predictive capacity with that of the SpAnish Familial hypErcHolEsterolemiA cohoRT (SAFEHEART) risk equation (SAFEHEART-RE). Data from the Catalan primary care system database (SIDIAP) of patients ≥18 years old with FHP in 2006–2013 were used to develop and validate two risk functions to predict incident and recurrent ASCVD. A validation dataset was also used to compare the model predictive capacity to that of SAFEHEART-RE. The new model (SIDIAP-FHP) included age, diabetes, smoking, sex (male), hypertension, and baseline low-density lipoprotein cholesterol in the primary prevention cohort and age, diabetes, smoking, and disease characteristics (progressive, recent, polyvascular, or included myocardial infarction) in the secondary prevention cohort. The models demonstrated a fair fit: C-Statistic: 0.71 (95%CI:0.68–0.75) in primary prevention and 0.65 (95%CI:0.60–0.70) in secondary prevention (higher than that of SAFEHEART-RE: 0.64 [95%CI:0.60–0.68] and 0.55 [95%CI:0.51–0.59], respectively; both p &lt; 0.01). The Brier scores obtained with the SIDIAP-FHP score were significantly lower than that obtained with SAFEHEART-RE in both the primary and secondary prevention cohorts. The SIDIAP-FHP score provides accurate ASCVD risk estimates for primary and secondary prevention in the FHP population, with better predictive capacity than that of SAFEHEART-RE in this general population, especially in persons with previous ASCVD. [Display omitted] •Familial hypercholesterolemia phenotype entails high risk of atherosclerotic events.•A patient-tailored assessment of risk may help achieve optimal treatment.•The SIDIAP-FHP score is useful in general population or primary care settings.•Clinicians may use the SIDIAP-FHP to prioritize referrals to lipids-clinic specialists.</abstract><cop>Ireland</cop><pub>Elsevier B.V</pub><pmid>31759248</pmid><doi>10.1016/j.atherosclerosis.2019.10.016</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0001-7970-5537</orcidid><orcidid>https://orcid.org/0000-0002-0789-4954</orcidid><orcidid>https://orcid.org/0000-0001-8146-5288</orcidid><orcidid>https://orcid.org/0000-0001-6907-0654</orcidid><orcidid>https://orcid.org/0000-0001-8235-0095</orcidid><orcidid>https://orcid.org/0000-0003-3320-554X</orcidid></addata></record>
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subjects Aged
Atherosclerosis
Atherosclerosis - diagnosis
Atherosclerosis - etiology
Cardiovascular diseases
Cohort Studies
Familial hypercholesterolemia
Female
Humans
Hyperlipoproteinemia Type II - complications
Hyperlipoproteinemia Type II - genetics
Male
Middle Aged
Models, Statistical
Phenotype
Prevention
Retrospective Studies
Risk assessment
Risk Assessment - methods
title Derivation and validation of SIDIAP-FHP score: A new risk model predicting cardiovascular disease in familial hypercholesterolemia phenotype
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