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Circulation. 2017 May 30;135(22):2133-2144. doi: 10.1161/CIRCULATIONAHA.116.024541. Epub 2017 Mar 8.

Predicting Cardiovascular Events in Familial Hypercholesterolemia: The SAFEHEART Registry (Spanish Familial Hypercholesterolemia Cohort Study).

Author information

1
From Cardiology Department, Hospital Clínico San Carlos, IDISSC, Universidad Complutense, Madrid, Spain (L.P.d.I.); Fundación Hipercolesterolemia Familiar, Madrid, Spain (L.P.d.I., R. Alonso, N.M., A.S., P.M.); Clínica las Condes, Santiago, Chile (R. Alonso); Department of Epidemiology, Madrid Health Authority, Madrid, Spain (N.M.); Clinical Epidemiology Unit, Servicio de Medicina Preventiva, Instituto de Investigación Sanitaria San Carlos, Universidad Complutense, Madrid, Spain (C.F.-P.); Department of Internal Medicine, Hospital Virgen del Rocío, Sevilla, Spain (O.M.); Department of Internal Medicine, Hospital Abente y Lago, A Coruña, Spain (J.L.D.-D.); Cardiology Department, Hospital del Tajo, Universidad Alfonso X el Sabio, Madrid, Spain (A.S.); Lipids and Atherosclerosis Unit, Instituto Maimónides de Investigación Biomédica de Córdoba/Reina Sofia University Hospital/University of Cordoba, Cordoba, Spain (F.F.-J.); Department of Internal Medicine, Fundación Jiménez Díaz, IIS, Madrid, Spain (R.d.A.); Department of Endocrinology, Hospital Clinic, Barcelona, Spain (D.Z.); Department of Internal Medicine, Hospital Universitario de Elche, Alicante, Spain (M.P.); Department of Internal Medicine, Hospital de Vega Baja, Orihuela, Alicante, Spain (J.M.C.); Department of Internal Medicine, Hospital de Terrassa, Barcelona, Spain (M.M.); Department of Internal Medicine, Hospital de Ciudad Real, Spain (J.G.); Department of Internal Medicine, Hospital de San Pedro, Logroño, Spain (A.B.); Department of Internal Medicine, Hospital San Pedro de Alcántara, Cáceres, Spain (J.F.S.M.-T.); Instituto Catalán Ciencias Cardiovasculares, Instituto de Investigación Biomédica-Sant Pau, Barcelona, Spain (T.P., L.B.); Department of Endocrinology, Hospital Universitario de Lugo, Spain (R. Argueso); Department of Internal Medicine, Hospital Universitariode Salamanca, Spain (J.P.M.-G.); Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital and Preventive Centre and Cardiology Program, Hospital Israelita Albert Einstein, Brazil (R.D.S.); and Lipid Disorders Clinic, Department of Cardiology, Royal Perth Hospital, School of Medicine and Pharmacology, University of Western Australia (G.F.W.).
2
From Cardiology Department, Hospital Clínico San Carlos, IDISSC, Universidad Complutense, Madrid, Spain (L.P.d.I.); Fundación Hipercolesterolemia Familiar, Madrid, Spain (L.P.d.I., R. Alonso, N.M., A.S., P.M.); Clínica las Condes, Santiago, Chile (R. Alonso); Department of Epidemiology, Madrid Health Authority, Madrid, Spain (N.M.); Clinical Epidemiology Unit, Servicio de Medicina Preventiva, Instituto de Investigación Sanitaria San Carlos, Universidad Complutense, Madrid, Spain (C.F.-P.); Department of Internal Medicine, Hospital Virgen del Rocío, Sevilla, Spain (O.M.); Department of Internal Medicine, Hospital Abente y Lago, A Coruña, Spain (J.L.D.-D.); Cardiology Department, Hospital del Tajo, Universidad Alfonso X el Sabio, Madrid, Spain (A.S.); Lipids and Atherosclerosis Unit, Instituto Maimónides de Investigación Biomédica de Córdoba/Reina Sofia University Hospital/University of Cordoba, Cordoba, Spain (F.F.-J.); Department of Internal Medicine, Fundación Jiménez Díaz, IIS, Madrid, Spain (R.d.A.); Department of Endocrinology, Hospital Clinic, Barcelona, Spain (D.Z.); Department of Internal Medicine, Hospital Universitario de Elche, Alicante, Spain (M.P.); Department of Internal Medicine, Hospital de Vega Baja, Orihuela, Alicante, Spain (J.M.C.); Department of Internal Medicine, Hospital de Terrassa, Barcelona, Spain (M.M.); Department of Internal Medicine, Hospital de Ciudad Real, Spain (J.G.); Department of Internal Medicine, Hospital de San Pedro, Logroño, Spain (A.B.); Department of Internal Medicine, Hospital San Pedro de Alcántara, Cáceres, Spain (J.F.S.M.-T.); Instituto Catalán Ciencias Cardiovasculares, Instituto de Investigación Biomédica-Sant Pau, Barcelona, Spain (T.P., L.B.); Department of Endocrinology, Hospital Universitario de Lugo, Spain (R. Argueso); Department of Internal Medicine, Hospital Universitariode Salamanca, Spain (J.P.M.-G.); Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital and Preventive Centre and Cardiology Program, Hospital Israelita Albert Einstein, Brazil (R.D.S.); and Lipid Disorders Clinic, Department of Cardiology, Royal Perth Hospital, School of Medicine and Pharmacology, University of Western Australia (G.F.W.). pmata@colesterolfamiliar.org leopisla@hotmail.com.

Abstract

BACKGROUND:

Although risk factors for atherosclerotic cardiovascular disease (ASCVD) in familial hypercholesterolemia (FH) have been described, models for predicting incident ASCVD have not been reported. Our aim was to use the SAFEHEART registry (Spanish Familial Hypercholesterolemia Cohort Study) to define key risk factors for predicting incident ASCVD in patients with FH.

METHODS:

SAFEHEART is a multicenter, nationwide, long-term prospective cohort study of a molecularly defined population with FH with or without previous ASCVD. Analyses to define risk factors and to build a risk prediction equation were developed, and the risk prediction equation was tested for its ability to discriminate patients who experience incident ASCVD from those who did not over time.

RESULTS:

We recruited 2404 adult patients with FH who were followed up for a mean of 5.5 years (SD, 3.2 years), during which 12 (0.5%) and 122 (5.1%) suffered fatal and nonfatal incident ASCVD, respectively. Age, male sex, history of previous ASCVD, high blood pressure, increased body mass index, active smoking, and low-density lipoprotein cholesterol and lipoprotein(a) levels were independent predictors of incident ASCVD from which a risk equation with a Harrell C index of 0.85 was derived. The bootstrap resampling (100 randomized samples) of the original set for internal validation showed a degree of overoptimism of 0.003. Individual risk was estimated for each person without an established diagnosis of ASCVD before enrollment in the registry by use of the SAFEHEART risk equation, the modified Framingham risk equation, and the American College of Cardiology/American Heart Association ASCVD Pooled Cohort Risk Equations. The Harrell C index for these models was 0.81, 0.78, and 0.8, respectively, and differences between the SAFEHEART risk equation and the other 2 were significant (P=0.023 and P=0.045).

CONCLUSIONS:

The risk of incident ASCVD may be estimated in patients with FH with simple clinical predictors. This finding may improve risk stratification and could be used to guide therapy in patients with FH.

CLINICAL TRIAL REGISTRATION:

URL: http://clinicaltrials.gov. Unique identifier: NCT02693548.

KEYWORDS:

diagnostic techniques, cardiovascular; genetics; heart diseases; hypercholesterolemia; risk assessment

[Indexed for MEDLINE]

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