Revista Española de Cardiología (English Edition) Revista Española de Cardiología (English Edition)
Rev Esp Cardiol. 2017;70:444-50 - Vol. 70 Num.06 DOI: 10.1016/j.rec.2016.10.010

Attainment of LDL Cholesterol Treatment Goals in Children and Adolescents With Familial Hypercholesterolemia. The SAFEHEART Follow-up Registry

Adriana Saltijeral a,, Leopoldo Pérez de Isla b, Rodrigo Alonso c, Ovidio Muñiz d, José Luis Díaz-Díaz e, Francisco Fuentes f, Nelva Mata g, Raimundo de Andrés h, Gonzalo Díaz-Soto i, José Pastor j, José Miguel Pinilla k, Daniel Zambón l, Xavier Pinto m, Lina Badimón n, Pedro Mata o

a Sección de Cardiología, Hospital Universitario del Tajo, Universidad Alfonso X el Sabio, Aranjuez, Madrid, Spain
b Servicio de Cardiología, Hospital Clínico San Carlos, Universidad Complutense de Madrid, IDISSC, Madrid, Spain
c Servicio de Medicina Interna, Clínica las Condes, Santiago de Chile, Chile
d Servicio de Medicina Interna, Hospital Virgen del Rocío, Sevilla, Spain
e Servicio de Medicina Interna, Hospital Abente y Lago, A Coruña, Spain
f Unidad de Lípidos y Aterosclerosis, IMIBIC, Hospital Universitario Reina Sofía, Universidad de Córdoba, Córdoba, Spain
g Departamento de Epidemiología, Consejería de Sanidad, Comunidad de Madrid, Madrid, Spain
h Servicio de Medicina Interna, Fundación Jiménez Díaz, Madrid, Spain
i Departamento de Endocrinología, Hospital Clínico, Valladolid, Spain
j Servicio de Pediatría, Hospital General Universitario de Elche, Alicante, Spain
k Centro de Salud San Miguel de Salinas, San Miguel de Salinas, Alicante, Spain
l Clínica de Lípidos, Servicio de Endocrinología, Hospital Clínic, Barcelona, Spain
m Servicio de Medicina Interna, Hospital Universitario de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain
n Centro de Investigación Cardiovascular, Instituto Catalán de Ciencias Cardiovasculares, IIB-Sant Pau, Barcelona, Spain
o Fundación Hipercolesterolemia Familiar, Madrid, Spain

Refers to

Keywords

Familial hypercholesterolemia. Low-density lipoprotein cholesterol goals. Lipid-lowering therapy. Cardiovascular disease. Children. Adolescents. Statins.

Abstract

Introduction and objectives

Little is known about the characteristics of persons with familial hypercholesterolemia (FH) younger than 18 years, the lipid-lowering therapy used in these patients, and the lipid goals reached in real life. Our aim was to evaluate the achievement of low-density lipoprotein cholesterol (LDL-C) treatment goals in FH patients younger than 18 years enrolled in a large national registry.

Methods

We analyzed patients younger than 18 years enrolled in a large ongoing registry of molecularly-defined patients with FH in Spain. The attainment of guideline-recommended plasma LDL-C goals at entry and follow-up was analyzed in relation to the use of lipid-lowering therapy.

Results

We enrolled 392 individuals younger than 18 years. Of these, 217 were molecularly-diagnosed FH patients and had a complete follow-up. The median follow-up time was 4.69 years (interquartile range, 2.48-6.38 years), 68.2% of FH patients were on statins, and 41.5% patients had LDL-C < 130 mg/dL. Statin use was the only predictor of LDL-C goal attainment.

Conclusions

This study shows that a high proportion of FH patients younger than 18 years have high LDL-C levels and fail to achieve recommended LDL-C targets. Statin use was the only independent predictor of LDL-C goal achievement. No safety concerns were detected during follow-up. These results indicate that many FH patients are not adequately controlled and that there is still room for treatment improvement.

1885-5857/© 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved

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