title: Atherosclerosis progression in patients with autosomal dominant hypercholesterolemia in clinical practice.
abstract: BACKGROUND
  Autosomal dominant hypercholesterolemias (ADH) are associated with high risk of premature cardiovascular disease (CVD). No data on progression of atherosclerosis in ADH population in clinical practice are available.
  
  
  OBJECTIVE
  To investigate atherosclerosis progression in ADH patients and its relationship with CVD risk factors.
  
  
  METHODS
  A total of 463 patients, 279 with familial hypercholesterolemia and 184 with familial combined hyperlipidemia, were prospectively followed during a median of 36.5 months. Carotid intima-media thickness (cIMT) was assessed at baseline and at the end of the follow-up by ultrasonography.
  
  
  RESULTS
  A total of 259 patients (55.9%) showed cIMT progression and 149 (32.2%) remained within normal age-adjusted cIMT. Baseline cIMT was the variable most inversely associated with cIMT progression (B = -0.313; P < .001). Hypertension, diabetes, and smoking during follow-up were variables positively associated with progression. Patients who began statin treatment during the study period had less progression than former statin users. The 83.7% of ADH with normal baseline cIMT, absence of major CVD risk factors and non-high-density lipoprotein (HDL) cholesterol <190 mg/dL at follow-up remained with normal cIMT at the end of the study. Non-HDL cholesterol concentration reached during the follow-up was associated with cIMT only in subjects with abnormal cIMT at baseline. In this subgroup, cIMT tended to avoid progression with non-HDL cholesterol <130 mg/dL.
  
  
  CONCLUSIONS
  Atherosclerosis progression varies greatly among ADH patients. cIMT progression was inversely related to baseline cIMT and previous use of statins, and positively with age and CVD risk factors during the follow-up. Patients previously treated with statins may not be the preferred candidates for atherosclerosis regression trials. Treatment recommendations in ADH should be based on baseline risk.
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain; Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain. Electronic address: estijarauta@gmail.com.
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Jarauta
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain.
authors.forename: Rocio
authors.initials: R
authors.lastname: Mateo-Gallego
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain.
authors.forename: Ana M
authors.initials: AM
authors.lastname: Bea
authors.affiliation: Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Crespo
authors.affiliation: Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Alba
authors.initials: A
authors.lastname: Ballester
authors.affiliation: Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: M Victoria
authors.initials: MV
authors.lastname: Rubio
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain.
authors.forename: Lucia
authors.initials: L
authors.lastname: Baila-Rueda
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Calmarza
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain.
authors.forename: Ana
authors.initials: A
authors.lastname: Cenarro
authors.affiliation: Department of Vascular Medicine, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Eric
authors.initials: E
authors.lastname: de Groot
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Hospital Universitario Miguel Servet, Instituto de Investigación Sanitaria de Aragón (IISAragon), Zaragoza, Spain; Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Fernando
authors.initials: F
authors.lastname: Civeira
mesh_terms: D000328:Adult; D000367:Age Factors; D050197:Atherosclerosis / Q000175:diagnosis* / Q000188:drug therapy; D002339:Carotid Arteries / Q000000981:diagnostic imaging / Q000473:pathology*; D059168:Carotid Intima-Media Thickness; D008076:Cholesterol, HDL / Q000378:metabolism; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D006938:Hyperlipoproteinemia Type II / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D055118:Medication Adherence; D008875:Middle Aged; D011446:Prospective Studies; D012307:Risk Factors
chemical_list: D008076:Cholesterol, HDL; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors
keywords: Atherosclerosis progression; Autosomal dominant hypercholesterolemia; Cardiovascular risk; Carotid intima-media thickness; Familial combined hyperlipidemia; Familial hypercholesterolemia; Subclinical atherosclerosis