title: Cardiovascular disease in patients with genotyped familial hypercholesterolemia in Norway during 1994-2009, a registry study.
abstract: Background Familial hypercholesterolaemia increases the risk for cardiovascular disease. The primary aim of the present study was to describe sex differences in incidence and prevalence of cardiovascular disease leading to hospitalisation in a complete cohort of genotyped familial hypercholesterolaemia patients. Design and methods In this registry study data on 5538 patients with verified genotyped familial hypercholesterolaemia were linked to data on all Norwegian cardiovascular disease hospitalisations, and hospitalisations due to pre-eclampsia/eclampsia, congenital heart defects and diabetes. Results During 1994-2009 a total of 1411 of familial hypercholesterolaemia patients were hospitalised, and ischaemic heart disease was reported in 90% of them. Mean (SD) age at first hospitalisation and first re-hospitalisation was 45.1 (16.5) and 47.6 (16.3) years, respectively, with no sex differences ( P = 0.66 and P = 0.93, respectively). More men (26.9%) than women (24.1%) with familial hypercholesterolaemia were hospitalised ( P = 0.02). The median (25th-75th percentile) number of hospital admissions was four (two to seven) per familial hypercholesterolaemia patient, with no sex differences ( P = 0.87). Despite having familial hypercholesterolaemia at the time of hospitalisation, the diagnosis of familial hypercholesterolaemia was registered in only 45.7% of the patients at discharge. Conclusion Most cardiovascular disease hospitalisations were due to ischaemic heart disease. Familial hypercholesterolaemia patients were first time hospitalised at age 45.1 years, with no significant sex differences in age, which are important novel findings. The awareness and registration of the familial hypercholesterolaemia diagnosis during the hospital stays were disturbingly low.
authors: 1 The Lipid Clinic, Oslo University Hospital Rikshospitalet, Norway.
authors: Liv
authors: L
authors: Mundal
authors: 2 Department of Nutrition, University of Oslo, Norway.|3 Oslo Centre for Biostatistics and Epidemiology, University of Oslo, Norway.
authors: Marit B
authors: MB
authors: Veierød
authors: 4 Department of Health Research, SINTEF Technology and Society, Norway.
authors: Thomas
authors: T
authors: Halvorsen
authors: 2 Department of Nutrition, University of Oslo, Norway.|5 National Advisory Unit for Familial Hypercholesterolemia, Oslo University Hospital, Norway.
authors: Kirsten B
authors: KB
authors: Holven
authors: 2 Department of Nutrition, University of Oslo, Norway.
authors: Leiv
authors: L
authors: Ose
authors: 2 Department of Nutrition, University of Oslo, Norway.|6 Department of Hematology, Oslo University Hospital Rikshospitalet, Norway.
authors: Per Ole
authors: PO
authors: Iversen
authors: 7 Department of Global Public Health and Primary Care, University of Bergen, Norway.|8 Department of Health Registries, Norwegian Institute of Public Health, Norway.
authors: Grethe S
authors: GS
authors: Tell
authors: 9 Unit for Cardiac and Cardiovascular Genetics, Oslo University Hospital Ullevaal, Norway.
authors: Trond P
authors: TP
authors: Leren
authors: 1 The Lipid Clinic, Oslo University Hospital Rikshospitalet, Norway.|2 Department of Nutrition, University of Oslo, Norway.
authors: Kjetil
authors: K
authors: Retterstøl
mesh_terms: D000328:Adult; D002318:Cardiovascular Diseases / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics; D005260:Female; D005838:Genotype; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009664:Norway / Q000453:epidemiology; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors
keywords: Familial hypercholesterolaemia; cardiovascular disease; registries