Open AccessMortality Among Patients With Familial Hypercholesterolemia: A Registry‐Based Study in Norway, 1992–2010
Author(s) -
Mundal Liv,
Sarancic Mirza,
Ose Leiv,
Iversen Per Ole,
Borgan JensKristian,
Veierød Marit B.,
Leren Trond P.,
Retterstøl Kjetil
Publication year - 2014
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.114.001236
Subject(s) - medicine , standardized mortality ratio , cause of death , norwegian , population , disease , mortality rate , familial hypercholesterolemia , pediatrics , cholesterol , linguistics , philosophy , environmental health
Background Untreated patients with familial hypercholesterolemia are at increased risk of premature cardiovascular death. The primary aim of this study was to investigate whether this is also the case in the statin era. Methods and Results In this registry‐based study, 4688 male and female patients from the Unit for Cardiac and Cardiovascular Genetics ( UCCG ) Registry with verified molecular genetic diagnosis of familial hypercholesterolemia in the period 1992–2010 were linked to the Norwegian Cause of Death Registry. Standardized mortality ratios and 95% CI s were estimated. There were 113 deaths. Mean age of death was 61.1 years. Cardiovascular disease was the most common cause of death (46.0%), followed by cancer (30.1%). Compared with the Norwegian population, cardiovascular disease mortality was significantly higher in the UCCG Registry in all age groups younger than 70 years (standardized mortality ratio 2.29, 95% CI 1.65 to 3.19 in men and women combined; standardized mortality ratio 2.00, 95% CI 1.32 to 3.04 in men; standardized mortality ratio 3.03, 95% CI 1.76 to 5.21 in women). No significant differences were found in all‐cause mortality or cancer mortality. Conclusions Despite prescription of lipid‐lowering drugs, familial hypercholesterolemia patients still had significantly increased cardiovascular disease mortality compared with the general Norwegian population.