Prognosis of Patients with Familial Hypercholesterolemia After Acute Coronary Syndromes
Background—Patients with heterozygous familial hypercholesterolemia (FH) and coronary heart disease have high mortality rates. However, in an era of high-dose statin prescription after acute coronary syndrome (ACS), the risk of recurrent coronary and cardiovascular events associated with FH might be mitigated. We compared coronary event rates between patients with and without FH after ACS.
Methods—We studied 4,534 patients with ACS enrolled in a multicenter prospective cohort study in Switzerland between 2009 and 2013, individually screened for FH based on clinical criteria according to three definitions: (1) the American Heart Association (AHA) (2) the Simon Broome and (3) the Dutch Lipid Clinic. We used Cox proportional models to assess the one-year risk of first recurrent coronary events defined as coronary death or myocardial infarction, and adjusted for age, sex, body mass index, smoking, hypertension, diabetes mellitus, existing cardiovascular disease, high-dose statin at discharge, attendance to cardiac rehabilitation, and the GRACE risk score for severity of ACS.
Results—At one-year follow-up, 153 (3.4%) patients had died, including 104 (2.3%) from fatal myocardial infarction. A further 113 (2.5%) patients experienced non-fatal myocardial infarction. The prevalence of FH was 2.5% using the AHA definition, 5.5% using Simon Broome, and 1.6% using the Dutch Lipid Clinic definition. Compared to patients without FH, the risk of coronary event recurrence after ACS was similar in patients with FH in unadjusted analyses, although patients with FH were more than 10 years younger. However, after multivariable adjustment including age, the risk was higher in patients with FH than without, with an adjusted hazard ratio (HR) of 2.46 (95% CI 1.07-5.65, p=0.034) for the AHA definition, 2.73 (95% CI 1.46-5.11, p=0.002) for the Simon Broome, and 3.53 (95% CI 1.26-9.94, p=0.017) for the Dutch Lipid Clinic definition. Depending on which clinical definition of FH was used, between 94.5% to 99.1% of patients with FH were discharged on statins, and between 74.0% to 82.3% on high-dose statins.
Conclusions—Patients with FH and ACS have more than a two-fold adjusted risk of coronary event recurrence within the first year following discharge than patients without FH, despite the widespread use of high-intensity statins.
- familial hypercholesterolemia
- acute coronary syndrome
- secondary prevention
- cardiovascular events
- Received April 15, 2016.
- Revision received July 7, 2016.
- Accepted July 20, 2016.