Among patients with extremely high LDL-C levels (≥400 mg/dL), deaths occurred in 20.8% and major adverse cardiovascular events in 16.2% over the follow-up period.
Cohort (n=456)
Patients with extremely high LDL-C (≥ 400 mg/dL) have a higher than anticipated prevalence and experience significant rates of mortality and major adverse cardiovascular events.
Abstract Background Although homozygous familial hypercholesterolemia (HoFH) is a rare genetic disease characterized by very high levels of low-density lipoprotein cholesterol (LDL-C), with reported prevalence of approximately 1:250,000, there remains a concern for undiagnosed or untreated patients with extremely high LDL-C levels (≥ 400 mg/dL), especially in the contemporary era. Objectives The aim of the study was to describe the clinical characteristics and cardiovascular outcomes of this population over time in Kaiser Permanente Northern California, a large integrated health care system with more than 4.5 million patients. Methods We used the electronic health record to identify patients with at least one LDL-C level ≥ 400 mg/dL from 2010 through 2024 with ages ranging from 10 years and above. We collected clinical data on these patients. To avoid patients with spurious high LDL-C levels, we excluded patients who had an LDL-C level ≤100 mg/dL at any time. Results A total of 456 patients meeting eligibility criteria were identified, with an approximate annual incidence of 1 new case per 21,905 patients per year. The median age was 51 years (interquartile range IQR: 37-61). Of these patients, 251 (55.0%) were women, 205 (45.0%) white, 93 (20.4%) Asian, 62 (13.6%) black, and 96 (21.1%) identified as multiple-race or were declined/missing; 75 (16.5%) of patients identified as Latino. Deaths were reported in 95 patients over the follow-up period (20.8%; 95% CI: 17.2% – 24.9%) and major adverse cardiovascular events were observed in 74 patients (16.2%; 95% CI: 13.0% – 19.9%). Conclusions Identification of patients with extremely high LDL-C (≥400 mg/dL) in a health care system may provide opportunities to improve diagnosis and treatment of HoFH. Further, prevalence of extremely high LDL-C (≥ 400 mg/dL) may be much higher than previously anticipated. Leveraging large healthcare system comprehensive data, further genetics and therapeutic interventions and secondary causes will be explored to provide unique insights.
Rana et al. (Mon,) conducted a cohort in Extremely high LDL-C levels (n=456). Extremely high LDL-C levels (≥400 mg/dL) was evaluated on Deaths (95% CI 17.2-24.9). Among patients with extremely high LDL-C levels (≥400 mg/dL), deaths occurred in 20.8% and major adverse cardiovascular events in 16.2% over the follow-up period.
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