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捷克共和国和斯洛伐克杂合子家族性高胆固醇血症患者的真实 LDL-C 治疗目标达成情况:PLANET 登记研究结果。

Real-life LDL-C treatment goals achievement in patients with heterozygous familial hypercholesterolemia in the Czech Republic and Slovakia: Results of the PLANET registry.

机构信息

Third Department of Internal Medicine, General University Hospital and First Faculty of Medicine, Charles University, U Nemocnice 1, 128 08 Prague 2, Czech Republic.

Coordination Centre for Familial Hypercholesterolemia and Institute of Nutrition, Slovak Medical University, Limbova 14, 833 03 Bratislava, Slovak Republic.

出版信息

Atherosclerosis. 2018 Oct;277:355-361. doi: 10.1016/j.atherosclerosis.2018.08.008.


DOI:10.1016/j.atherosclerosis.2018.08.008
PMID:30270071
Abstract

BACKGROUND AND AIMS: Despite the high prevalence of familial hypercholesterolemia (FH) and available effective lipid-lowering therapy, most of the individuals with this disorder remain undiagnosed and undertreated. The aim of the PLANET registry was to assess the real-life attainment of low-density lipoprotein cholesterol (LDL-C) therapeutic target level in patients with heterozygous FH, to characterize prescribed lipid-lowering therapy with assessment of its efficiency according to the attainment of the target LDL-C level, and to characterize cardiovascular events observed in this patient population again in relation to LDL-C target level attainment. METHODS: PLANET registry was designed as a non-interventional, retrospective, cross-sectional, multicentre disease registry for adult patients with heterozygous FH in the Czech Republic and Slovakia. RESULTS: Overall, 1755 patients were enrolled at 32 sites specialized in FH treatment. 15.4% of patients attained the target LDL-C value. The proportion of patients with LDL-C goal achievement increased to 17.3% in the subgroup of patients receiving high-intensity statin therapy (54.6% of study population). Out of 55 patients receiving inhibitors of proprotein convertase subtilisin/kexin type 9 (PCSK9), 61.8% reached the LDL-C treatment goal. Of all cardiovascular events reported, 14.0% occurred in patients attaining the LDL-C goal, while it was 86.0% in the not-at-target group. It was documented (p=0.004) that the longer is the patient in care at the specialized FH centre, the higher is the probability that he/she will attain the target LDL-C level. CONCLUSIONS: Although target LDL-C level attainment remains relatively low, the likelihood of LDL-C goal attainment increases with duration of specialized care.

摘要

背景与目的:尽管家族性高胆固醇血症(FH)的患病率很高,且有有效的降脂治疗方法,但大多数此类患者仍未被诊断和治疗不足。PLANET 登记研究旨在评估杂合子 FH 患者的低密度脂蛋白胆固醇(LDL-C)治疗目标水平的实际达标情况,根据 LDL-C 目标水平的达标情况来描述处方降脂治疗,并再次根据 LDL-C 目标水平的达标情况来描述该患者人群中观察到的心血管事件。

方法:PLANET 登记研究是一项非干预性、回顾性、横断面、多中心的疾病登记研究,纳入捷克共和国和斯洛伐克的成年杂合子 FH 患者。

结果:共有 32 个 FH 治疗专科中心的 1755 例患者入组。15.4%的患者达到了 LDL-C 目标值。在接受高强度他汀类药物治疗的患者亚组中(占研究人群的 54.6%),达到 LDL-C 目标值的患者比例增加至 17.3%。在接受前蛋白转化酶枯草溶菌素 9(PCSK9)抑制剂治疗的 55 例患者中,61.8%达到了 LDL-C 治疗目标。在报告的所有心血管事件中,14.0%发生在达到 LDL-C 目标值的患者中,而未达到目标值的患者中为 86.0%。研究记录(p=0.004),患者在 FH 专科中心的治疗时间越长,达到 LDL-C 目标值的可能性就越高。

结论:尽管 LDL-C 目标值达标率仍然相对较低,但随着专科治疗时间的延长,达到 LDL-C 目标值的可能性会增加。

相似文献

[1]
Real-life LDL-C treatment goals achievement in patients with heterozygous familial hypercholesterolemia in the Czech Republic and Slovakia: Results of the PLANET registry.

Atherosclerosis. 2018-10

[2]
LDL-cholesterol target achievement in patients with heterozygous familial hypercholesterolemia at Groote Schuur Hospital: Minority at target despite large reductions in LDL-C.

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[3]
Treatment pattern of familial hypercholesterolemia in Slovakia: Targets, treatment and obstacles in common practice.

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[4]
High burden of recurrent cardiovascular events in heterozygous familial hypercholesterolemia: The French Familial Hypercholesterolemia Registry.

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[5]
Lipid-modifying therapy and low-density lipoprotein cholesterol goal attainment in patients with familial hypercholesterolemia in Germany: The CaReHigh Registry.

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[6]
Reducing cardiovascular risk in patients with familial hypercholesterolemia: Risk prediction and lipid management.

Prog Cardiovasc Dis. 2019-10-25

[7]
Attainment of LDL-Cholesterol Treatment Goals in Patients With Familial Hypercholesterolemia: 5-Year SAFEHEART Registry Follow-Up.

J Am Coll Cardiol. 2016-3-22

[8]
Familial hypercholesterolemia treatments: Guidelines and new therapies.

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[9]
Familial hypercholesterolemia in Canada: Initial results from the FH Canada national registry.

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[10]
Lipoprotein Apheresis and Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors in Patients With Heterozygous Familial Hypercholesterolemia: A One Center Study.

J Cardiovasc Pharmacol Ther. 2021-1

引用本文的文献

[1]
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Circ Cardiovasc Qual Outcomes. 2024-8

[2]
Real-World Effectiveness of PCSK9 Inhibitors in Reducing LDL-C in Patients With Familial Hypercholesterolemia in Italy: A Retrospective Cohort Study Based on the AIFA Monitoring Registries.

J Am Heart Assoc. 2023-11-7

[3]
How Can Implementation Science Improve the Care of Familial Hypercholesterolaemia?

Curr Atheroscler Rep. 2023-4

[4]
Prevalence of Diabetes and Its Association with Atherosclerotic Cardiovascular Disease Risk in Patients with Familial Hypercholesterolemia: An Analysis from the Hellenic Familial Hypercholesterolemia Registry (HELLAS-FH).

Pharmaceuticals (Basel). 2022-12-28

[5]
Novel Insights into the Management of Patients with Very High Cardiovascular Risk Eligible for PCSK9 Inhibitor Treatment: Baseline Findings from the PERI-DYS Study.

Cardiovasc Drugs Ther. 2024-2

[6]
Clinical Features of Familial Hypercholesterolemia in Children and Adults in EAS-FHSC Regional Center for Rare Diseases in Poland.

J Clin Med. 2021-9-22

[7]
Genetics of Familial Hypercholesterolemia: New Insights.

Front Genet. 2020-10-7

[8]
Higher Responsiveness to Rosuvastatin in Polygenic versus Monogenic Hypercholesterolaemia: A Propensity Score Analysis.

Life (Basel). 2020-5-20

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