演題

CRAS-P3-3

The Strict Management of Low-Density Lipoprotein Cholesterol in Heterozygous Familial Hypercholesterolemia for the Secondary Prevention of Cardiovascular Disease

[演者] Takuro Abe:1
[共同演者] Haruki Sekiguchi:1, Jihaeng Imu:1, Eri Yamamoto:1, Makiko Kimura:1, Akiko Sakai:1, Kayoko Sato:1, Toshiyuki Yamamoto:2, Nobuhisa Hagiwara:1
1:Department of Cardiology, Tokyo Women's Medical University Hospital, Tokyo, 2:Institute of Medical Genetics, Tokyo Women's Medical University, Tokyo

Background: Heterozygous familial hypercholesterolemia(heFH) is the high risk of cardiovascular disease(CVD), however, the rate of diagnosis is less than 1% in Japan. Case: A 68-year-old man visited our hospital for complying dyspnea and abnormal ECG change in March 2016. He had been diagnosed as hypercholesterolemia since 58years old without medications. He was diagnosed as heFH, because of his mother had premature CVD and his low-density lipoprotein cholesterol(LDL-C) was 213mg/dl. The results of exercise stress myocardial scintigraphy showed the severe ischemia, and he was hospitalized for the coronary angiography(CAG) in June. He had 3vessels disease and underwent Coronary Artery Bypass Grafting(CABG). His LDL-C level went down to 77mg/dl by pitavastatin 2mg and ezetimibe 10mg. Nine months after CABG, he had the heart attack and emergency hospitalization. All of bypass grafts were occluded and the atherosclerotic plaque was observed even in RIMA by IVUS. The emergency percutaneous coronary intervention was performed to his bypass graft. His other lipoprotein prolife was still higher after statine treatments(oxidized LDL 246U/L and APOB 165mg/dl). Moreover, the gene analysis found that he had polygenetic gene mutations as PCSK-9(V4I and A53V) and ApoA5(G158C). Decision-making: This high risk patient received evolocumab140mg twice in one month, after which LDL-C, oxidized LDL, and APOB were decreased to 10-30mg/dl, 29U/L, and 16mg/dl respectively without side effects. We confirmed no re-stenosis and obvious atherosclerotic plaque improvement in grafts by IVUS at the re-study CAG after 5 months. Conclusion: For the secondary prevention of CVD in the high risk heFH patients, especially with gene mutations, the target of LDL-C levels should be deeply concern.

[Keywords] dyslipidemia / acute coronary syndrome
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