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一例伴有心脏X综合征及射血分数保留的心力衰竭的韦纳综合征病例。

A case of Werner's syndrome with cardiac syndrome X and heart failure with preserved ejection fraction.

作者信息

Yamamoto Masahiro, Yamamoto Eiichiro, Yasuda Osamu, Yasuda Hisayo, Sakamoto Kenji, Tsujita Kenichi, Izumiya Yasuhiro, Kaikita Koichi, Hokimoto Seiji, Ogawa Hisao

机构信息

Department of Cardiovascular Medicine, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan.

Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.

出版信息

J Cardiol Cases. 2015 Oct 1;12(6):195-198. doi: 10.1016/j.jccase.2015.08.009. eCollection 2015 Dec.

DOI:10.1016/j.jccase.2015.08.009
PMID:30546594
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6281855/
Abstract

We herein report a case of Werner's syndrome (WS) with cardiac syndrome X (CSX) and heart failure with preserved ejection fraction (HFpEF), receiving nicorandil treatment. A 58-year-old woman with chest discomfort on exercise was suspected of having effort-angina pectoris because of broad ST-depression in electrocardiogram of exercise test and reversible defect in the posterior-wall portion of left ventricle in exercise thallium myocardial scintigraphy. This patient also exhibited HFpEF, diagnosed by increased ratio of early-transmitral-flow-velocity to tissue-Doppler early-diastolic mitral annular velocity (E/e') in echocardiography and plasma B-type natriuretic peptide (BNP) levels. However, coronary angiography revealed no organic stenosis in epicardial coronary arteries, and coronary physiological measurements by PressureWire™ (St. Jude Medical, St Paul, MN, USA) demonstrated that coronary flow reserve (CFR) was greatly decreased. Because impaired CFR represents coronary microvascular dysfunction in the absence of obstructive coronary narrowing, we diagnosed CSX, and initiated the administration of nicorandil to improve coronary microcirculation. After three-month-treatment of nicorandil, the patient's symptoms were diminished, and reversible defect in exercise myocardial scintigraphy was improved. Furthermore, both E/e' and BNP were decreased, indicating the improvement of HFpEF via the restoration of microvascular dysfunction. Thus, nicorandil administration could bring beneficial effects in WS with CSX and HFpEF, accompanied by coronary microcirculation dysfunction. < Contrary to previous case reports regarding Werner's syndrome (WS) with obstructive coronary artery disease (CAD), we herein report a case of WS with cardiac syndrome X (CSX) without obstructive CAD, complicated with heart failure with preserved ejection fraction (HFpEF). Because impaired coronary microcirculation is known to be associated with left ventricular hypertrophy and HFpEF, nicorandil could improve not only CSX but HFpEF via the restoration of coronary microvascular dysfunction.>.

摘要

我们在此报告一例患有心脏X综合征(CSX)和射血分数保留的心力衰竭(HFpEF)的维尔纳综合征(WS)患者接受尼可地尔治疗的病例。一名58岁女性,运动时出现胸部不适,运动试验心电图显示广泛ST段压低,运动铊心肌闪烁显像显示左心室后壁部分可逆性缺损,怀疑患有劳力性心绞痛。该患者还表现出HFpEF,经超声心动图检查早期二尖瓣血流速度与组织多普勒早期舒张期二尖瓣环速度之比(E/e')升高及血浆B型利钠肽(BNP)水平升高确诊。然而,冠状动脉造影显示心外膜冠状动脉无器质性狭窄,使用PressureWire™(美国明尼苏达州圣保罗市圣犹达医疗公司)进行的冠状动脉生理测量表明冠状动脉血流储备(CFR)大幅降低。由于CFR受损代表在无阻塞性冠状动脉狭窄情况下的冠状动脉微血管功能障碍,我们诊断为CSX,并开始给予尼可地尔以改善冠状动脉微循环。尼可地尔治疗三个月后,患者症状减轻,运动心肌闪烁显像中的可逆性缺损得到改善。此外,E/e'和BNP均降低,表明通过恢复微血管功能障碍改善了HFpEF。因此,给予尼可地尔可能对伴有CSX和HFpEF且伴有冠状动脉微循环功能障碍的WS患者带来有益影响。<与之前关于患有阻塞性冠状动脉疾病(CAD)的维尔纳综合征(WS)的病例报告相反,我们在此报告一例无阻塞性CAD的患有心脏X综合征(CSX)的WS病例,并发射血分数保留的心力衰竭(HFpEF)。由于已知冠状动脉微循环受损与左心室肥厚和HFpEF有关,尼可地尔不仅可以改善CSX,还可以通过恢复冠状动脉微血管功能障碍改善HFpEF。>

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本文引用的文献

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Circ Cardiovasc Interv. 2014 Feb;7(1):43-8. doi: 10.1161/CIRCINTERVENTIONS.113.000953. Epub 2014 Jan 7.
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