A case of effort angina complicated with cerebrotendinous xanthomatosis involving severe coronary artery calcification a
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IMAGES IN CARDIOVASCULAR INTERVENTION
A case of effort angina complicated with cerebrotendinous xanthomatosis involving severe coronary artery calcification and the detection of a calcified nodule on optical coherence tomography Kohei Tonai1 · Yoshiyuki Miyamoto1 · Tetsuya Iwaguro1 · Masaoki Miyamoto1 · Yaushi Okumoto1 · Keizo Kimura1 Received: 15 April 2019 / Accepted: 5 February 2020 © Japanese Association of Cardiovascular Intervention and Therapeutics 2020
A 43-year-old male was admitted to our hospital with effort angina. At the age of 31, he had exhibited a disturbance of memory, Achilles tendon xanthomas, and an elevated serum cholestanol level of 9.9 mg/dl, which led to a suspicion of cerebrotendinous xanthomatosis (CTX). His total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol (LDL-C) levels were 208 mg/dl, 65 mg/dl, and 130 mg/dl, respectively. He was finally diagnosed with CTX via a genetic analysis, which demonstrated the p.Arg372Gln variant in the CYP27A1 gene (which codes for sterol 27-hydroxylase). He was treated with chenodeoxycholic acid and a statin. At our hospital, he exhibited bilateral Achilles tendon xanthomas. His serum low-density-lipoprotein cholesterol level was 95 mg/dl. Computed tomography (CT) showed severe calcification in the left coronary artery. Coronary angiography revealed 99% stenosis in the proximal portion of the left descending artery (LAD). The right coronary artery was poorly developed. We decided to perform a percutaneous coronary intervention (PCI) involving the LAD. Since it was impossible to advance a balloon or imaging catheter beyond the stenotic lesion due to the presence of severe calcification, we performed rotational atherectomy and then proceeded to implant a drug-eluting stent. Follow-up angiography conducted 12 months after the PCI showed no in-stent restenosis and no significant stenosis in the left circumflex (LCX) artery. To obtain detailed images of the LCX, optical
coherence tomography (OCT) was carried out. It demonstrated a calcified nodule (CN) in the LCX wall, which protruded into the lumen. The CN was not distinguishable from the CN observed in common arteriosclerosis (Fig. 1). CTX is an autosomal recessive disorder, caused by a deficiency of sterol 27-hydroxylase and the overproduction of cholestanol [1]. Coronary artery events have been reported in patients with CTX [2]. However, few reports have described the coronary artery pathology or imaging of this disease. The mechanism responsible for arteriosclerosis in CTX is not fully understood. The accumulation of cholestanol in the subendothelial region might lead to arteriosclerosis. In an autopsy study, however, cholestanol was found to account for just 2.8% of the sterols in aortic plaques [3]. Recent studies have suggested that sterol 27-hydroxylase, which is produced in macrophages and endothelial cells as well as in the liver, seems to play an important role in the transportation of cholesterol from the peripheral organs to the liver. The accumulati
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