(Circulation. 2000;101:2446.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Tangier Disease With Continuous Massive and Longitudinal Diffuse Calcification in the Coronary Arteries
Demonstration by the Sagittal Images of Intravascular Ultrasonography
From the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, Osaka University, Osaka, Japan.
Correspondence to Ken-ichi Hirano, MD, PhD, Department of Internal Medicine and Molecular Science, Graduate School of Medicine, B5, Osaka University, 2–2, Yamadaoka, Suita, Osaka 565-0871, Japan. E-mail khirano{at}kb3.so-net.ne.jp
A 48-year-old man was
first referred to our clinic in January 1989 because of marked
hypocholesterolemia with very low HDL
cholesterol, anemia, and hyperbilirubinemia. He had large
tonsils, corneal opacities, hepatosplenomegaly, and thrombocytopenia.
Serum levels of total cholesterol,
triglycerides, and HDL cholesterol were 0.72,
2.6, and 0.16 mmol/L (28, 232, and 6 mg/dL), respectively.
Concentrations of apolipoproteins (apo) A-I and A-II were 1.3 and
0.9 µmol/L (3.9 and 1.5 mg/dL), respectively. His daughter’s
serum levels of total cholesterol, HDL
cholesterol, and apo A-I were 3.3 mmol/L, 0.64
mmol/L, and 34.7 µmol/L (128, 24, and 104 mg/dL), respectively.
He was diagnosed with Tangier disease by clinical manifestations,
analysis of lipoproteins, and 2D electrophoresis, which
confirmed the increase of preproapo A-I. Interestingly, xanthoma in the
Achilles’ tendons, which had rarely been reported in patients with
Tangier disease, was observed in the patient, and the thickness was
9 mm (mean of bilateral determinations; control, 6±2 mm).
Subsequently, the diagnosis of homozygous Tangier disease was also
established by biopsy of the patient’s bone marrow, showing the
presence of foam cells (Figure 1
).
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Our patient began having exertional chest pain in January 1997.
Myocardial perfusion images with 201Tl revealed a
defect in the inferoposterior wall with an incomplete redistribution;
thus, a coronary angiogram was performed in July 1997. It
revealed massive and longitudinal diffuse calcifications in the 3
coronary arteries that could be seen only on the scout
radiograph (Figure 2A and 2C). We also
found severe atherosclerosis in all 3 vessels,
including the left main trunk (LMT); 90% stenosis in the mid
portion of the right coronary artery (RCA) (segment 2), 75%
stenosis in the LMT (segment 5), 75% stenosis in the
proximal portion of the left anterior descending
coronary artery (LAD) (segment 6), 75% stenosis in the
mid portion of the LAD (segment 7), 90% stenosis in the just
proximal portion of the left circumflex artery (LCx) (segment 11), and
90% stenosis in the mid portion of the LCx (segment 13),
respectively (Figures 2B, 2D, and 3).
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We performed intravascular ultrasonography (IVUS)
simultaneously with the coronary angiogram, and it
demonstrated notably that the atherosclerotic plaque with intimal
thickening and its superficial calcification protruded toward the
center of the lumen in cross-sectional IVUS images (Figure 3
, I
and II). Conversely, only a very thin plaque was observed on the
opposite side of the protrusion (Figure 3, I and II).
Consequently, the lumen there, especially in II, appeared
crescent-shaped (Figure 3, red trace). We then obtained
different sagittal sections of 3D-reconstructed IVUS images to
analyze the coronary arteries more precisely. It was
also noteworthy that massive and longitudinal diffuse calcifications
extended continuously from distal to the second diagonal branch to the
LMT (Figure 3, bottom right, white
arrows).
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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