(Circulation. 2000;101:2771.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
From the Zena and Michael A. Wiener Cardiovascular Institute (S.G.W., G.H., Z.A.F., V.F., A.G.Z., M.S., J.J.B.) and the Department of Radiology (Z.A.F., M.S.), Mount Sinai Medical Center, New York, NY.
Correspondence to Juan J. Badimon, PhD, Director, Cardiovascular Biology Research Laboratory, Zena and Michael A. Wiener Cardiovascular Institute, One Gustave L. Levy Place, Box 1030, New York, NY 10029-6574. E-mail jbadimo{at}smtplink.mssm.edu
Aortic dissection is a
well-known complication of Marfan syndrome. Aortic dissection in
patients with Marfan syndrome is associated with aortic root
dilatation. As imaging techniques have improved, it has become clear
that some patients with Marfan syndrome, in the absence of symptoms,
may have evidence of a prior aortic dissection. A 47-year-old white man
with documented Marfan syndrome and no prior symptomatology referable
to aortic dissection had elective high-resolution MRI of the aorta for
an unrelated research project. Importantly, he had no known risk
factors for atherosclerotic disease. The MR images showed that his
aortic root was not dilated, with a diameter of 36 mm (Figure 1A
). His ascending aorta was not dilated,
with normal wall thickness and no evidence of an intimal flap (Figure 1B
). However, a small defect was evident in the wall of the
descending thoracic aorta that was more readily appreciated on
magnified views of the region (Figure 2
).
Proton densityweighted (Figure 2A
) and T2-weighted (Figure 2B
) images clearly showed a very small but distinct defect
within the aortic wall, consistent with a false lumen. Review
of the complete series of MR images confirmed a small, limited type B
dissection, beginning just beyond the origin of the left subclavian
artery and extending distally into the descending thoracic aorta for
5 cm. No communicating channels were identified between the 2
lumens.
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With improvements in vascular imaging techniques that permit high-resolution noninvasive analysis of the arterial wall, we may find more asymptomatic, previously undetected aortic dissections in patients with Marfan syndrome. Generally, it has been assumed that this occurs in association with aortic root dilatation; in this case, however, the dimensions of the aortic root were within normal limits. The natural history of this phenomenon in patients with Marfan disease is unclear.
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Lukes 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 Lukes Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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