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(Circulation. 2004;109:671.)
© 2004 American Heart Association, Inc.

Images in Cardiovascular Medicine

Bicuspid Aortic Valve Aneurysm

Osman Ratib, MD, PhD; Joseph K. Perloff, MD; John S. Child, MD

From the Department of Radiological Sciences and the Ahmanson/UCLA Adult Congenital Heart Disease Center, the David Geffen School of Medicine at UCLA, Los Angeles, California

Correspondence to Joseph K. Perloff, MD, Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Center for the Health Sciences, 650 Charles E. Young Dr S, Room 47-123-CHS, Box 951679, Los Angeles, CA 90095-1679. E-mail josephperloff{at}earthlink.net

Congenitally abnormal aortic valves are defined morphologically according to the number and type of cusps and commissures. The bicuspid aortic valve, first recognized by Leonardo da Vinci in the early 16th century, is the most common congenital anomaly to which that structure is subject. The estimated incidence is 0.9% to 2% of the general population, or a prevalence in the United States of 4 million. Two varieties of bicuspid aortic valves are based on commissural fusion, namely, fusion between the left and right cusps or fusion between the right and noncoronary cusps. Three varieties of bicuspid aortic valves are based on cuspal size, namely, 2 cusps of equal size, 2 cusps of unequal size, or a conjoined cusp that is twice the size of its mate. The greatest degree of cuspal inequality is represented by the conjoined cusp. The images shown here represent a unique variety of cuspal inequality caused by aneurysmal dilatation of the nonconjoined cusp (Figure). Dilatation of the ascending aorta, which is a common coexisting feature of a bicuspid aortic valve, is caused by an inherent medial abnormality that attenuates the ascending aortic wall, and is coupled with the bicuspid morphology of the congenitally malformed valve rather than its functional state. The aneurysmal cusp shown in the Figure might harbor a tissue abnormality analogous to the inherent medial fault that attenuates the dilated ascending aorta.

View larger version (149K): [in this window] [in a new window]   The 2 coronal images on the left are from dynamic cine MRIs. Left, Upper image in diastole shows aneurysmal dilatation (Aneur) of 1 cusp of a congenitally bicuspid aortic valve. There is no regurgitation. The ascending aorta (AscAo) is moderately dilated. Lower image in systole shows unobstructed flow across the left ventricular outflow tract (LVOT). LV indicates left ventricular. Right, These 2 oblique images were acquired across the bicuspid aortic valve. Upper image in diastole shows the aneurysmal nonconjoined aortic cusp (Aneur) and the smaller conjoined second cusp with its false raphe (FR). Lower image in systole shows the unobstructed bicuspid valve (BicuspV), which was functionally normal despite the cuspal aneurysm.

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/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ratib, O. Articles by Child, J. S. Search for Related Content PubMed PubMed Citation Articles by Ratib, O. Articles by Child, J. S. Related Collections CT and MRI Pediatric and congenital heart disease, including cardiovascular surgery