CARDIOVASCULAR FLASHLIGHT. Cleft bicuspid aortic valve: the Achilles' heel of echocardiography?

European Heart Journal (dal numero del 31 settembre 2010):


Gianluca Piccoli *, Gianaugusto Slavich, Pasquale Gianfagna, and Daniele Gasparini. Department of Radiology and Cardiopulmonary Sciences, Santa Maria della Misericordia University Hospital Udine, Italy.
* Corresponding author
Tel: +39 0432552894, Fax +39 0432552885
Email: piccoli.gianluca@gmail.com

In 2002, Thiene and co-workers reported the possibility that echocardiography might not be able to correctly differentiate a bicuspid valve with a median cleft (simulating a rudimentary commissure) from a true tricuspid valve. This is the only published report on this subject so far.
In this brief report, we describe a similar case where transthoracic echocardiography (TTE) missed the diagnosis which was later established by cardiac magnetic resonance imaging (MRI).
The patient is a 36-year-old male with an ascending aorta diameter of 5 cm, moderate aortic stenosis, and a tricuspid aortic valve by TTE (Panel A). The patient underwent a cardiac MRI at our institution: the presence of a bicuspid aortic valve was documented along with a pseudoraphe (Panel B: systolic short-axis view; Panel C: diastolic short axis view) and a 5 cm aneurysm of the ascending aorta (Panel D). On the basis of the MRI findings, the patient underwent the Bentall procedure with favourable outcome.
Both the aortic valve specimen (Panel E) and the 3D reconstruction of a CT scan of the excised aortic valve (Panel F) demonstrate a bicuspid valve. In both instances, the valve presented a small incision (pseudoraphe) involving the larger cusp and simulating a tricuspid configuration (white arrow).
Cardiac MRI is an important diagnostic tool for the evaluation of patients with abnormalities of the ascending aorta and of the aortic valve. The high-contrast resolution, the absence of acoustic window limitations, and the good temporal resolution provide an accurate definition of aortic valve morphology. This is quite relevant because, as evidenced in this case, the combination of bicuspid valve-dilated ascending aorta will dictate the surgical therapeutic strategy.