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Page 2 of 4 Salsano et al. Vessel Plus 2020;4:21 I http://dx.doi.org/10.20517/2574-1209.2020.12

Figure 1. Surgical view of the aortic valve and the left main ostium during the procedure. A: native valve in situ, left main ostium located
in proximity to the posterior commissure; B: marked stent location after native valve removal; C: implanted bioprostethic valve; the valve
stents are not in conflict with the left main ostium, which has been left in its original location

Various classifications have been introduced to describe the different morphologies of BAVs. One of the
most used in clinical practice is the Sievers classification. Introduced in 2007, it is based on the number of
raphes to define the phenotype of the BAV, and includes three classes: types 0 (no raphe), 1 (one raphe) and
2 (two raphes); type 0 valves are divided into anteroposterior (AP) or lateral according to spatial position
[2]
and orientation between the cusps and the coronary ostia .

BAVs can develop stenosis or regurgitation and be associated with vascular anomalies. Some studies claim
[3]
that ascending aortic dilatation is the most common vascular anomaly found in patients with BAVs .
Various mechanisms are involved including cystic medial necrosis, fragmentation of elastic fibers or the
loss of smooth muscle cells in the ascending aorta wall . The association between BAV and coronary
[4]
[5-7]
anomalies has been described as a rare finding in the current literature . We report a case of BAV stenosis
associated with an ascending aortic aneurysm and a positional anomaly of the left main coronary ostium.

CASE REPORT
A 60-year-old woman presented with acute respiratory distress and chest pain to the emergency room.
Her main cardiovascular risk factors included smoking, recurrent bronchitis, hypercholesterolemia,
β-thalassemia trait and a family history of coronary artery disease.

On admission, trans-thoracic echocardiography revealed a dilated ascending aorta (45 mm) with a normal
aortic root, severe aortic valve stenosis (mean gradient 83 mmHg) in the presence of bicuspid aortic valves,
and a regular left ventricular ejection fraction (50%). Coronary angiography revealed apparently normal
coronary anatomy without any critical lesion [Video 1]. Chest x-ray, EKG and Doppler examination of the
supra-aortic vessels did not elucidate any anomaly. The patient was therefore transferred to our Division
for surgical treatment. We planned for replacement of the aortic valve and ascending aorta through a mini-
sternotomy approach, according to current guidelines .
[8,9]
In the operating room, a Sievers type 0 AP BAV was recognized. The left main coronary ostium was found
to originate in proximity to the posterior commissure, having an intramural take-off and, it was surrounded
by thick fibrous tissue, possibly due to acquired jet lesions [Figure 1A]. Interestingly, this anomaly was not
detected during the previous routine angiography [Video 1].

Once the native leaflets had been removed and the annulus decalcified, we chose to implant a bioprosthetic
valve (Carpentier Edwards Magna Ease size 21), respecting the patient’s wishes.