Asian Cardiovasc Thorac Ann 2008;16:266-267
© 2008 Asia Publishing EXchange Ltd
IMAGES IN CARDIOTHORACIC MEDICINE AND SURGERY |
Unicuspid Aortic Valve and Aortic Arch Aneurysm in a Patient with Turner Syndrome
Anuj Bansal, MD,
Sandeep Arora, MD,
Darren Traub, MD1,
David Haybron, MD2
Department of Internal Medicine
1 Department of Cardiology
2 Department of Cardiothoracic Surgery Pittsburgh, USA
For reprint information contact: Sandeep Arora, MD Tel: 1 412 578 6929 Fax: 1 412 578 6804 Email: sandeeparora24{at}hotmail.com, Western Pennsylvania Hospital/Temple University Program, 4800 Friendship Avenue, Pittsburgh, PA 15224, USA.
A 45-years-old female with Turner syndrome, hypertension and aortic stenosis presented to the hospital with recurrent episodes of dizziness and chest pain. Examination revealed short stature, webbed neck and low posterior hairline, pulse rate of 84·beat–1, blood pressure of 126/84 mm Hg and bilateral carotid bruits. Cardiac auscultation revealed grade 3/6 ejection systolic murmur heard throughout the precordium. Transthoracic echocardiogram was remarkable for severe aortic stenosis with peak aortic valve gradient of 80 mm Hg and calculated valve area of 0.7 cm2. Trans-esophageal echocardiogram showed a unicommissural unicuspid aortic valve with an eccentric orifice (Figure 1
) and dilated aortic root. Cardiac catheterization revealed normal coronary arteries and computed tomographic scan of the chest showed aneurismal dilatation of ascending and arch of aorta (measuring 5 x 4.6 cm in maximum diameter) extending up to the level of the left common carotid artery with involvement of base of the innominate artery (Figure 2). She was taken to the operating room for aortic arch replacement. Direct inspection of the valve confirmed a severely stenotic, calcified and thickened unicuspid aortic valve (Figures 3A, 3B). She underwent aortic root replacement with a 21-mm porcine bioprosthesis and reimplantation of left and right coronary ostia. Innominate artery aneurysm was excised and was replaced with a 10-mm hemashield Dacron graft. She had subtotal aortic arch replacement with placement of a 24-mm Dacron hemashield beveled arch and ascending aortograft under deep hypothermic circulatory arrest and selective cerebral perfusion. She tolerated the procedure well and was discharged home on 7th post-operative day.
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Figure 1. Transesophageal echocardiogram at mid-esophageal level showing a unicommissural unicuspid aortic valve with eccentric orifice and a raphe.
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Figure 2. Computed tomographic scan of the chest (3-D reconstruction image, posterior view) showing aneurismal dilatation of ascending aorta and innominate artery.
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Figure 3. Intraoperative (A) and gross (B) examination revealing severely stenotic, calcified and thickened unicommissural unicuspid aortic valve.
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Adult unicuspid aortic valve is a rare cardiac anomaly seen in 
5% of patients undergoing isolated aortic valve replacement for aortic stenosis (with or without aortic regurgitation) and without associated mitral valve disease.1 Unicuspid valve can have a central orifice without any true commissure (acommissural) or may have one true commissure (unicommissural) usually located posteriorly, with an eccentric orifice, which could be linear (like an exclamation mark), triangular, oval or "tear drop" shape. Unicuspid aortic valves are usually stenotic at birth requiring aortic valve replacement in 3rd decade of life. Though dilatation of ascending aorta is a common finding in patients with unicuspid valve and is thought to be a result of premature medial layer smooth muscle cells apoptosis and ensuing cystic medial necrosis, dilatation of arch of aorta is almost unheard of in these patients. Our patient was unique because of presence of unicuspid aortic valve and aortic arch aneurysm, both of which have not been previously reported in association with Turner syndrome.
REFERENCES
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