European Journal of Echocardiography Advance Access originally published online on February 19, 2008
European Journal of Echocardiography 2008 9(4):575-576; doi:10.1093/ejechocard/jen036
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
Quadricuspid aortic valve associated with unruptured sinus of Valsalva aneurysm
Sridevi R. Pitta,
Ashok Kondur and
Luis Afonso*
Department of Cardiology, Harper University Hospital, Wayne State University, 1 Brush, 3990 John R Street, Detroit, MI 48201-1916, USA
Received 16 December 2007; accepted after revision 6 January 2008; online publish-ahead-of-print 19 February 2008.
* Corresponding author. Tel: +1 313 745 2620. E-mail address: lafonso{at}dmc.org
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Abstract
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Quadricuspid aortic valve is a rare congenital anomaly. It may
present as an isolated anomaly but is occasionally associated
with aortic regurgitation. Sinus of Valsalva aneurysm (SVA)
is also an infrequent congenital anomaly, typically associated
with tricuspid aortic valves. There are only a few reported
cases of SVA (ruptured) associated with quadricuspid aortic
valves in the literature. We report the first case of the association
of quadricuspid aortic valve with an unruptured SVA in an adult
patient.
Keywords: Quadricuspid Aortic Valve; Sinus of Valsalva Aneurysm; Echocardiography
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Case report
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A 40-year-old African-American female presented with shortness
of breath and haemoptysis. Past medical history was unremarkable.
Pertinent exam findings included a blood pressure of 118/40
mmHg, a water hammer pulse, jugular venous distension to the
angle of jaw, a prominent early diastolic murmur in the aortic
area, and a hyperdynamic apical impulse. A transthoracic echocardiogram
showed a possible quadricuspid aortic valve, sinus of valsalva
aneurysm (SVA) and severe aortic regurgitation. These findings
were confirmed on transesophageal echocardiography (
Figure 1A and B) and later intraoperatively, following which, our patient
uneventfully underwent surgical resection (
Figure 2) of
the SVA and aortic valve replacement with a 21 mm Carbomedics
mechanical valve.
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Figure 1 (A and B) Transesophageal echocardiogram (TEE) short axis views in the vicinity of the aortic valve plane, depicting the four symmetric cusps and SVA (A, left panel) and following agitated saline injection (B, right panel), delineating the shape and extent of the windsock SVA ( 2.5 cm in length), and origin from the accessory sinus of Valsalva (4th sinus).
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Discussion
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Quadricuspid aortic valve is a rare congenital anomaly that
predominantly affects males and has a reported incidence of
0.008–0.033%.
1 Hurwitz and Roberts
2 classified quadricuspid
valves based on cusp size and degree of cusp equality into seven
subtypes. The competency of a quadricuspid valve has been shown
to be related to aortic cusp morphology and symmetry. The risk
of aortic insufficiency and infective endocardits is reportedly
lowest in valves with four symmetric cusps.
1 It is estimated
that
44% of quadricuspid valves have associated aortic insufficiency.
3 Congenital SVA is a very rare anomaly with a reported incidence
of 0.09% in the US population.
4 SVA is associated with multiple
conotruncal congenital malformations because of a single embryologic
event affecting conbulbar septation and aortic arch development
occurring at the level of neural crest development.
4 Only a
few published cases
5,6 have described the unusual association
of a quadricuspid aortic valve with SVA, all of which reported
ruptured SVAs. To the best of our knowledge, our case is the
first to report the association of quadricuspid valve with an
unruptured SVA (see
supplementary data online). Our case illustrates
the utility of conventional and contrast echocardiography in
precisely defining the morphologic abnormalities preoperatively
and emphasizes the importance of early recognition in the prevention
of potential complications such as SVA rupture and infective
endocarditis.
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Supplementary data
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Supplementary data are available at European Journal of Echocardiography online.
Conflict of interest: none declared.
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References
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- Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves. Clin Cardiol (2002) 25:548–52.[CrossRef][Web of Science][Medline]
- Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol (1973) 31:623–6.[CrossRef][Web of Science][Medline]
- Bonde P, Sachithanandan A, McClements B, Gladstone DJ. Quadricuspid aortic valve: a rare cause of aortic insufficiency. J Heart Valve Dis (2002) 11:506–8.[Medline]
- Sundeen JT, Bloom S. Sinus of Valsalva aneurysm associated with multiple conotruncal congenital malformations. Hum Pathol (1987) 18:96–9.[CrossRef][Medline]
- Cullen S, Sullivan ID. Images in cardiovascular medicine. Ruptured sinus of valsalva with aorta-to-right atrial fistula. Circulation (1998) 98:2503–4.[Free Full Text]
- Egred M, Patel JC, Metcalfe MJ. Sinus of Valsalva fistula with quadricuspid aortic valve, a first reported association. Int J Cardiol (2005) 101:151–2.[CrossRef][Medline]
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Abstract
Case report