European Journal of Echocardiography 2008 9(3):386-387; doi:10.1016/j.euje.2006.12.001
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Endocarditis complicating a congenital quadricuspid aortic valve
Fabrice Bauer1,2,*,
Pierre-Yves Litzler3,
Alfred Tabley3,
Alain Cribier1,2 and
Jean-Paul Bessou2,3
1 Department of Cardiology, Rouen University Hospital, Rouen, France
2 Unité INSERM U644, Rouen University Medical School, Rouen, France
3 Department of Cardiovascular Surgery, Rouen University Hospital, Rouen, France
Received 7 September 2006; accepted after revision 4 December 2006; online publish-ahead-of-print 26 February 2007.
* Corresponding author: Cardiologie, Section of Echocardiography, Department of Cardiology, Rouen University Hospital Charles Nicolle, 1, rue de Germont, 76031 Rouen Cedex, France. Tel: +33 232888232; fax: +33 232888714. E-mail address: fabrice.bauer{at}chu-rouen.fr (F. Bauer).
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Abstract
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The most common aortic valve congenital abnormality is observed
in bicuspid aortic valve. Only a few cases of aortic valve quadricuspidy
have been reported in the literature. We report a new case of
endocarditis complicating a congenital quadricuspid aortic valve.
Keywords: Aortic insufficiency; Endocarditis; Aortic valve replacement; Echocardiography; Transesophageal echocardiography
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Case report
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A 68-year-old male with a history of hypertension and angina
was referred to our hospital for a septic spondylodiscitis after
complaining of inflammatory back pain and chronic fever. Physical
examination revealed a 3/6 diastolic murmur and no signs of
heart failure. Numerous blood cultures were positive to
Streptococcus oralis. Transesophageal echocardiography short-axis view demonstrated
a quadricuspid aortic valve with fully opening four equal leaflets
(
Figure 1). Additionally, transesophageal echocardiography
detected multiple vegetations involving all four leaflets (
Figure 2).
Destruction of the aortic valve was evidenced by a severe aortic
regurgitation documented from the transesophageal echocardiography
long-axis view (
Figure 3). No other cardiac structural
abnormalities were found. Antibiotic therapy was instituted
parenterally by ampicillin plus gentamycin based on susceptibility
tests. After three weeks of treatment, the patient was operated
because of significant aortic valve deterioration and left ventricular
enlargement as demonstrated by transthoracic echocardiography.
Intra-operatively, the surgical view corroborated the ultrasound
findings (
Figure 4). The quadricuspid aortic valve presented
with multiple vegetations on each leaflet. A prosthetic aortic
valve was employed to replace the defective valve. The patient
was discharged after six weeks of additional antibiotic treatment
with no evidence of active infectious or prosthetic aortic valve
dysfunction.
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Discussion
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The quadricuspid aortic valve has been recognized as a rare
congenital abnormality accounting for less than 0.001% of the
population at autopsy.
1 The aortic valve normally forms from
the embryonic truncus arteriosus. Abnormalities in this area
will lead to the development of a quadricuspid aortic valve
and others coexisting congenital cardiac defects implicating
the coronary arteries, the left ventricular outflow tract, the
pulmonary valve or the inter-atrial septum. Aortic valve quadricuspidy
is frequently misdiagnosed by transthoracic echocardiography
necessitating the transesophageal approach.
2 The quadricuspid
aortic valve is usually composed of four unequal-sized leaflets
with a fixed orientation of the commissures (anterior–posterior
and horizontal commissures). The coronary arteries arise from
the two opposite anterior-right and posterior-left leaflets.
The quadricuspid aortic valve with four equal-sized leaflet
found in our patient is less frequent and corresponds to type
a in the Hurwitz and Roberts' classification.
3 With degeneration of aging valve, sclerosis and calcification
can occur.
4 Incidence of hemodynamic abnormality is not documented
but aortic regurgitation seems to be more prevalent than aortic
stenosis.
The importance of quadricuspid aortic valve as a potential substrate for infective endocarditis is not known but could be related to the morphology of the valve. Usually, patients with infective endocarditis have unequal-sized leaflets.5 The asymmetrical morphology of the cusps is a predisposing condition to turbulences, thus infective endocarditis as described in aortic bicuspid valve. Quadricuspid aortic valve with equal-sized leaflets is infrequently associated with infective endocarditis.6 We report the third case in the literature. The clinical presentation of quadricuspid aortic valve endocarditis does not differ from common acute infective endocarditis. Here, spondylodiscitis resulted from embolic complication. Multiple vegetations were the only echocardiographic predictor of systemic embolisation in our patient. We did not see large, pedunculated, noncalcified or prolapsing valvular vegetation.
Therefore, aortic valve quadricuspidy is prone to infective endocarditis irrespective of its morphology. This rare congenital abnormality should deserve close attention, medical care, patient education for a good oral and dental hygiene, and appropriate antibiotic prophylaxis for procedures.
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References
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- Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol (1973) 31:623–6.[CrossRef][Web of Science][Medline]
- Kucukoglu MS, Erdogan I, Okcun B, Baran T, Mutlu H, Uner S. Quadricuspid aortic valve abnormality associated with aortic stenosis and aortic insufficiency. J Am Soc Echocardiogr (2002) 15:90–2.[CrossRef][Web of Science][Medline]
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- Teragaki M, Sakai Y, Asawa K, Matsumoto R, Kasayuki N, Nakayama K, et al. Quadricuspid aortic valve: report of three cases. Am J Med Sci (2004) 328:281–5.[CrossRef][Web of Science][Medline]

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