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European Journal of Echocardiography 2008 9(3):386-387; doi:10.1016/j.euje.2006.12.001
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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).


    Abstract
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 Abstract
 Case report
 Discussion
 References
 
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


    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.


Figure 1
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Figure 1 Short-axis transesophageal view at 60° showing congenital quadricuspid aortic valve with four equalsized leaflets.

 


Figure 2
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Figure 2 Short-axis transesophageal view at 60° showing fully opening congenital quadricuspid aortic valve with multiple vegetations.

 


Figure 3
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Figure 3 Long-axis transesophageal image at 170° demonstrating severe aortic regurgitation.

 


Figure 4
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Figure 4 The gross anatomy view of the quadricuspid aortic valve from a surgical perspective. Blue arrows indicate the four commissures.

 

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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.


    References
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 Abstract
 Case report
 Discussion
 References
 

  1. Simonds JP. Congenital malformations of the aortic and pulmonary valves. Am J Med Sci (1923) 166:584–95.[Web of Science]
  2. Watanabe T, Hosoda Y, Sasaguri S, Aikawa Y. A quadricuspid aortic valve diagnosed by transesophageal echocardiography: report of a case. Surg Today (1998) 28:1102–4.[CrossRef][Web of Science][Medline]
  3. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol (1973) 31:623–6.[CrossRef][Web of Science][Medline]
  4. 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]
  5. Takeda N, Ohtaki E, Kasegawa H, Tobaru T, Sumiyoshi T. Infective endocarditis associated with quadricuspid aortic valve. Jpn Heart J (2003) 44:441–5.[CrossRef][Medline]
  6. 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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