European Journal of Echocardiography 2008 9(2):318-320; doi:10.1016/j.euje.2007.03.041
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Three-dimensional echocardiographic evaluation of an incidental quadricuspid aortic valve
Todd A. Armen,
Rashmi Vandse*,
Katherine Bickle and
Nadia Nathan
Department of Anesthesiology, The Ohio State University Medical Center, N-416 Doan Hall, 410 W 10th Avenue, Columbus, OH 43210, USA
Received 5 March 2007; accepted after revision 24 March 2007.
* Corresponding author. Tel.: +1 610 996 5345. E-mail address: rashmi_vandse{at}yahoo.com
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Abstract
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Background: Quadricuspid aortic valve is one of the rare forms of congenital
cardiac valvular disease. Its diagnosis is often missed, even
with the transthoracic echocardiogram. Many of these patients
progress to aortic incompetence later in life requiring surgical
intervention. In addition, quadricuspid aortic valve can be
associated with other congenital cardiac deformities. Hence
early recognition and follow-up is critical in these patients.
Case presentation: We report a patient with quadricuspid aortic valve identified on intraoperative transesophageal 3-D echocardiography. This 66-year-old male presented with the features of congestive heart failure. The preoperative transthoracic echocardiogram (TTE) disclosed, moderately severe aortic valve insufficiency along with severe mitral and tricuspid regurgitation, but failed to reveal the quadricuspid anomaly of the aortic valve. Interestingly, this patient had undergone transthoracic echocardiography on two previous occasions during the past seven years for the evaluation of his valvular heart disease, which all failed to document this anomaly. Intraoperatively, transesophageal echocardiography (TEE) displayed an aortic valve composed of three medium and one small cusps.
Conclusion: Our patient's case demonstrates the usefulness of transesophageal echocardiography in detection of this uncommon congenital malformation.
Keywords: Aortic valve insufficiency; Valvular heart disease; Quadricuspid aortic valve; Transthoracic echocardiography; Transesophageal echocardiography; 3-D echocardiography
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Background
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The quadricuspid aortic valve (QAV) is a rare form of congenital
valvular malformation with a reported incidence ranging from
0.003 to 0.043%.
1,2 It is often detected incidentally, but is
one of the well-recognized causes for aortic incompetence requiring
surgical intervention. Hence early recognition and follow-up
is critical in these patients. Its diagnosis is often missed,
even with the transthoracic echocardiogram, in which case, the
transesophageal echocardiography is a potentially valuable diagnostic
tool in uncovering this anomaly. We report a case of patient
with quadricuspid aortic valve identified on intraoperative
transesophageal 3-D echocardiography.
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Case description
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A 66-year-old African-American male presented to the Emergency
Department with a history of shortness of breath and swelling
of the lower limbs for the past 3 days.
On physical examination, his systemic blood pressure was 162/85 mmHg, with a heart rate of 99 per min and respiratory rate of 34 per min, cardiac auscultation revealed an early diastolic murmur of grade 4 on the second right intercostal space. The electrocardiogram showed normal sinus rhythm and left atrial and left ventricular hypertrophy. Chest radiography displayed marked cardiomegaly. Transthoracic echocardiogram disclosed moderately severe aortic valve insufficiency. The ventricle was markedly dilated with an end diastolic dimension of 6.7 cm and end systolic dimension of 6.0 cm. He also had severe (Grade 3–4+) mitral regurgitation and tricuspid regurgitation (Grade 3+) with moderate pulmonary hypertension. Left ventricular ejection fraction was only 15% with severe global systolic dysfunction. Cardiac catheterization showed triple vessel disease with severe diffuse stenosis of the right coronary artery, left coronary artery and first diagonal artery.
Patient had undergone transthoracic echocardiographic examination on two previous occasions during the past seven years, for the evaluation of his valvular heart disease, which documented the presence of mild aortic valve insufficiency along with mitral regurgitation. Patient was noncompliant on cardiac follow-up and was not on any cardiac medications at the time of presentation other than Aspirin 81 mg OD and carbamazepine 400 mg/day for trigeminal neuralgia.
Patient was planned for aortic valve replacement, mitral valvuloplasty and coronary artery bypass grafting for his coronary artery disease. Intraoperatively transesophageal echocardiography displayed a quadricuspid aortic valve (Figure 1). There were three medium cusps of roughly equal size (area of 2.1, 2.0 and 1.9 cm2) and one smaller cusp (area of 0.9 cm2) as shown in Figure 2. The real time 3-D echocardiography was also performed displaying the valvular morphology (Figure 3). Patient underwent coronary artery bypass grafting (CABG) with three grafts, mitral valve annuloplasty with a 26 mm St. Jude® medical saddle ring along with the chordal cutting and aortic valve replacement with a 25 mm St. Jude® medical biocor bioprosthetic valve. Post-operatively the recovery was uneventful. Retrospectively the review of the preoperative transthoracic echocardiographic images did suggest a possibility of QAV (Figure 4).
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Discussion
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The knowledge of existence of QAV dates back to 1862 with its
first description by Balington (quoted in Ref.
3). Since then
there have been more than 190 cases reported in the literature
with the echocardiography being the leading mode of detection
51.1%, followed by surgery 22.6%, autopsy 15.6% and aortography
accounting for another 6.5%.
4 The incidence of this congenital
anomaly ranges from 0.003 to 0.043% as reported in the literature.
1,2 The advent of echocardiography has greatly increased the frequency
of detection. Sometimes, it may not be possible to visualize
the aortic leaflets adequately with the transthoracic echo,
but the intraoperative TEE has discovered few of these cases
which were not identified by the previous TTE
5,6 as it is in
our case.
Hurwitz and Roberts introduced a classification nomenclature for QAV that included seven subtypes named A to G.7 This case report belongs to type G with four unequal cusps.
The clinical significance of QAV arises from its frequently abnormal function. Aortic incompetence appears to be the most prevalent hemodynamic abnormality accounting for 50–75% of the cases whereas valvular stenosis is rare. Even though it is a congenital malformation, most of the QAVs seem to be competent in childhood. The malcoaptation of the cusps, asymmetric mechanical stress around the four cusps and fibrous thickening leads to aortic incompetence later in life, which also increases the risk of endocarditis. The mean age of diagnosis is 49 years.1 If QAV is found incidentally at the time of echocardiography for other reasons, then, echocardiographic follow-up is advisable to assess the state of the valve. Most of the patients will require valve replacement during the fifth or sixth decade. The most prevalent other cardiac malformations associated with quadricuspid aortic valve are anomalies involving the coronary ostia, which have been reported in 10% of the cases.4 Other malformation associated with quadricuspid aortic valve includes stenosis of pulmonic valve, nonobstructive cardiomyopathy, subaortic stenosis, and ventricular septal defect.1,4,6–8
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Conclusion
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QAV is an unusual congenital malformation noted frequently in
adult life commonly associated with aortic incompetence, with
an increased risk of infective endocarditis. This anomaly may
also be associated with other congenital malformations of the
heart. This calls for early recognition and follow-up of this
rare entity. TEE plays an important role in reaching the appropriate
diagnosis.
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Acknowledgements
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We thank Dr. Chittoor. B. Sai-Sudhakar MD, FRCS, FACS, Department
of Cardiothoracic Surgery, who operated on this patient and
helped us with the case report.
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References
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- Feldman BJ, Khandheria BK, Warnes CA, Seward JB, Taylor CL, Tajik AJ. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol (1990) 65:937–8.[CrossRef][Web of Science][Medline]
- Simonds JP. Congenital malformations of the aortic and pulmonary valves. Am J Med Sci (1923) 166:584–95.[Web of Science]
- Robicsek F, Sanger PW, Daugherty HK, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Am J Cardiol (1969) 23:288–90.[CrossRef][Web of Science][Medline]
- Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis (2004) 13:534–7.[Web of Science][Medline]
- Dencker M, Stagmo M. Quadricuspid aortic valve not discovered by transthoracic echocardiography. Cardiovasc Ultrasound (2006) 4:41.[CrossRef][Medline]
- Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves. Clin Cardiol (2002) Dec;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]
- Janssens U, Klues HG, Hanrath P. Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature. Heart (1997) 78:83–7.[Abstract/Free Full Text]
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Abstract
Background
