European Journal of Echocardiography 2006 7(4):330-331; doi:10.1016/j.euje.2005.06.007
Copyright © 2005, The European Society of Cardiology
Two cases of quadricuspid aortic valve
Sibel Turhan*,
Cansin Tulunay,
Tamer Say
n,
Cagdas Ozdol,
Mustafa Klckap and
rem Dinçer
Ankara University School of Medicine, Department of Cardiology, Ankara, Turkey
Received 24 March 2005; received in revised form 2 June 2005; accepted after revision 15 June 2005.
* Corresponding author. Ankara Universitesi Tp Fakultesi Ibni Sina Hastanesi Kardiyoloji, ABD, 06100 Sihhiye, Ankara, Turkey. Tel.: +90 310 33 33/2523 (work), +90 312 225 30 46 (home); fax: +90 312 312 52 51. sblturhan{at}yahoo.com
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Abstract
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Quadricuspid aortic valve is a rare congenital malformation
of the aortic valve. Most cases are discovered incidentally
at autopsy or at aortic valve replacement. Recent advances in
echocardiography epecially transesophageal echocardiography
led to diagnosis of more cases before surgery. Two cases of
quadricuspid aortic valve are presented.
Keywords: Quadricuspid aortic valve; Atrial septal defect; Ventricular septal defect; Pulmonary valve stenosis; Bicuspid pulmonary valve
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Case 1
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A 31-year-old man presented with a history of dyspnea on exertion
that began seven years ago. Physical examination showed central
cyanosis, clubbing and a 4/6-grade systolic ejection murmur
at all areas accompanied by a thrill. Transthoracic echocardiography
(TTE) showed biventricular enlargement, right ventricular hypertrophy,
and a large perimembranous ventricular septal defect (VSD).
A maximum gradient of 107mmHg was present across the pulmonary
valve. Transesophageal echocardiography revealed a large perimembranous
VSD, secundum type atrial septal defect (ASD) and a quadricuspid
aortic valve (QAV). Aortic valve cusps were equal in size, opening
was normal. There was mild aortic regurgitation. The thicknesses
of the pulmonary valves were increased, with a limitation in
the movement. Cardiac catheterization confirmed the diagnosis.
The patient was referred to surgery. VSD and ASD were repaired.
As the pulmonary valve turned up to be bicuspid and stenotic,
the operations valvotomy, myectomy and myotomy were performed.
Surgery was not performed for the aortic valve.
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Case 2
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A 24-year old man was admitted with dyspnea. His physical examination
was normal. TTE showed a QAV with four equal cusps on both systolic
and diastolic frames (
Figs. 1 and 2
).
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Figure 2 Diastolic transthoracic echocardiographic view of quadricuspid aortic valve with four equal cusps in Case 2. On the short axis view of the aortic valve, the commissural lines formed by the adjacent cusps resulted in an "X" configuration rather than the "Y" configuration of the normal tricuspid aortic valve.
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Autopsy series have reported an incidence of QAVs between 0.008%
and 0.033%.
1 QAVs may be isolated or associated with other congenital
cardiac abnormalities. A case of quadricuspid pulmonary valve
and bicuspid aortic valve has been reported.
2 The cause of the
combined abnormalities might be both an abnormality of mesenchymal
proliferation in common trunk and aberrant fusion of the aortopulmonary
septum. To our knowledge this is the first case of complex cardiac
abnormality where QAVs are associated with ASD, VSD, pulmonary
valve stenosis, and bicuspid pulmonary valve.
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References
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- Hurwitz L.E., Roberts W.C. Quadricuspid semilunar valves. Am J Cardiol (1973) 31:623–626.[CrossRef][Web of Science][Medline]
- Hirooka K., Hashimoto S., Tanaka N., Yamada N., Masuda Y., Hanatani A., et al. Combined abnormalities of semilunar valves: quadricuspid pulmonary and bicuspid aortic valves. Circulation (2001 Jan 2) 103(1):E7.
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Abstract
Case 1