We report a rare case of a quinticuspid aortic valve associated with regurgitation and dilation of the ascending aorta, which was diagnosed and post-surgically followed up by cardiovascular magnetic resonance and dual source computed tomography

Lijun Zhang

Xiaoming Yang

Yanfeng Meng

Yongmei Wang

Zhaoqi Zhang

Journal of Cardiovascular Magnetic Resonance

PubMed

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Cardiovascular magnetic resonance of quinticuspid aortic valve with aortic regurgitation and dilated ascending aorta

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BioMed Central
Journal of Cardiovascular Magnetic 
Resonance
ssOpen AcceCase report
Cardiovascular magnetic resonance of quinticuspid aortic valve 
with aortic regurgitation and dilated ascending aorta
Yanfeng Meng1,2, Lijun Zhang*1, Zhaoqi Zhang*1, Yongmei Wang1 and 
Xiaoming Yang2
Address: 1Radiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, PR China  and 2Image-Guided Bio-Molecular 
Interventions Research, Radiology, University of Washington, Seattle, WA 98109, USA
Email: Yanfeng Meng - meng.yanfeng@yahoo.com; Lijun Zhang* - lijunzhang016@yahoo.com; Zhaoqi Zhang* - zhaoqi5000@vip.sohu.com; 
Yongmei Wang - wangyongmei523283@yahoo.com.cn; Xiaoming Yang - xmyang@u.washington.edu
* Corresponding authors    
Abstract
We report a rare case of a quinticuspid aortic valve associated with regurgitation and dilation of
the ascending aorta, which was diagnosed and post-surgically followed up by cardiovascular
magnetic resonance and dual source computed tomography.
Background
Aortic valve malformation is a common congenital car-
diac anomaly in the adult population. Dysfunction of the
malformed aortic valve has been shown to cause signifi-
cant morbidity and mortality. Among the malformations,
bicuspid is the most common form, which has been iden-
tified in 1–2% of population with male predominance
[1]. To our knowledge, an aortic valve containing 5 leaf-
lets (quinticuspid) is very rare [2]. Here we report a case of
quinticuspid aortic valve associated with aortic regurgita-
tion and dilation of the ascending aorta.
Case report
A 24-year-old Chinese man presented with worsening
symptoms of palpitation, chest discomfort and shortness
of breath over a two-year period. There was no prior his-
tory of rheumatic fever or endocarditis. On physical exam-
ination, his pulse was 68 per minute with a blood pressure
of 145/41 mmHg. Subsequently, the patient underwent a
series of imaging studies including echocardiography,
chest X-ray, dual source x-ray computed tomography (CT)
and cardiovascular magnetic resonance (CMR) (Figure 1),
which showed a quinticuspid aortic valve. The CT scan
demonstrated an aortic valve with 5 valve cusps and
slightly thickened leaflet edges. In the systolic phase, the
valve orifice measures 6.3 cm2 in area and opened like a
pentagon, while in the diastolic phase, the valve orifice
measured 1.2 cm2 in area and closed like pentagram (Fig-
ure 1a, b, d, e). Five sinuses of valsalva were identified
with variable sizes, of which the posterior sinus was nota-
bly smaller than all other sinuses. CMR SSFP for the cine
sequence demonstrated incomplete closure of the five
aortic valve cusps, in which a small orifice was found in
diastolic phase (Figure 1d, Additional file 1). In addition,
aortic regurgitation was noted as blood back-flow was
demonstrated from aorta to left ventricle at diastolic
phase (Figure 1f). Phase contrast images demonstrated
the peak flow velocity of regurgitation at 480 cm/s. Fur-
thermore, the patient was found to have an enlarged left
ventricle chamber (484 ml for the end diastolic volume
with a normal range of 77–195 ml, and 270 ml for the end
systolic volume with a normal range of 19–72 ml), and a
dilated ascending aorta (49 mm in diameter; normal
range: < 40 mm) (Figure 1c, f).
Published: 11 August 2009
Journal of Cardiovascular Magnetic Resonance 2009, 11:28 doi:10.1186/1532-429X-11-28
Received: 26 March 2009
Accepted: 11 August 2009
This article is available from: http://www.jcmr-online.com/content/11/1/28
© 2009 Meng et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
Journal of Cardiovascular Magnetic Resonance 2009, 11:28 http://www.jcmr-online.com/content/11/1/28Given the aggravating symptoms of aortic insufficiency
and imaging finding of aortic valve anomaly, the patient
proceeded to receive an aortic valve replacement with a
concurrent Robicsek procedure, a surgery of reinforced
aortoplasty. The aortic root was transected and the mal-
formed aortic valve was replaced with an ON-X25
mechanical valve (ON-X technologies, Inc., Austin, Texas,
USA). A 32 mm artificial vessel was used to enwrap the
aortic root with Robicsek procedure. Postoperative CT and
CMR scans were performed and showed functional artifi-
cial aortic valve with no regurgitations detected (Figure
1g–i).
Discussion
In congenital aortic anomalies, the number of aortic leaf-
lets can deviate from the normal three leaflets. Bicuspid is
the most common form. Uni- and quadricuspids are seen
much less frequently [3-6]. To our knowledge, this is the
first reported case of quinticuspid aortic valve accompa-
nied with detailed imaging studies.
The cause of this type of aortic valve anomaly is unknown.
During embryologic development, the aortic valve nor-
mally develops into three commissures and three leaflets.
If this process fails, the valve may be less than three cusps.
If more than three commissures develop, it would cause a
valve with four or more cusps. Valves with abnormal
numbers may open improperly to cause stenosis, or may
close incompletely resulting in regurgitation. Patients
with these abnormalities may expect to develop hemody-
namically significant complications at some point in their
lifetime. In our case, the patient developed left ventricular
chamber dilation and ascending aorta dilation at a rela-
tively young age of 24 years.
CT and CMR have been demonstrated to be effective
imaging methods for accurate diagnosis of aortic valve
malformation [7-9]. In this case, accurate preoperative
diagnosis of aortic valve abnormality was made easier
with dual source CT and CMR. CT yielded images with
higher spatial and temporal resolution than CMR. We
were able to demonstrate detailed anatomy of the quinti-
cuspid aortic valve and size of each sinus of valsalva. How-
ever, CMR yielded hemodynamic data in addition,
including a detailed assessment of aortic regurgitation
with signal changes from the abnormal blood flow. Both
CT and MR are useful in postoperative follow-up of the
morphology and function of the artificial valve. Although
echocardiography is both sensitive and specific in detect-
ing bicuspid aortic valves, it proved to be less useful in this
case due to poor echocardiographic windows, and did not
identify the 5 closely related leaflets.
In general, patients with known aortic valve anomaly are
closely followed up to monitor the symptoms and signs
relating to aortic stenosis or insufficiency. The goal is to
prevent permanent damage to the left ventricle, or exces-
sive dilation of the ascending aorta leading to aortic dis-
section and aneurysm. Same cases may necessitate
surgical intervention, which commonly include aortic
valve replacement with procedures to manage dilated
ascending aorta. Given the fact that the currently available
valve substitutes are not ideal for young patients, some
choose valve repair instead. Tricuspidization is suitable
for bicuspid and quadricuspid valves [10-12], and bicus-
pidization, for unicuspid aortic valves [13]. Our patient
was successfully managed with aortic valve replacement
with a Robicsek procedure for aortic root narrowing.
Conclusion
In summary, we have illustrated an unusual case of quin-
ticuspid aortic valve in a young adult presented with aortic
regurgitation, left ventricular enlargement, and dilation of
the ascending aorta. This case also demonstrates the value
of CT and CMR in the diagnosis of this condition.
CT and CMR of quinticuspid aortic valve pre- and post-oper-ative examinationFigure 1
CT and CMR of quinticuspid aortic valve pre- and 
post-operative examination. (a) Dual source CT 
revealed malformed aortic valve in diastolic phase. (b) Dual-
source CT showed malformed aortic valve in systolic phase. 
(c) Dilated ascending aorta. (d) CMR revealed malformed 
aortic valve in diastolic phase. (e) CMR showed malformed 
aortic valve in systolic phase. (f) Regurgitation in diastolic 
phase revealed as signal void (arrow). (g) Dual source CT 
showed mechanical valve in systolic phase. (h) Dual source 
CT showed mechanical valve in diastolic phase. (i) There is 
no regurgitation in diastolic phase.Page 2 of 3
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Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
YM was responsible for the analysis and interpretation of
the images, drafting of the manuscript. LZ was responsible
for the generation of images, analysis and reconstruction
of images. ZZ was responsible for the revision of the man-
uscript. YW was responsible for acquirement of images
and provision of clinical information. XY was responsible
for the revision and editing of the manuscript
Authors' information
YM, MD, Fellow of Radiology. LZ, MD, Resident of Radi-
ology. ZZ, MD, Professor and Chairman of Radiology.
YW, MD, Associated professor of Radiology. XY, MD,
PhD, Professor and Director of Image-Guided Bio-Molec-
ular Interventions Research, Radiology
Additional material
Acknowledgements
Authors want to thank Hongxiu Ji, MD, PhD for her editorial assistance.
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Additional file 1
CMR cine of aortic valve movement. the CMR movie showed the move-
ment of the quinticuspid aortic valve in a cardiac cycle.
Click here for file
[http://www.biomedcentral.com/content/supplementary/1532-
429X-11-28-S1.avi]Page 3 of 3
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Cardiovascular magnetic resonance of quinticuspid aortic valve with aortic regurgitation and dilated ascending aorta

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