CARDIOVASCULAR JOURNAL OF AFRICA • Vol 21, No 2, March/April 2010
AFRICA
113
Case Report
The unicuspid aortic valve
SHI-MIN YUAN, HUA JING, JACOB LAVEE
Summary
The unicuspid aortic valve is a very rare congenital anomaly,
which usually presents as aortic stenosis, incompetence, or
a combination of both. Other congenital disorders may
accompany this phenomenon and aortic dilatation and left
ventricular hypertrophy are frequent complications. We
present a case report of a young, symptomatic patient with a
unicuspid aortic valve, complicated by dilatation of the aortic
root and ascending aorta, with left ventricular hypertrophy.
The patient recovered fully after a Bentall procedure.
Keywords:
aortic dilatation, echocardiography, unicuspid aortic
valve
Submitted 20/7/09, accepted 13/10/09
Cardiovasc J Afr
2010;
21
: 113–114
The unicuspid aortic valve is a rare congenital cardiovascular
anomaly, which is often misdiagnosed as a bicuspid aortic valve.
1
The true incidence of the unicuspid aortic valve may be underes-
timated in the asymptomatic population.
2
The clinical and diag-
nostic implications of this anomaly have been reviewed before.
2-6
Case report
These images (Fig. 1A–1D) are those of a 32-year-old male who
presented with intermittent chest pain. Clinically, a combined
systolic and diastolic murmur was audible over the left paraster-
nal region. Chest radiography demonstrated a dilated ascending
aorta. Echocardiography additionally revealed a unicuspid aortic
valve – with one raphe and commissure. Severe aortic regurgita-
tion with mild aortic stenosis, resulting in left ventricular hyper-
trophy was also present.
At operation, the aortic valve was unicuspid and severely
regurgitant with an eccentric orifice, with one commissural
attachment at the left- and non-coronary commissural, and one
raphe at the right- and left-coronary commissural positions, with
leaflet thickening and calcification. Aortic dilatation involving
the aortic root and ascending aorta was an additional operative
finding. The patient underwent a Bentall procedure and had an
uncomplicated post-operative course.
Discussion
The unicuspid aortic valve is a rare congenital malformation
seen in 0.019% of patients during echocardiographic evaluation
and in 5.59% of patients during aortic valve replacement.
2,3
The
unicuspid aortic valve can be categorised into two types: acom-
missural pin-hole shaped, and unicommissural slit-shaped.
1
The
acommissural type has no lateral attachment to the aorta with a
central orifice, and the unicommissural type has one attachment
with an eccentric orifice.
3
Patients with a unicuspid aortic valve are always very young
at the time of diagnosis or surgery, ranging from 14 to 75 years
old.
1,4
The typical age of unicuspid aortic valve patients at presen-
tation is the third to the sixth decade, indicating an earlier onset
and a higher rate of progression of aortic stenosis in comparison
to patients with a tricuspid aortic valve. Collins
et al
.
5
have
shown in a retrospective analysis that a decreased number of
aortic cusps are associated with an increased occurrence of
pathological changes of these cusps and the ascending aorta.
Severe aortic stenosis or mixed stenosis and regurgitation
is the predominant disorder that accompanies patients with a
unicuspid aortic valve.
6
Left ventricular dilatation might be
present at the time of diagnosis.
7
Similar to the bicuspid aortic
valve, the unicuspid aortic valve is prone to be associated with
dilatation or dissection of the aorta, involving the aortic root,
8
ascending aorta,
9
or aortic arch,
10
which typically requires surgi-
cal intervention. Other associated disorders include aortic coarc-
tation, an aberrant right subclavian artery,
1
and a single coronary
artery and ventricular septal defects.
8
Recently, magnetic resonance imaging, cardiac computed
tomography, and multislice tomography angiography were also
applied as auxiliary diagnostic tools in such patients by virtue of
their promising assessment of aortic valve morphology, includ-
ing the exact morphology of the aortic valve and the severity of
the aortic stenosis and regurgitation.
1,11,12
However, echocardiog-
raphy remains a reliable method for the pre-operative diagnosis
of a unicuspid aortic valve, preferable to the radiological diag-
nostic tools mentioned above.
Echocardiographic imaging allows diagnostic accuracy of
aortic valve morphology in most patients. The commissural
attachment zone, the valvular orifice, the free edge of the leaflet,
and the configuration of the aortic valve can be clearly visual-
ised. Besides, echocardiography can even distinguish true from
false unicuspid aortic valves.
2
Aortic valve repair, including bicuspidisation, can be
performed with low risk and excellent operative results.
9
Department of Cardiac and Thoracic Surgery, The Chaim
Sheba Medical Center, Tel Hashomer, Israel
SHI-MIN YUAN, MD, PHD
JACOB LAVEE, MD,
Department of Cardiothoracic Surgery, Jinling Hospital,
School of Clinical Medicine, Nanjing University, Nanjing,
Jiangsu Province, People’s Republic of China
SHI-MIN YUAN, MD, PHD
HUA JING, MD,
