Note: This copy is for your personal, non-commercial use only.

To order presentation-ready copies for

distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights.

ORIGINAL RESEARCH
Bicuspid Aortic Valve:
Four-dimensional MR Evaluation

n CARDIAC IMAGING
of Ascending Aortic Systolic
Flow Patterns1
Michael D. Hope, MD
Purpose: To use time-resolved three-dimensional phase-contrast
Thomas A. Hope, MD
magnetic resonance (MR) imaging, also called four-
Alison K. Meadows, MD, PhD
dimensional flow MR imaging, to evaluate systolic blood
Karen G. Ordovas, MD flow patterns in the ascending aorta that may predispose
Thomas H. Urbania, MD patients with a bicuspid aortic valve (BAV) to aneurysm.
Marcus T. Alley, PhD
Charles B. Higgins, MD Materials and The HIPAA-compliant protocol received institutional review
Methods: board approval, and informed consent was obtained. Four-
dimensional flow MR imaging was used to assess blood flow
in the thoracic aorta of 53 individuals: 20 patients with a
BAV, 25 patients with a tricuspid aortic valve (TAV), and
eight healthy volunteers. The Fisher exact test was used to
evaluate the significance of flow pattern differences.

Results: Nested helical flow was seen at peak systole in the ascend-
ing aorta of 15 of 20 patients with a BAV but in none of
the healthy volunteers or patients with a TAV. This flow
pattern was seen both in patients with a BAV with a di-
lated ascending aorta (n = 6) and in those with a normal
ascending aorta (n = 9), was seen in the absence of aortic
stenosis (n = 5), and was associated with eccentric sys-
tolic flow jets in all cases. Fusion of right and left leaflets
gave rise to right-handed helical flow and right-anterior
flow jets (n = 11), whereas right and noncoronary fusion
gave rise to left-handed helical flow with left-posterior
flow jets (n = 4).

Conclusion: Four-dimensional flow MR imaging showed abnormal heli-

cal systolic flow in the ascending aorta of patients with a
BAV, including those without aneurysm or aortic stenosis.
Identification and characterization of eccentric flow jets in
these patients may help identify those at risk for develop-
ment of ascending aortic aneurysm.

q
RSNA, 2010

1
From the Department of Radiology, University of California
San Francisco, 505 Parnassus Ave, Box 0628, San
Francisco, CA 94143-0628 (M.D.H., T.A.H., A.K.M., K.G.O.,
T.H.U., C.B.H.); and Department of Radiology, Stanford
University School of Medicine, Stanford, Calif (M.T.A.).
Received August 4, 2009; revision requested September 17;
revision received September 30; accepted October 15; final
version accepted October 28. Supported by a 2008 RSNA
Research Resident Grant. Address correspondence to
M.D.H. (e-mail: michael.hope@radiology.ucsf.edu).

q
RSNA, 2010

Radiology: Volume 255: Number 1—April 2010 n radiology.rsna.org 53

CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns
A
bicuspid aortic valve (BAV) is the
most common congenital heart
defect and may account for more
morbidity and mortality than all other
congenital cardiac malformations com-
bined (1). Patients with a BAV frequently
have dilated ascending thoracic aortas
compared with healthy subjects with a
tricuspid aortic valve (TAV), even when
matched for the degree of aortic stenosis
and regurgitation (2,3). Some investiga-
tors (3,4) have used this data to discount
the role that altered hemodynamics play
in ascending aortic dilation, arguing that
because dilation is out of proportion to
the degree of stenosis and regurgitation,
it must be secondary to nonhemodynam-
ic factors in patients with a BAV. Patho-
logic evidence has been used to support
an alternative theory for aortic dilation,
in which it is postulated that a genetic or
developmental abnormality in the proxi-
mal aortic tissue leads to weakness of the
aortic wall with a BAV (5–7).
There are some hemodynamic and
anatomic observations, however, that
are at odds with the theory that an in-
trinsic wall abnormality explains aortic
dilation. Echocardiographic evaluation
has shown significantly higher peak
aortic velocities and skewing of peak ve-
locities toward the anterolateral aspect
of the ascending aorta in patients with
a BAV compared with matched healthy
subjects with a TAV (8,9). The region
of peripherally elevated peak velocities
correlates with the typical location of
asymmetric aortic dilation seen in pa-
Advances in Knowledge
n Time-resolved three-dimensional
phase-contrast MR imaging, or
four-dimensional flow MR imag-
ing, allows visualization of eccen-
tric systolic flow jets in the
ascending thoracic aorta that
have not been well characterized
with other techniques.
n Four-dimensional flow MR imag-
ing shows markedly abnormal
helical systolic flow in the
ascending aorta of patients with
a bicuspid aortic valve (BAV),
including those without aneu-
rysm or aortic stenosis.
54
tients with a BAV (10). Furthermore,
investigators (11,12) have shown that
the specific segments of dilated aorta
vary with the type of aortic valve leaflet
fusion. This is difficult to explain with
a theory of intrinsic wall abnormality
alone and raises the possibility that ab-
normal flow patterns arising from dif-
ferent aortic valve morphologies may
account for variations in segmental aor-
tic dilation in patients with a BAV.
Time-resolved three-dimensional (3D)
phase-contrast magnetic resonance (MR)
imaging (also know as four-dimensional
[4D] flow MR imaging) is well suited
for evaluation of multidirectional blood
flow velocity data in the thoracic aorta.
Characterization of normal aortic flow
patterns with the 4D technique has
been shown to agree with that with
two-dimensional and 3D phase-contrast
techniques (13–18). Additionally, 4D
flow MR imaging allows characterization
of abnormal secondary blood flow pat-
terns that are not well visualized with
other techniques. For example, 4D flow
MR imaging has been used to uncover
abnormal helical- and vortical-type flow
in aneurysmal ascending thoracic aortas
(17,18). In this study, we used 4D flow
MR imaging to evaluate systolic flow in
the ascending aorta of patients with a
BAV with the goal of uncovering abnor-
mal flow patterns that may predispose
this patient population to aneurysms.
Materials and Methods
M.T.A. receives research funding from
GE Healthcare (Milwaukee, Wis).
The Health Insurance Portability
and Accountability Act–compliant pro-
Implications for Patient Care
n Evaluation of eccentric systolic
flow jets in the ascending aorta
in patients with a BAV can be
achieved in a single free-
breathing acquisition.
n Identification and characteriza-
tion of eccentric flow jets in the
ascending aorta in patients with
a BAV may help identify those at
risk for development of an
ascending aortic aneurysm.
radiology.rsna.org
Hope et al
tocol was approved by the institutional
review board of the University of Cali-
fornia San Francisco, and informed
consent was obtained from all partici-
pants. Time-resolved 3D phase-contrast
MR imaging, or 4D flow MR imaging,
was used to assess thoracic aortic blood
flow in 53 individuals: 20 patients with a
BAV, 25 patients with a TAV (see Table 1
for patient characteristics), and eight
healthy volunteers (age, 30.8 years 6
5.2; one woman, seven men).
The 4D flow MR imaging technique
used has been previously validated
(19,20). Imaging was performed on a
1.5-T system (Signa CV/i, GE Health-
care) (Gmax, 40 mT/m; rise time, 268 mi-
croseconds) by using a radiofrequency-
spoiled gradient-echo pulse sequence
(repetition time msec/echo time msec,
4.6–5/1.7–2; flip angle, 15°; velocity
encoding, 160–200 cm/sec; fractional
field of view, 300 3 270 mm; slab thick-
ness, 78 mm; matrix, 256 3 192 3 30;
spatial resolution, 1.17 3 1.56 3 2.60 mm;
temporal resolution, 74–77 millseconds)
and an oblique-sagittal slab encompass-
ing the thoracic aorta. Imaging was per-
formed with an eight-channel cardiac
coil, respiratory compensation, and ret-
rospective electrocardiographic gating.
Parallel imaging with an acceleration
factor of two was used. A total of 735
heartbeats were required for data ac-
quisition, resulting in imaging times of
Published online
10.1148/radiol.09091437
Radiology 2010; 255:53–61
Abbreviations:
BAV = bicuspid aortic valve
4D = four-dimensional
3D = three-dimensional
TAV = tricuspid aortic valve
Author contributions:
Guarantors of integrity of entire study, M.D.H., C.B.H.;
study concepts/study design or data acquisition or data
analysis/interpretation, all authors; manuscript drafting
or manuscript revision for important intellectual content,
all authors; approval of final version of submitted manu-
script, all authors; literature research, M.D.H., A.K.M.,
C.B.H.; clinical studies, all authors; statistical analysis,
M.D.H.; and manuscript editing, M.D.H., T.A.H., A.K.M.,
T.H.U., C.B.H.
See Materials and Methods for pertinent disclosures.
n Radiology: Volume 255: Number 1—April 2010
CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns Hope et al

Table 1 8–15 minutes (mean, 11 minutes). Four-

Patient Population Characteristics dimensional flow MR imaging was per-
formed after standard cardiac MR imag-
Characteristic Patients with a BAV (n = 20) Patients with a TAV (n = 25) ing in all patients. Clinical indications for
No. of women 8 10 MR evaluation were aortic coarctation
Age (y)* 23.5 6 11.3 29.4 6 19.7 (n = 30), Tetralogy of Fallot (n = 8), and
Aortic coarctation 16 14 ascending aortic aneurysm (n = 7). Prior
Previously repaired 15 13 to visualization, data were corrected for
Tetralogy of Fallot 1 7 Maxwell phase effects, encoding errors
Ascending aorta maximum diameter† due to the gradient field distortions, and
Normal 13 21 effects from eddy currents (21–23).
Dilated 7‡ 4 Corrected velocity data were im-
Aortic insufficiency ported into 3D visualization software
Mild 5 2 (EnSight; CEI, Apex, NC), which enabled
Moderate or severe 1 … the dynamic visualization of complex
Aortic stenosis 4D datasets by providing a variety of
Mild 5 …
data manipulation tools, including two-
Moderate or severe 5 …
dimensional velocity vector fields mapped
Note.—Unless otherwise specified, data are numbers of patients. onto planes of interest, 3D streamlines, and
* Data are means 6 standard deviations. particle traces. Streamlines are imagi-
†
Normal is ,4 cm or ,2.2 cm/m2, dilated is ⱖ4 cm or ⱖ2.2 cm/m2. nary lines that are aligned with the local
‡
One patient had a maximum aortic diameter .5 cm.
velocity vector field at a given moment
in time and provide a 3D perspective of

Figure 1

Figure 1: Normal systolic flow in a patient with a TAV and

normal thoracic aorta dimensions. (a) Four-dimensional flow MR
imaging data in an oblique-sagittal orientation with 3D stream-
lines (color-coded for velocity, see key in b) during peak systole.
Left: from right side of thoracic aorta. Right: from left side of
thoracic aorta. Note smooth trajectory and absence of substantial
secondary flow features. (b) Close-up of area in white box in a.
(c) Vector analysis at sinotubular junction during peak systole.
Left: cross-sectional depiction. Right: sagittal depiciton. Note
relatively central velocity profile. Ant = anterior.

Radiology: Volume 255: Number 1—April 2010 n radiology.rsna.org 55

CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns
Figure 2
Figure 2: Images in a patient with a BAV and a focal ascending aortic aneurysm. (a) MR angiographic and
(b) T1-weighted spin-echo MR images show focal aneurysm of proximal ascending aorta (up to 4.8 cm).
Incidental note is made of mild proximal descending aorta narrowing at site of coarctation repair. Streamline
analysis from (c) right and (d) left sides of thoracic aorta shows dramatic systolic right-handed helical flow in
aortic root.
instantaneous blood flow. Particle traces
integrate flow over time and, thus, in-
corporate the temporal evolution of
velocities (24,25).
Data Collection and Analysis Methods
The number of aortic valve leaflets and
the type of leaflet fusion in patients
56
with a BAV were determined with
transthoracic echocardiography and/or
steady-state free-precession cine MR
imaging (Fiesta; GE Healthcare) (26).
One author (C.B.H., with over 25 years
experience in cardiac imaging) evaluated
four cases for which only limited visu-
alization of the aortic valve was avail-
radiology.rsna.org
Hope et al
able and determination of the fusion
pattern was challenging; two of these
cases were excluded from our study be-
cause the fusion pattern could not be
reliably determined. Degree of aortic
regurgitation and stenosis was assessed
by using echocardiography and was cat-
egorized as nonexistent or trace, mild,
or moderate or severe. Dimensions of
the thoracic aorta were measured by
using MR images in orthogonal planes
at the levels of the sinuses of Valsalva
and sinotubular junction; dimensions
of the middle ascending aorta, at the
level of the right pulmonary artery; and
dimensions of the aortic arch, immedi-
ately proximal to the innominate artery.
On the basis of their maximum ascend-
ing aorta diameter, participants were
placed into one of two groups: normal
(,4 cm or ,2.2 cm/m2) or dilated (ⱖ4
cm or ⱖ2.2 cm/m2) (27–29).
Nested helical systolic flow was de-
fined as greater than 180° curvature of
the majority of high velocity peak sys-
tolic streamlines around slower central
helical flow in the ascending thoracic
aorta and was characterized as either
right- or left-handed. Vector analysis
was performed at peak systole by us-
ing an orthogonal plane at the level of
the sinotubular junction, and eccentric
flow jets were defined as predominantly
peripheral high-velocity vectors and/or
clustering of high-velocity vectors away
from midline into one of four quad-
rants: right-anterior, right-posterior, left-
anterior, and left-posterior. Flow char-
acterization was performed by two
authors (M.D.H. and T.A.H., with 7 and
6 years experience with 4D flow MR
imaging, respectively). A Fisher exact
test was used to evaluate the statistical
significance of flow pattern differences.
Results
Patients with Normal Ascending Aorta
Diameter
Four-dimensional flow MR imaging evalu-
ation of the ascending thoracic aorta
during peak systole revealed no rel-
evant secondary flow features in any
of the healthy volunteers (n = 8) or
patients with a TAV and normal aortic
n Radiology: Volume 255: Number 1—April 2010
CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns
Table 2
Ascending Thoracic Aorta Systolic Flow in Patients with a BAV
Characteristic
Age (y)*
Ascending aorta maximum diameter
Normal (n = 13)
Dilated (n = 7)
Aortic insufficiency
Mild (n = 5)
Moderate or severe (n = 1)
Aortic stenosis
Mild (n = 5)
Moderate or severe (n = 5)
Aortic leaflet fusion
Right-left (n = 15)
Right-noncoronary (n = 5)
Note.—Unless otherwise specified, data are numbers of patients.
* Data are means 6 standard deviations.
dimensions (n = 21). High-velocity sys-
tolic streamlines spanned the ascend-
ing aorta with minimal deviance from
the direction of bulk flow in all of these
participants (Fig 1). Additionally, all of
these participants exhibited some de-
gree of normal skewing of bulk systolic
flow to the right side of the ascending
aorta and slight right-handed twisting
of relatively slow peripheral streamlines
along the left side of the ascending aorta,
which became more pronounced in late
systole.
Patients with a Dilated Ascending Aorta
Ten of 11 patients with dilated ascending
thoracic aorta had abnormal second-
ary flow features. These abnormalities
could be subdivided into two distinct
categories that corresponded to the
presence of a TAV or BAV. Patients with
a TAV with dilated ascending aortas
(n = 4) had an abnormal systolic flow
pattern in which streamlines wrapped
back toward the aortic valve (a “vertical
vortex”). Six of seven patients with a
BAV with dilated ascending aortas had
marked right-handed nested helical sys-
tolic flow (Fig 2).
Patients with a BAV
The nested helical flow demonstrated
at peak systole in patients with a BAV
Radiology: Volume 255: Number 1—April 2010
Nested Helical Flow
Normal Flow (n = 5) Right-handed (n = 11) Left-handed (n = 4)
21.2 6 9.4 28.6 6 11.0 12.3 6 4.7
4 7 2
1 4 2
1 2 2 aortic stenosis, one had mild aortic
… 1 … stenosis, and two had concomitant mild
… 4 1 Of the patients with a BAV with
… 2 3 normal aortic dimensions (n = 13), four
4 11 … helical systolic flow. Five of these nine
1 … 4 patients with nested helical flow were
and a dilated ascending aorta was also
seen in nine of 13 patients with a BAV
and normal aortic dimensions. Overall,
this flow pattern was present in 15 of
the 20 patients with a BAV but in none
of the healthy volunteers or patients
with a TAV. The association between
nested helical systolic flow and pres-
ence of BAV was shown to be significant
(P , .001). No association was found be-
tween aortic coarctation repair (n = 28,
15 of whom had a BAV) or gothic aortic
arch morphology (n = 8, four of whom
had a BAV) and abnormal systolic flow.
Patients with a BAV exhibited normal
or right-handed or left-handed nested
helical systolic flow in the ascending
thoracic aorta (Table 2). Those with
normal systolic streamlines (n = 5) had
systolic flow jets that appeared to be
nearly normal (Fig 3). Their mean age
was 21.2 years 6 9.4 (standard devia-
tion). Four had normally sized aortas,
one had mild aortic insufficiency, and
none had aortic stenosis.
Patients with right-handed nested
helical flow (n = 11) had marked pe-
ripheral skewing of systolic flow jets to-
ward the right-anterior quadrant (Fig 4).
Their mean age was 28.6 years 6 11.0.
The ascending aorta was normally
sized in seven patients and dilated in
four. Seven patients had some degree of
n radiology.rsna.org
Hope et al
aortic valve disease. Five had no aortic
stenosis. All 11 had fusion of the right
and left aortic valve leaflets.
The four patients with left-handed
nested systolic helical flow and left-
posterior flow jets had fusion of the
right and noncoronary leaflets (Fig 5).
Their mean age was 12.3 years 6 4.7.
All had some degree of aortic valve dis-
ease: Three had moderate or severe
aortic insufficiency.
had normal flow and nine had nested
younger than 20 years (ie, 6, 14, 14,
17, and 19 years), and the remaining
four were at least 30 years old (ie, 30,
39, 43, and 45 years).
The patients with a BAV with mod-
erate or severe aortic stenosis (n = 5)
had some aliasing of signal at the vena
contracta, but this did not preclude ac-
curate assessment of the overall flow
pattern.
Discussion
Abnormal systolic helical flow is seen in
the ascending thoracic aorta of patients
with a BAV. Nested helical flow was
demonstrated at peak systole in 75%
of patients with a BAV but in none of
the healthy volunteers or patients with
a TAV. This abnormal helical flow was
always associated with an eccentric sys-
tolic flow jet in the proximal ascending
aorta.
Similar helical flow has been de-
scribed in ascending aortic aneurysms
associated with a BAV (18). We have
replicated this flow pattern in six patients
with dilated ascending aortas. However,
we have also demonstrated the flow pat-
tern in nine patients with normal aortic
dimensions, suggesting that the pattern
is not secondary to a dilated aorta and
may be implicated in the pathogenesis
of aneurysm formation. Of the nine pa-
tients with normal aortic dimensions and
helical flow, four were at least 30 years
old, which provides an argument against
the hypothesis that the abnormal helical
57
CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns
Figure 3
Figure 3: Normal systolic flow in a patient with a BAV and normal thoracic aorta dimensions.
(a) Streamline analysis does not show any substantial secondary flow features. Left: from right
side of thoracic aorta. Right: from left side of thoracic aorta. (b) Vector analysis shows a nearly
normal velocity profile. Left: cross-sectional depiction. Right: sagittal depiction. Ant = anterior.
flow inevitably leads to aneurysm forma-
tion. An alternative theory, which other
investigators have considered (30), is
that intrinsic aortic wall abnormality in
some patients with a BAV predisposes
them to greater dilation in the presence
of abnormal hemodynamic stress, such
as the eccentric systolic flow jets that we
have demonstrated.
The degree and direction of flow jet
eccentricity may be crucial for deter-
58
mining risk of segmental aneurysm for-
mation in patients with a BAV. We have
categorized systolic flow as eccentric on
the basis of qualitative criteria of pe-
ripheral peak systolic vectors and/or
clustering of these vectors into a single
quadrant at the level of the sinotubular
junction. With this qualitative charac-
terization, we have shown that different
aortic leaflet fusions result in different
orientations of flow jets. Right-anterior
radiology.rsna.org
Hope et al
flow jets and right-handed nested heli-
cal flow were seen only in patients with
right-left leaflet fusion (n = 11), whereas
left-posterior flow jets and left-handed
nested helical flow were seen only in
patients with right-noncoronary leaflet
fusion (n = 4).
Investigators have recently uncov-
ered associations between the type of
leaflet fusion and different pathologic
manifestations in patients with a BAV.
The more common fusion of right and
left leaflets, which is seen in greater
than 70% of cases, has been linked to
increased aortic root dimensions and
stiffness, an increased rate of aortic
dilation when not associated with co-
arctation, and more severe of wall
degeneration in the ascending aorta
(11,31–33). On the other hand, fusion
of the right and noncoronary leaflets
has been linked to more rapid progres-
sion of aortic stenosis and regurgita-
tion, a shorter time to valve interven-
tion, and increased dimensions of the
aortic arch (31,34). In one of the most
detailed studies of segmental aortic di-
lation in patients with a BAV, Fazel et al
(12) found right and left leaflet fusion
in all nine patients with disproportion-
ate dilation of the tubular portion of
the ascending aorta and aortic dilation
extending to the arch in seven of eight
patients with right and noncoronary
leaflet fusion.
The underlying mechanism for
these reported variations in disease
presentation with different aortic valve
morphologies is unknown, but the
simplest and oldest theory for aortic
disease associated with a BAV—altered
hemodynamics related to abnormal
valve morphology—should not be ruled
out. Although aortic dilation has been
demonstrated in the absence of aortic
stenosis and regurgitation and been
demonstrated to be out of proportion
to the degree of stenosis and regurgi-
tation when matched with control sub-
jects with a TAV (2,3), altered hemody-
namics are still a viable mechanism for
the development of aortic pathologic
changes.
Specifically, eccentric flow jets that
do not meet criteria for aortic stenosis
and can only be fully characterized with
n Radiology: Volume 255: Number 1—April 2010
CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns Hope et al

Figure 4 ascending aorta. This was beyond the

Figure 4: Right-handed nested helical flow in a patient with normal aortic dimensions and a
BAV involving right-left leaflet fusion. (a) Streamline analysis shows greater than 180° curva-
ture of peak systolic streamlines in a right-handed twist around slower central helical flow. Left:
from right side of ascending thoracic aorta. Right: from left side of ascending thoracic aorta. (b)
Vector analysis shows a right-anterior eccentric systolic flow jet. Left: cross-sectional depiction.
Right: sagittal depiction. Ant = anterior.
4D flow MR imaging should be con-
sidered. They explain the echocardio-
graphic observation of increased peak
systolic velocities in the anterolateral
ascending aorta in patients with a BAV,
which is the location where asymmet-
ric dilation typically occurs (8–10). As
Schaefer et al (11,31) have speculated,
variations in segmental aortic dilation
that correlate with specific valve mor-
phologic changes may be related to
differences in eccentric flow jets that
lead to a differential distribution of wall
shear stress.
The principal limitation of our study
was the lack of follow-up on the patients
with a BAV whom we have identified
as having abnormal systolic flow in the
scope of our study, which was aimed at
identifying and characterizing abnormal
flow patterns in the context of a BAV. To
further evaluate the proposed hypoth-
esis that eccentric flow jets are impli-
cated in ascending aortic dilation, data
documenting increased rates of growth
are needed. Furthermore, quantifica-
tion of degree of jet eccentricity is pref-
erable to the qualitative assessment we
used, but this would require consensus
standards. The high percentage (80%)
of patients with a BAV in our study who
had aortic coarctation likely skewed
our findings, since this presumably in-
creased the number of patients with
right-left leaflet fusion; this fusion type
has been reported in nearly 90% of pa-
tients with BAV in the setting of coarct
(34,35). Our control group of partici-
pants with a TAV was heterogeneous,
comprising both healthy volunteers and
patients after repair of aortic coarcta-
tion and Tetralogy of Fallot. We believe
this to be a strength of our study: Even
in a mixed group of patients with con-
genital heart disease, not a single case
of nested systolic helical flow was iden-
tified, supporting our claim that this
flow pattern is unique to patients with
abnormal aortic valve morphology.
We limited the focus of our study
to peak systolic flow in the ascending
aorta to simplify the issue of normal
versus abnormal flow. Normal flow pat-
terns over the entirety of the cardiac
cycle have been studied (13–18), but
because we found the most convinc-
ing differences in flow at peak systole,
we decided to exclude the rest of the
cardiac cycle from our analysis. In do-
ing so, we may have missed additional
abnormal flow features that could affect
the development of pathologic changes
in the context of a BAV. Additionally, al-
though we did not find nested systolic
helical flow in any of our patients who
had a TAV, a similar flow pattern has
been reported (18,36) in at least two
patients with a TAV, one of whom had
a documented sclerotic aortic valve.
Because this flow pattern appears to
be secondary to eccentric flow jets, it
should be seen with some frequency
in settings other than a BAV, such as

Radiology: Volume 255: Number 1—April 2010 n radiology.rsna.org 59

CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns Hope et al

Figure 5 Bicuspid aortic valves are associated with

aortic dilatation out of proportion to co-
existent valvular lesions. Circulation 2000;
102(19 suppl 3):III35–III39.

4. Nistri S, Sorbo MD, Marin M, Palisi M,

Scognamiglio R, Thiene G. Aortic root
dilatation in young men with normally
functioning bicuspid aortic valves. Heart
1999;82(1):19–22.

5. Bonderman D, Gharehbaghi-Schnell E,
Wollenek G, Maurer G, Baumgartner H,
Lang IM. Mechanisms underlying aortic dil-
atation in congenital aortic valve malforma-
tion. Circulation 1999;99(16):2138–2143.

6. Fedak PWM, Verma S, David TE, Leask

RL, Weisel RD, Butany J. Clinical and
pathophysiological implications of a bicus-
pid aortic valve. Circulation 2002;106(8):
900–904.

7. Niwa K, Perloff JK, Bhuta SM, et al.

Structural abnormalities of great arterial
walls in congenital heart disease: light and
electron microscopic analyses. Circulation
2001;103(3):393–400.

8. Nkomo VT, Enriquez-Sarano M, Ammash

NM, et al. Bicuspid aortic valve associated
with aortic dilatation: a community-based
study. Arterioscler Thromb Vasc Biol
2003;23(2):351–356.

9. Bauer M, Siniawski H, Pasic M, Schaumann

B, Hetzer R. Different hemodynamic stress
of the ascending aorta wall in patients with
bicuspid and tricuspid aortic valve. J Card
Surg 2006;21(3):218–220.

10. Bauer M, Gliech V, Siniawski H, Hetzer R.

Configuration of the ascending aorta in
Figure 5: Left-handed nested helical systolic flow in a patient with normal aortic dimensions patients with bicuspid and tricuspid aor-
and a BAV involving right-noncoronary leaflet fusion. (a) Streamline analysis reveals greater tic valve disease undergoing aortic valve
than 180° curvature of peak systolic streamlines in a left-handed twist around slower central replacement with or without reduction
helical flow. Left: from right side of ascending thoracic aorta. Right: from left side of ascending aortoplasty. J Heart Valve Dis 2006;15(5):
thoracic aorta. (b) Vector analysis shows a left-posterior eccentric flow jet. Left: cross-sectional 594–600.
depiction. Right: sagittal depiction. Ant = anterior. 11. Schaefer BM, Lewin MB, Stout KK, Byers
PH, Otto CM. Usefulness of bicuspid aortic
valve phenotype to predict elastic proper-
ties of the ascending aorta. Am J Cardiol
2007;99(5):686–690.
a sclerotic and/or stenotic TAV. The help identify those at risk for develop-
unique finding related to a BAV is that ment of an ascending aortic aneurysm. 12. Fazel SS, Mallidi HR, Lee RS, et al. The
aortopathy of bicuspid aortic valve disease
eccentric flow jets that cause abnormal
has distinctive patterns and usually in-
helical flow can result from nonstenotic References volves the transverse aortic arch. J Thorac
aortic valves (n = 5). 1. Ward C. Clinical significance of the bicus- Cardiovasc Surg 2008;135(4):901–907.
In conclusion, by using 4D flow MR pid aortic valve. Heart 2000;83(1):81–85.
13. Kilner PJ, Yang GZ, Mohiaddin RH, Firmin
imaging, we have provided evidence 2. Hahn RT, Roman MJ, Mogtader AH, DN, Longmore DB. Helical and retrograde
that markedly abnormal helical flow can Devereux RB. Association of aortic dilation secondary flow patterns in the aortic arch
be seen in the ascending thoracic aorta with regurgitant, stenotic and functionally studied by three-directional magnetic
of patients with a BAV, including those normal bicuspid aortic valves. J Am Coll resonance velocity mapping. Circulation
without aneurysm or aortic stenosis. Cardiol 1992;19(2):283–288. 1993;88(5 Pt 1):2235–2247.
Identification and characterization of 3. Keane MG, Wiegers SE, Plappert T, 14. Klipstein RH, Firmin DN, Underwood SR,
eccentric flow jets in these patients may Pochettino A, Bavaria JE, Sutton MG. Rees RS, Longmore DB. Blood flow patterns

60 radiology.rsna.org n Radiology: Volume 255: Number 1—April 2010

CARDIAC IMAGING: Bicuspid Aortic Valve: Systolic Aortic Flow Patterns
in the human aorta studied by magnetic
resonance. Br Heart J 1987;58(4):316–323.
15. Bogren HG, Klipstein RH, Firmin DN,
et al. Quantitation of antegrade and retro-
grade blood flow in the human aorta by
magnetic resonance velocity mapping. Am
Heart J 1989;117(6):1214–1222.
16. Fogel MA, Weinberg PM, Haselgrove J.
Nonuniform flow dynamics in the aorta of
normal children: a simplified approach to
measurement using magnetic resonance
velocity mapping. J Magn Reson Imaging
2002;15(6):672–678.
17. Markl M, Draney MT, Hope MD, et al.
Time-resolved 3-dimensional velocity map-
ping in the thoracic aorta: visualization of
3-directional blood flow patterns in healthy
volunteers and patients. J Comput Assist
Tomogr 2004;28(4):459–468.
18. Hope TA, Markl M, Wigström L, Alley MT,
Miller DC, Herfkens RJ. Comparison of
flow patterns in ascending aortic aneurysms
and volunteers using four-dimensional mag-
netic resonance velocity mapping. J Magn
Reson Imaging 2007;26(6):1471–1479.
19. Markl M, Chan FP, Alley MT, et al. Time-
resolved three-dimensional phase-contrast
MRI. J Magn Reson Imaging 2003;17(4):
499–506.
20. Bammer R, Hope TA, Aksoy M, Alley MT.
Time-resolved 3D quantitative flow MRI
of the major intracranial vessels: initial
experience and comparative evaluation at
1.5T and 3.0T in combination with paral-
lel imaging. Magn Reson Med 2007;57(1):
127–140.
21. Bernstein MA, Zhou XJ, Polzin JA, et al.
Concomitant gradient terms in phase con-
Radiology: Volume 255: Number 1—April 2010 n radiology.rsna.org
trast MR: analysis and correction. Magn
Reson Med 1998;39(2):300–308.
22. Markl M, Bammer R, Alley MT, et al. Gen-
eralized reconstruction of phase contrast
MRI: analysis and correction of the effect
of gradient field distortions. Magn Reson
Med 2003;50(4):791–801.
23. Walker PG, Cranney GB, Scheidegger MB,
Waseleski G, Pohost GM, Yoganathan AP.
Semiautomated method for noise reduction
and background phase error correction in
MR phase velocity data. J Magn Reson Im-
aging 1993;3(3):521–530.
24. Napel S, Lee DH, Frayne R, Rutt BK. Vi-
sualizing three-dimensional flow with simu-
lated streamlines and three-dimensional
phase-contrast MR imaging. J Magn Reson
Imaging 1992;2(2):143–153.
25. Anderson JD. Computational fluid dynam-
ics. New York, NY: McGraw-Hill, 1995.
26. Gleeson TG, Mwangi I, Horgan SJ, Cradock
A, Fitzpatrick P, Murray JG. Steady-state
free-precession (SSFP) cine MRI in distin-
guishing normal and bicuspid aortic valves.
J Magn Reson Imaging 2008;28(4):873–878.
27. Kersting-Sommerhoff BA, Sechtem UP, Schiller
NB, Lipton MJ, Higgins CB. MR imaging
of the thoracic aorta in Marfan patients. J
Comput Assist Tomogr 1987;11(4):633–639.
28. Roman MJ, Devereux RB, Kramer-Fox R,
O’Loughlin J. Two-dimensional echocar-
diographic aortic root dimensions in nor-
mal children and adults. Am J Cardiol
1989;64(8):507–512.
29. Bireley WR 2nd, Diniz LO, Groves EM, Dill K,
Carroll TJ, Carr JC. Orthogonal measure-
ment of thoracic aorta luminal diameter
using ECG-gated high-resolution contrast-
Hope et al
enhanced MR angiography. J Magn Reson
Imaging 2007;26(6):1480–1485.
30. Ferencik M, Pape LA. Changes in size
of ascending aorta and aortic valve func-
tion with time in patients with congeni-
tally bicuspid aortic valves. Am J Cardiol
2003;92(1):43–46.
31. Schaefer BM, Lewin MB, Stout KK, et al.
The bicuspid aortic valve: an integrated
phenotypic classification of leaflet mor-
phology and aortic root shape. Heart 2008;
94(12):1634–1638.
32. Thanassoulis G, Yip JW, Filion K, et al.
Retrospective study to identify predictors
of the presence and rapid progression of
aortic dilatation in patients with bicuspid
aortic valves. Nat Clin Pract Cardiovasc
Med 2008;5(12):821–828.
33. Russo CF, Cannata A, Lanfranconi M,
Vitali E, Garatti A, Bonacina E. Is aor-
tic wall degeneration related to bicuspid
aortic valve anatomy in patients with val-
vular disease? J Thorac Cardiovasc Surg
2008;136(4):937–942.
34. Fernandes SM, Khairy P, Sanders SP,
Colan SD. Bicuspid aortic valve morphol-
ogy and interventions in the young. J Am
Coll Cardiol 2007;49(22):2211–2214.
35. Ciotti GR, Vlahos AP, Silverman NH.
Morphology and function of the bicuspid
aortic valve with and without coarctation
of the aorta in the young. Am J Cardiol
2006;98(8):1096–1102.
36. Markl M, Harloff A, Föll D, Langer M,
Hennig J, Frydrychowicz A. Sclerotic
aortic valve: flow-sensitive 4-dimensional
magnetic resonance imaging reveals 3 dis-
tinct flow-pattern changes. Circulation 2007;
116(10):e336–e337.
61