DOI:10.2214/AJR.07.2936
AJR 2008; 190:W294-W303
© American Roentgen Ray Society
Cardiac Valve Disease: Spectrum of Findings on Cardiac 64-MDCT
Ronan Ryan1,
Suhny Abbara2,
Rivka R. Colen2,
Samer Arnous3,
Martin Quinn2,
Ricardo C. Cury3 and
Jonathan D. Dodd1
1 Department of Radiology, Cardiac CT/MRI Program, St. Vincent's University
Hospital, Elm Park, 24 Castledawson, Sion Hill, Blackrock, Dublin 4,
Ireland.
2 Cardiac MRI-PET-CT Program, Massachusetts General Hospital and Harvard Medical
School, Boston, MA.
3 Department of Cardiology, Cardiac CT/MRI Program, St. Vincent's University
Hospital, Dublin, Ireland.
Received July 25, 2007;
accepted after revision October 6, 2007.
Address correspondence to J. D. Dodd
(j.dodd{at}st-vincents.ie).
WEB
This is a Web exclusive article.
Abstract
OBJECTIVE. Recent studies have established that cardiac MDCT
generates high-quality images of the cardiac valves. Images are acquired
during a single breath-hold (inspiration) after the injection of iodinated
contrast material (5 mL/s) followed by a saline bolus chaser. Incremental data
sets are then reconstructed throughout the R-R interval, and after transfer to
a workstation, specialized software combines data sets sequentially to
generate cine loops of the heart throughout the cardiac cycle. The purpose of
this article is to describe the cardiac MDCT techniques allowing optimal
cardiac valve depiction and to illustrate the MDCT appearances of the most
important valve diseases.
CONCLUSION. Cardiac MDCT provides an excellent imaging method for
illustrating cardiac valve disease. Radiologists should be aware of the
various appearances of the common and most important cardiac valve diseases on
cardiac MDCT.
Keywords: cardiac imaging cardiac valve disease congenital heart disease coronary artery disease hemodynamics MDCT
Introduction
In 2006, the total estimated mortality attributable to cardiac valve
disease in the United States was 19,989 deaths
[1]. Aortic valve disease
accounted for 12,471 deaths, and mitral valve disease for 2,759 deaths, with
the remainder divided evenly between diseases of the tricuspid and pulmonary
valves. Echocardio graphy and MRI are the principally used imaging techniques
for evaluation of the heart
[2]. Echocardiography is widely
available and is cost-effective, but it is user-operator-dependent and some
patients may have poor acoustic windows
[3]. Transesophageal
echocardiography (TEE) is invasive and has several contraindications (i.e.,
recent esophageal surgery, recent oral ingestion, un stable cervical spine
injuries, unevaluated gastrointestinal bleeding). Cardiac MRI provides high
temporal and spatial resolution images of the cardiac valves without ionizing
radiation, but it is expensive, is time-consuming, and also has several contra
indications (e.g., pacemakers, implantable defibrillators, claustrophobia)
[4].
Cardiac MDCT is an emerging technique in noninvasive cardiac imaging
[5]. Using data recorded during
the cardiac cycle, it is possible to reconstruct multiple incremental data
sets throughout the R-R interval
[6]. These data sets can be
sequentially combined to provide functional imaging in a cine loop that allows
evaluation of valvular leaflet morphology and function. Cardiac MDCT is
becoming a useful first-line investigation in patients with suspected coronary
artery disease (CAD), and as a result, in our experience, many patients are
referred without prior echo cardio graphy or cardiac MRI
[7]. Thus, it is of increasing
importance that radiologists are aware of and can recognize the most important
cardiac valve diseases. The purpose of this article is to describe the cardiac
MDCT techniques that allow optimal depiction of the cardiac valves and to
review the imaging appearances of the most important cardiac valve
diseases.
64-MDCT Protocol for Cardiac Valve Evaluation
With 64-MDCT, acquisition parameters include a collimation of 0.6 mm,
gantry rotation time of 330 milliseconds, pitch of 0.2, tube voltage of 120
kVp, and tube current of 800 mAs. Images are reconstructed using a small field
of view of 12–16 cm to improve spatial resolution, and scanning ranges
from the carina to the apex of the heart. In our center, a timing bolus
technique is used: 20 mL of contrast material is injected at 5 mL/s with a
region of interest placed over the ascending aorta to ascertain the optimum
time to peak enhancement. Images are then acquired during a single breath-hold
in mid inspiration after the administration of 80–90 mL (depending on
the scanning range) of iodinated contrast material (iodixanol [Visipaque 370,
GE Healthcare]) at 5 mL/s followed by a 30-mL saline bolus chaser. Images are
usually acquired in a craniocaudal direction.
Coronary data sets are reconstructed at between 60% and 70% of the R-R
interval because that is generally the phase with the least amount of cardiac
motion (Fig. 1A). However,
incremental data sets may be reconstructed throughout the R-R interval
[8]
(Fig. 1B). For cardiac valve
evaluation, generally 10 image data sets are reconstructed at 10% intervals.
Some centers reconstruct 20 image data sets at 5% increments to further
improve temporal resolution, although that protocol requires increased data
storage space [8]. Images are
reconstructed using 1-mm slices and a 512 x 512 matrix.

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Fig. 1A —ECG-rhythm strip in 52-year-old woman undergoing cardiac MDCT
for suspected coronary artery disease. See Figure S1C in supplemental data.
For coronary artery evaluation, data sets are usually reconstructed at
60–70% (arrow) of each R-R interval, which is portion of
cardiac cycle with least amount of motion. Note pulse of 50–53 beats per
minute after β-blocker administration, which improves image quality.
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Fig. 1B —ECG-rhythm strip in 52-year-old woman undergoing cardiac MDCT
for suspected coronary artery disease. See Figure S1C in supplemental data.
For cardiac valve evaluation, multiple data sets (gray bars) are
reconstructed in 10% increments, commencing at 0% and ending at 90% of each
R-R interval.
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Perpendicular plane images are superior to parallel plane images for
depicting the mitral and tricuspid valve structures for all cardiac phases
[8]. For the aortic and
pulmonary valves, a cross-sectional image plane perpendicular to the valve
cusps provides optimal depiction
[9]. After transfer of the data
sets to a workstation, specialized software adds data sets together
sequentially to generate cine loops of the heart throughout the cardiac cycle,
as shown in Figure S1C; this cine CT, and the others mentioned in this
article, can be viewed from the information box in the upper right corner of
the article at
www.ajronline.org.
Volume-rendering techni ques may also be used to produce endoluminal views of
the valve leaflets and provide high-resolution 3D imaging of the valve
apparatus.
Mitral Valve
Normal Findings
The normal mitral valve is a bileaflet structure with an ovoid orifice. The
anterior leaflet tends to be more mobile and thicker than the posterior
leaflet. The leaflets shows complex movements during the cardiac cycle:
Initially, passive opening is followed by rapid, maximal opening with atrial
contraction; then, partial closure at end-diastole; and, finally, complete
closure from atrial inflow deceleration and ventricular contraction.
Cardiac MDCT provides highly accurate depiction of the normal mitral valve
apparatus (Figs. 2A,
2B, and S2C, cine CT at
www.ajronline.org).
In a recent study of 37 patients with normal mitral valves, image quality was
excellent for depicting the mitral valve leaflets, apposition point,
commissures, and annulus
[8].

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Fig. 2A —Cardiac MDCT in 48-year-old woman being evaluated for
suspected coronary artery disease. See Figure S2C, cine CT, in supplemental
data. Optimal image plane for normal mitral valve is three-chamber long-axis
view. Image at 55% of R-R interval (diastolic phase) shows normal opening of
anterior and posterior mitral leaflets (straight arrows) into left
ventricle. Attachments to commissures are clearly depicted. Anterolateral and
posteromedial papillary muscles (curved arrows) and chordae tendineae
(arrowheads) can be clearly seen attached to leaflet tips. Aortic
valve is closed as expected.
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Fig. 2B —Cardiac MDCT in 48-year-old woman being evaluated for
suspected coronary artery disease. See Figure S2C, cine CT, in supplemental
data. Image at 5% of R-R interval (systolic phase) shows complete coaptation
of leaflets (thin straight arrows). Chordae tendineae
(arrowhead) and papillary muscles (curved arrows) remain
well visualized in ventricular systole. Aortic valve (thick straight
arrow) is open as expected.
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Mitral Stenosis
Mitral stenosis is a narrowing of the inlet valve of the left ventricle
that leads to inadequate diastolic filling. The normal mitral valve orifice
measures between 4 and 6 cm2. Narrowing to < 2.5 cm2
impedes the free flow of blood into the left ventricle, and when reduced to
< 1 cm2, severe mitral sten osis results
[10]. Most cases are secondary
to rheumatic heart disease. Less common causes include tumor or thrombus
prolapse, infective endocarditis, severe mitral annular calcification (Figs.
3 and S3, cine CT at
www.ajronline.org),
systemic lupus erythematosus (SLE), rheumatoid arth ritis, and pulmonary
carcinoid tumors.

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Fig. 3 —Cardiac MDCT in 68-year-old man being evaluated for suspected
coronary artery disease. Three-chamber view shows severe mitral annular
calcification involving posterior commissure (curved arrow) impeding
normal movement of posterior mitral leaflet, resulting in restricted mitral
valve orifice (straight arrow). Note calcification also of aortic
cusps. See Figure S3, cine CT, in supplemental data.
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The prevalence of mitral stenosis from rheumatic fever is declining in the
developed world because of antibiotic therapy
[11]. Rheumatic heart disease
is the most serious complication of rheumatic fever and follows pharyngitis
with group A β-hemolytic Streptococcus pyogenes. Approximately
40% of patients develop isolated mitral stenosis. Conversely, rheumatic
involvement is present in 99% of stenotic mitral valves excised during mitral
valve replacement.
Less common causes of mitral stenosis include tumors, such as left atrial
myxoma or thrombus prolapsing into the mitral orifice
[12]. Cardiac myxoma is a
benign neoplasm and is the most common primary tumor of the heart. Clinical
presentation depends on the location and size of the tumor and on its tendency
to cause embolism.
Radiologic Assessment
Messika-Zeitoun et al. [13]
prospectively compared 2D echocardiography with cardiac MDCT in 29 patients
with a spectrum of severity of mitral stenosis. They found that correlation
between mitral valve area assessed by cardiac MDCT and echocardiography was
excellent (r = 0.88, p < 0.0001), with a small mean
absolute difference between the two techniques (0.20 ± 0.17
cm2). Characteristic features of mitral stenosis on cardiac MDCT
include thickening and calcification of the leaflets and narrowing of the
orifice during diastole (Fig.
4). The left atrium can be enlarged, although often not to the
extent seen in mitral regurgitation.

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Fig. 4 —Cardiac MDCT in 68-year-old man being evaluated for atypical
chest pain and dyspnea. Multiplanar reformat four-chamber image during
ventricular systole shows marked thickening of anterior leaflet of mitral
valve (arrow) and severely restricted opening of mitral leaflets.
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Characteristic features of myxoma on cardiac MDCT include a well-defined
spherical or ovoid intracavitary lesion with smooth contours, classically
arising in the left atrium from a small pedicle attached to the fossa ovalis
of the interatrial septum (Figs.
5A,
5B, and S5C, cine CT at
www.ajronline.org).
Tumor enhancement is usually homogeneous, although heterogeneous atten uation
may reflect hemorrhage, necrosis, or calcification
[14].

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Fig. 5A —Cardiac MDCT in 53-year-old man being evaluated for coronary
artery disease before tumor resection. See Figure S5C, cine CT, in
supplemental data. Multiplanar reformat three-chamber long-axis image shows
low-density, oval, well-circumscribed mass (straight arrow) in left
atrium. Note characteristic attachment to interatrial septum. Mitral valve
leaflets (curved arrow) are closed indicating ventricular
systole.
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Fig. 5B —Cardiac MDCT in 53-year-old man being evaluated for coronary
artery disease before tumor resection. See Figure S5C, cine CT, in
supplemental data. Four-chamber view shows prolapse of mass (straight
arrow) through mitral valve orifice into left ventricle. Widely open
mitral valve leaflets (curved arrows) indicate ventricular
diastole.
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Mitral Regurgitation
Mitral regurgitation occurs from incomplete coaptation of the mitral valve
leaflets, allowing backward blood flow into the left atrium. The most common
causes include chordae tendineae abnormalities in congenital mitral valve
prolapse (MVP); chordae tendineae or papillary muscle rupture from trauma,
infection, or myocardial infarction (Figs.
6A,
6B, and S6C, cine CT at
www.ajronline.org);
myxoid degeneration of the leaflets (Figs.
7 and S7, cine CT at
www.ajronline.org);
and mitral annular dilatation from left ventricular dilatation
(Fig. 8). Less commonly,
infective endocarditis may cause perforation of the leaflets.

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Fig. 6A —Cardiac MDCT in 36-year-old man with known mitral valve
prolapse who developed sudden-onset severe dyspnea. See Figure S6C, cine CT,
in supplemental data. Multiplanar reformat three-chamber long-axis image in
ventricular systole shows prolapse of posterior leaflet of mitral valve
(arrow) into left atrium.
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Fig. 6B —Cardiac MDCT in 36-year-old man with known mitral valve
prolapse who developed sudden-onset severe dyspnea. See Figure S6C, cine CT,
in supplemental data. Multiplanar reformat three-chamber long-axis image shows
no attachment of chordae tendineae (arrow) of posteromedial papillary
muscle to posterior leaflet. Findings were confirmed on transesophageal
echocardiography (not shown).
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Fig. 7 —Cardiac MDCT in 42-year-old woman being evaluated for
coronary artery disease before mitral valve surgery for mitral regurgitation.
Multiplanar reformat three-chamber long-axis image in ventricular systole
shows thickened anterior and posterior leaflets (straight arrows).
Posterior leaflet shows slight prolapse into left atrium. Tips of posterior
leaflet (curved arrow) do not coapt fully, consistent with mitral
regurgitation. See Figure S7, cine CT, in supplemental data.
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Fig. 8 —Cardiac MDCT in 68-year-old woman being evaluated for
coronary artery disease. Multiplanar reformat three-chamber long-axis image in
ventricular systole shows incomplete coaptation of mitral leaflets
(straight arrow), consistent with mitral regurgitation. Left
ventricle is dilated, and apex (curved arrow) is thin and partly
calcified, consistent with chronic myocardial infarction in left anterior
descending vascular territory. Appearances are consistent with
ischemia-induced mitral annular dilatation.
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MVP is the most common cause of mitral re gurgitation and occurs when the
valve leaflets "billow" backward into the atrium during systole
(Figs. 9 and S9, cine CT at
www.ajronline.org).
The cause of MVP is currently thought to be an underlying defect of connective
tissue, supported by the fact that MVP is a prevalent feature in several
connective tissue diseases including Marfan syndrome and Ehlers-Danlos
syndrome. Deposition of mucoid material within the valve leaflets and
associated structures causes myxomatous degeneration. Lengthening of the
chordae tendineae is a characteristic feature, and the posterior leaflet is
most commonly affected.

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Fig. 9 —Cardiac MDCT in 52-year-old woman being evaluated for
coronary artery disease before surgery for mitral valve prolapse. Multiplanar
reformat three-chamber long-axis image in ventricular systole shows severe
prolapse of posterior mitral leaflet (straight arrow) into left
atrium. There is incomplete coaptation of mitral leaflets (curved
arrow), consistent with mitral regurgitation. See Figure S9, cine CT, in
supplemental data.
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Radiologic Assessment
In the acute setting, rapid left atrial and ventricular volume overload
results in acute severe left heart failure and acute pulmonary edema. In the
chronic setting, adaptation of the left atrium and the left ventricle to
volume overload allows left atrial and left ventricular dilatation and less
severe pulmonary vascular congestion and rarely, if ever, pulmonary
hypertension. Alkadhi et al.
[15] recently prospectively
assessed 19 patients with mitral regurgitation with cardiac MDCT, TEE, and
surgery. Cardiac MDCT proved highly accurate in depicting mitral annulus
calcification, thickened leaflets, thickened tendinous chords, and leaflet
prolapse, but four ruptured chords were missed on MDCT. Planimetric
regurgitant orifice area depicted with MDCT correlated well with the severity
of mitral regurgitation on TEE and ventriculography (r = 0.81 and
0.92, respectively).
Aortic Valve
Normal Findings
The normal aortic valve is a trileaflet structure. It shows leaflet closure
at the midpoint of the aortic root and opening throughout systole to the walls
of the aortic root.
Cardiac MDCT provides excellent image quality of the aortic valve apparatus
(Figs. 10A,
10B, and S10C, cine CT at
www.ajronline.org).
Image quality and the widest opening of the cusps depend on the phase of
reconstruction. One study found the largest aortic opening occurred at 50
milliseconds after the R wave and that image quality for the aortic valve was
optimum in the midsystolic phases (50–150 milliseconds)
[16].

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Fig. 10A —Cardiac MDCT in 65-year-old woman being evaluated for
coronary artery disease. See Figure S10C, cine CT, in supplemental data. N =
noncoronary cusp, R = right coronary cusp, L = left coronary cusp. Multiplanar
reformat cross-section image across aortic cusps in ventricular diastole shows
normal tricuspid aortic valve apparatus that forms characteristic
"Mercedes-Benz" appearance. Cusps show normal complete coaptation
in center of valve orifice (arrow).
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Fig. 10B —Cardiac MDCT in 65-year-old woman being evaluated for
coronary artery disease. See Figure S10C, cine CT, in supplemental data. N =
noncoronary cusp, R = right coronary cusp, L = left coronary cusp. Image in
ventricular systole shows wide and symmetric opening of all three cusps
(arrows).
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Aortic Stenosis
Aortic stenosis is a narrowing of the aortic valve orifice. The area of a
normal aortic valve measures between 3 and 4 cm2. Planimetric
measurement of aortic valve area of less than 2 cm2 is clinically
significant and less than 0.8 cm2 defines critical stenosis. In
young patients, the most common causes are congenital bicuspid aortic valves
and rheumatic fever [2]. Rarely
but importantly, the ob structive form of hypertrophic cardio myopathy may
cause subvalvular aortic steno sis due to systolic anterior motion of the
anterior mitral valve leaflet (Figs.
11A,
11B, and S11C, cine CT at
www.ajronline.org).

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Fig. 11A —Cardiac MDCT in 44-year-old man being evaluated for atypical
chest pain. See Figure S11C, cine CT, in supplemental data. Multiplanar
reformat three-chamber long-axis image in ventricular diastole shows
asymmetric hypertrophy of left ventricular septal myocardium
(arrows).
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Fig. 11B —Cardiac MDCT in 44-year-old man being evaluated for atypical
chest pain. See Figure S11C, cine CT, in supplemental data. During ventricular
systole, there is systolic anterior motion of anterior mitral valve leaflet
(curved arrow) with obstruction of left ventricular outflow tract
(straight arrow).
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Bicuspid aortic valve is the most common congenital cardiac malformation
and results from complex abnormal cusp formation during valve development. In
contrast to the degenerative form of aortic stenosis, aortic stenosis from
bicuspid aortic valve presents in young individuals.
In older patients, the most common causes of aortic stenosis include de
generative senile calcification of a morpho logically normal valve and
infective endo carditis [17].
Degenerative (senile) calcific stenosis is thought to develop secondary to
normal "wear and tear" from hemodynamic injury and usually
manifests in the seventh to eighth decades (Figs.
12 and S12, cine CT at
www.ajronline.org).
The degree of calcification is the strongest independent risk factor for dis
ease pro gression and an ad verse clinical out come
[18]. The severity and
location of aortic valve calcification are associated with increased pressure
gradient across the aortic valve
[19].

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Fig. 12 —Cardiac MDCT in 82-year-old man being evaluated for chest
pain. Multiplanar reformat cross-section image across aortic cusps in peak
ventricular systole shows degenerative aortic valve disease with thickened
calcified cusps (arrows) and restricted opening. See Figure S12, cine
CT, in supplemental data.
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Radiologic Assessment
Bouvier et al. [20]
recently studied 30 patients with aortic stenosis. Good agree ment was shown
between cardiac MDCT-based aortic valve area and echo cardiography (mean
difference on Bland-Altman analysis of –7 mm2). Similarly,
Alkadhi et al. [21] evaluated
the aortic valve of 20 patients and 20 control subjects using cardiac MDCT and
compared the findings with echocardiography. Significant corre lations were
found between aortic valve area as depicted on cardiac MDCT and both TEE and
transvalvular gradient (r = 0.99, p < 0.001 and 0.74,
p < 0.01, respectively). In the absence of the normal three-cusp
appearance, a bicuspid aortic valve, which shows two symmetric aortic cusps
(Figs. 13A,
13B, and S13C cine CT at
www.ajronline.org),
can be easily diagnosed on cardiac MDCT. During systole, the open bicuspid
aortic leaflets assume an ellipsoid shape.

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Fig. 13A —Cardiac MDCT in 42-year-old man being evaluated for atypical
chest pain. See Figure S13C, cine CT, in supplemental data. Multiplanar
reformat cross-section image across aortic cusps in ventricular systole shows
two cusps (arrows) that do not open; this finding is fully consistent
with congenital bicuspid valve and aortic stenosis.
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Fig. 13B —Cardiac MDCT in 42-year-old man being evaluated for atypical
chest pain. See Figure S13C, cine CT, in supplemental data. Endoluminal view
provides higher definition than A of congenital bicuspid valve cusps
(arrows).
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Aortic Regurgitation
Aortic regurgitation is the diastolic flow of blood from the aorta into the
left ventricle. Incompetence of the aortic valve or any disturbance of the
valvular apparatus results in aortic regurgitation. In acute regurgitation,
the left ventricle has no time to adapt, whereas in chronic regurgitation,
hypertrophy of the left ventricle allows some adaptation and clinical
presentations may be less severe
[22]. The most common causes
of aortic regurgitation include rheumatic heart disease and infective endo
carditis. Less commonly, it may be caused by dilatation of the aortic root due
to a connective tissue disease, such as Marfan syndrome, or may be secondary
to aortitis; occasionally, it may be caused by aortic dissection when the
dissection flap extends proximally down to the valvular annulus.
Infective endocarditis is an infection of the endocardial surface of the
heart, most commonly the valves. Many patients have a preexisting underlying
valve condition, such as a bicuspid aortic valve (Figs.
14 and S14, cine CT at
www.ajronline.org),
predisposing to the development of infective endocarditis. Streptococcus
viridans is the most common organism. In the acute setting, vegetations
can be characteristically depicted on the ventricular side of the aortic valve
in the direction of intracardiac blood flow (Figs.
15 and S15, cine CT at
www.ajronline.org).

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Fig. 14 —Cardiac MDCT in 41-year-old man with sepsis after ascending
aortic graft repair for ascending aortic aneurysm. Multiplanar reformat
cross-section image across aortic cusps in ventricular systole shows two cusps
(thin straight arrows), consistent with bicuspid aortic valve. Large
vegetation (curved arrow) is noted on anterolateral commissure.
Infection had eroded through aortic graft resulting in extravasation and
perigraft hematoma (thick straight arrows). See Figure S14, cine CT,
in supplemental data.
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Fig. 15 —Cardiac MDCT in 38-year-old woman being evaluated for
coronary artery disease before surgery. Multiplanar reformat three-chamber
long-axis image in ventricular diastole shows flail aortic cusp (curved
arrow) with complete loss of coaptation. Note small vegetations (thin
straight arrows) on two aortic cusps. Small outpouching (thick
straight arrow) on aortic wall distal to valve represents surgically
proven mycotic aneurysm secondary to infected embolus that impacted in wall.
See Figure S15, cine CT, in supplemental data.
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Radiologic Assessment
Feuchtner et al. [23] found
the sensitivity and specificity of cardiac MDCT in detecting aortic
regurgitation was 81% and 91%, respectively, when compared with Doppler trans
thoracic echocardiography (TTE). MDCT results were better for moderate to
severe aortic regurgitation, and MDCT showed a decreased diagnostic accuracy
for mild aortic regurgitation and valve calci fications. More recently, Jassal
et al. [24] found 64-MDCT to
have a sensitivity of 70%, specificity of 100%, positive predictive value of
100%, and negative predictive value of 79% in detecting aortic regurgitation
when compared with echocardiography. Anatomic area of the regurgi tant orifice
measured by cardiac MDCT correlated well with the TTE-derived vena contracta
(r = 0.79, p < 0.001), the ratio of the jet to the left
ventricular outflow tract (LVOT) height (r = 0.79, p <
0.001), and the ratio of the jet to the LVOT cross-sectional area (r
= 0.75, p < 0.001)
[22].
Tricuspid Valve
Normal Findings
The normal tricuspid valve is a trileaflet structure with anterior, septal,
and posterior leaflets. The latter two are variable in size and shape.
Tricuspid Regurgitation
Tricuspid regurgitation occurs because of incomplete coaptation of the
tricuspid valve leaflets, allowing backward blood flow into the right atrium.
Abnormalities of any atrioventricular valve apparatus components (leaflets,
chordae, annulus, papillary muscles, or adjacent right ventricular muscle) may
cause tricuspid regurgitation. Ebstein's anomaly accounts for the most common
congenital cause. The most common acquired conditions include infective
endocarditis, floppy valve syndrome, and connective tissue disorders such as
Marfan syndrome.
The pathophysiology and morphology of Ebstein's anomaly reflect the
embryologic development of the tricuspid valve. The anterior leaflet develops
first, arising from the mesenchyme. The posterior and septal leaflets arise
through the creation of a diverticulum and resorption of the right ventricular
myocardium. Failure or incorrect myocardium resorption results in apical
displacement of the septal and posterior tricuspid valve leaflets, leading to
atrialization of the inlet of the right ventricle (Figs.
16 and S16, cine CT at
www.ajronline.org)
[25].

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Fig. 16 —Cardiac MDCT of 53-year-old man with Ebstein's anomaly. Axial
image shows atrialization of right ventricle (asterisk). Note apical
displacement of septal leaflet (straight arrow) relative to anterior
mitral leaflet hinge point. Anterior leaflet is tethered to trabeculae of
right ventricular free wall (curved arrow). Note also bowing of
interventricular septum into left ventricular cavity secondary to increased
right heart pressure. See Figure S16, cine CT, in supplemental data.
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Radiographic Assessment
The normal tricuspid valve area measures between 3 and 5 cm2.
Tricuspid regurgitation may be shown on MDCT by incomplete leaflet coaptation
and the presence of reflux of contrast material into the inferior vena cava
(IVC) and hepatic veins during the first pass of contrast material
[26]. The presence of reflux
into the hepatic veins was originally considered highly sensitive and specific
for tricuspid regurgitation at low injection rates (i.e.,
3 mL/s), but
for current cardiac MDCT protocols, this is not the case because of increased
injection rates [27].
Additional described CT abnormalities in Ebstein's anomaly include clockwise
rotation of the heart from the enlarged right heart chambers, dilatation of
the right atrial appendage, and contrast reflux into enlarged IVC and hepatic
veins from tricuspid regurgitation.
Tricuspid Stenosis
Isolated tricuspid stenosis is an uncommon finding. The most common causes
include carcinoid heart disease and SLE. Less commonly, a right atrial tumor
may cause hemodynamic obstruction (Fig.
17); rarely, constrictive pericarditis may cause functional
tricuspid stenosis.

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Fig. 17 —Cardiac MDCT in 42-year-old woman with shortness of breath.
Axial 0.75-mm image shows large mass (arrow) in right atrium
encroaching onto tricuspid valve orifice and causing obstructive tricuspid
stenosis. Mass was surgically confirmed to be atrial myxoma. Note
characteristic interatrial septal attachment.
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Carcinoid tumors are rare neuroendocrine malignancies arising from neural
crest amine precursor uptake decarboxylation cells. Approximately 90% of
tumors are located in the gastrointestinal system and characteristically
metastasize to the liver. In carcinoid syndrome, hepatic metastases overwhelm
hepatic metabolism of tumor products, which are then secreted in high doses
into the hepatic veins. Such substances cause fibrous endocardial plaques,
classically on the tricuspid and pulmonary valves. Cardiac involvement has
been recognized in more than half of patients with carcinoid syndrome.
Radiographic Assessment
Cardiac MDCT in carcinoid syndrome shows thickened, retracted tricuspid and
pulmonary valves, which can be fixed with little movement during the cardiac
cycle [28] (Figs.
18A,
18B, and S18C, cine CT at
www.ajronline.org).
The right atrium and ventricle may be enlarged, and there may be decreased
pulmonary vascularity in severe pulmonic stenosis.

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Fig. 18A — 53-year-old man with known carcinoid syndrome secondary to
liver metastases from ileal primary tumor. See Figure S18C, cine CT, in
supplemental data. Axial 1-mm image shows thickening of tricuspid leaflets
(straight arrow) and chordae tendineae (curved arrow) within
right ventricle. Note enlarged right atrium (asterisk).
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Fig. 18B — 53-year-old man with known carcinoid syndrome secondary to
liver metastases from ileal primary tumor. See Figure S18C, cine CT, in
supplemental data. Axial 1-mm image through liver shows multiple hypervascular
enhancing lesions (arrowheads), consistent with extensive carcinoid
liver metastases.
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Pulmonary Valve
Normal Findings
The pulmonary valve is a trileaflet structure. It is commonly diseased in
patients with congenital disorders but rarely so in those with acquired
disease. Cardiac MDCT allows visualization of the leaflets and assessment of
dilatation of the pulmonary artery. Trivial pulmonary regurgitation is present
in most of the healthy population.
Pulmonary Stenosis
Pulmonic stenosis is most commonly congenital and can be valvular (90%),
subvalvular, or peripheral (supravalvular). Valvular pulmonic stenosis is
typically an isolated anomaly and comprises 10% of all congenital heart
disease. Acquired causes are rare and include carcinoid, rheumatic fever, and
infective endocarditis.
Approximately 10–15% of patients with valvular pulmonic stenosis have
dysplastic pulmonic valves composed of myxomatous tissue. The failure of
normal development of the pulmonic valves at 6–9 weeks' gestation may
result in fusion of two cusps in three leaflets that are thickened and
partially fused at the commissures or in a single coned-shaped valve (Figs.
19A and
19B).

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Fig. 19A — Cardiac MDCT in 33-year-old man with congenital pulmonary
stenosis. Axial 0.75-mm image across pulmonary valve during ventricular
diastole shows normal coaptation of valve leaflets (arrow).
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Fig. 19B — Cardiac MDCT in 33-year-old man with congenital pulmonary
stenosis. During ventricular systole, valve leaflets (arrow) do not
open widely and are thickened. Echocardiogram (not shown) confirmed pulmonary
stenosis.
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Radiographic Assessment
The normal trileaflet pulmonic valve area measures 2
cm2/m2 of body surface area (range, 2.5–4.0
cm2). Mild pulmonic stenosis is defined as < 1 cm2
and severe as < 0.5 cm2. Classically, the three leaflets are
thin and pliant with partially fused commissures, resulting in a conical or
dome-shaped structure with a narrowed central orifice. Poststenotic pulmonary
artery dilatation may occur owing to jet effect hemodynamics. Hwang et al.
[29] described the chest CT
appearance of severe pulmonic stenosis in a 44-year-old man. Images showed a
calcified peri cardial ring encompassing the aorta and pulmonary trunk at
valve apparatus level causing severe pulmonic stenosis and poststenotic
dilatation of the left pulmonary artery. Cardiac MDCT of patients with
pulmonic stenosis may depict thickened immobile leaflets and a valvular
annulus that is small; the supravalvular area of the pulmonary trunk is often
hypoplastic.
Summary
Cardiac MDCT allows accurate assessment of the cardiac valves. Currently,
MDCT is not considered a first-line invest igation for cardiac valve
evaluation. It is becoming a primary imaging investigation for CAD in certain
patient subgroups. Many patients with CAD will have unsuspected underlying
valvular disease. Thus, radiologists reading cardiac MDCT should to be able to
recognize the most common and most important valve diseases. In addition,
patients with aortic stenosis may present with angina and be referred for
preoperative assessment for CAD. Cardiac MDCT can provide accurate evaluation
of both conditions in a single investigation. Cardiac MDCT may be superior to
echocardiography in patients with heavily calcified valves because of the
limitations of acoustic shadowing. It may also be a useful alternative in
patients with contraindications to TEE.
Several limitations exist with current MDCT scanners. Radiation dose
remains a major concern and contraindicates cardiac MDCT in pregnant patients.
Optimal images are obtained in patients with sinus rhythm, and because
valvular heart disease is commonly associated with arrhythmias, particularly
atrial fibrillation, this may lead to suboptimal image quality.
Conclusion
The role of MDCT continues to expand in the evaluation of cardiac disease.
Showing structures beyond the coronary arteries, MDCT provides an accurate
noninvasive imaging method for valve evaluation. Radiologists should be aware
of the most important cardiac valve diseases and their appearance on cardiac
MDCT.
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