Valsalva Sinus Aneurysms: Findings at CT and MR Imaging

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EDUCATION EXHIBIT
99
Valsalva Sinus Aneurysms: Findings at CT
and MR Imaging1
Aliye Ozsoyoglu Bricker, MD • Bindu Avutu, BS • Tan-Lucien H.
Mohammed, MD • Eric E.Williamson, MD • Imran S. Syed, MD
Paul R. Julsrud, MD • Paul Schoenhagen, MD • Jacobo Kirsch, MD
Aneurysms of the Valsalva sinus (aortic sinus) can be congenital or acquired and are rare. They are more common among men than women
and among Asians than other ethnic groups. Nonruptured aneurysms
may be asymptomatic and incidentally discovered, or they may be
symptomatic and manifest acutely with mass effect on adjacent cardiac structures. Ruptured Valsalva sinus aneurysms result in an aortocardiac shunt and may manifest as insidiously progressive congestive
heart failure, severe acute chest pain with dyspnea, or, in extreme cases,
cardiac arrest. Although both ruptured and nonruptured Valsalva
sinus aneurysms may have potentially fatal complications, after treatment the prognosis is excellent. Thus, prompt and accurate diagnosis
is critical. Most Valsalva sinus aneurysms are diagnosed on the basis of
echocardiography, with or without angiography. However, both electrocardiographically gated computed tomography and magnetic resonance (MR) imaging can provide excellent anatomic depiction, and
MR imaging can provide valuable functional information.
©
RSNA, 2010 • radiographics.rsna.org
Abbreviations: ECG = electrocardiography, MIP = maximum intensity projection, SSFP = steady-state free precession
RadioGraphics 2010; 30:99–110 • Published online 10.1148/rg.301095719 • Content Codes:
From the Cleveland Clinic Foundation, Department of Radiology, 9500 Euclid Ave, Cleveland, OH, 44195. Received March 10, 2009; revision
requested May 5 and received May 14; accepted May 20. P.R.J. is a research consultant for Medtronic; E.E.W. is a consultant for GE Healthcare and
Siemens, is a member of an expert advisory committee for Siemens and Bayer, and receives research support from Bayer; all other authors have no
financial relationships to disclose. Address correspondence to A.O.B. (e-mail: brickea2@ccf.org).
1
©
RSNA, 2010
100 January-February 2010
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Figure 1. Normal Valsalva sinuses. (a) Thin-section maximum intensity projection (MIP)
image of the heart (three-chamber view) obtained at ECG-gated CT shows the left ventricular outflow tract, the valve commissures (white arrow), and the sinotubular junction
(black arrows). Sov = Valsalva sinuses. (b) Oblique axial thin-section MIP image obtained
at ECG-gated CT at the level of the aortic valve shows the Valsalva sinuses and the coronary
arteries. LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp.
Introduction
Valsalva sinus aneurysms are rare and can be either congenital or acquired. Congenital aneurysms
may result from localized weakness of the elastic
lamina or an underlying deficiency of normal
elastic tissue. Acquired aneurysms commonly are
caused by infectious diseases such as bacterial endocarditis, syphilis, and tuberculosis; degenerative
conditions such as atherosclerosis and cystic medial necrosis; and injury from deceleration trauma.
Although rare, Valsalva sinus aneurysms are
slightly more common in men and people of Asian
descent than they are in other patient groups.
Both ruptured and nonruptured Valsalva
sinus aneurysms are associated with potentially
fatal complications; however, the prognosis after
treatment is excellent. Thus, it is important to
make a prompt and accurate diagnosis. Most
Valsalva sinus aneurysms are seen at echocardiography, but both electrocardiographically (ECG)
gated computed tomography (CT) and magnetic
resonance (MR) imaging can provide excellent depiction of the relevant anatomy, and MR imaging
can provide valuable functional information.
This article reviews the developmental anatomy of the Valsalva sinuses and describes the
pathogenesis, prevalence, and clinical features
of congenital and acquired Valsalva sinus aneurysms; common complications and current treatment options; and CT and MR imaging findings.
Developmental Anatomy
The Valsalva sinuses are three subtle dilatations of
the aortic root wall that arise between the aortic
valve annulus and the sinotubular ridge. Each sinus is associated with a corresponding right, left,
or noncoronary aortic valve cusp (Fig 1). A true
Valsalva sinus aneurysm occurs above the aortic
valve annulus and must be distinguished from a
prolapsing aortic cusp, which occurs below the
annulus (1). Mature Valsalva sinuses are thought
to allow enough space for the aortic valve leaflets
to open during systole without causing occlusion of the coronary artery ostia or damage to the
valve leaflets from striking the aortic root wall
(Fig 2). Furthermore, currents within the Valsalva
sinuses may promote closure of the aortic valve
leaflets at the end of systole (2,3).
Left and right truncoconal swellings develop
along the inferior end of the truncus arteriosus
during the 5th week of embryogenesis, just before
septation of the truncus into anterior pulmonary
and posterior aortic channels. After septation, each
ventricular outflow tract contains three tubercles,
Teaching
Point
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Figure 2. Normal Valsalva sinuses. Thin-section MIP images obtained at ECG-gated CT
at end diastole (a) and end systole (b) provide three-chamber views of the heart, showing
the sinuses as spaces that help prevent direct contact of the valve leaflets (arrow in b) with
the coronary ostia.
which later form the cusps of the aortic valve and
the main pulmonary artery. During the 5th and
6th weeks of embryogenesis, the Valsalva sinuses
and aortic valve leaflets begin to form. The right
and left main coronary arteries bud simultaneously from their respective developing sinuses. By
the 9th week, formation of the aortic valve leaflets
and Valsalva sinuses generally is complete (2).
Pathogenesis and Prevalence
Valsalva sinus aneurysms, first described in
1839 by Hope et al (4), are rare. They have been
found in 0.09% of 8138 autopsy subjects and in
0.15%–3.5% of patients who underwent open
heart surgery (5–9). These aneurysms are three
to four times as common in men as in women,
and five times as common in Eastern and Asian
countries as in Western countries (6,8–10).
Valsalva sinus aneurysms may be congenital
or acquired. Congenital aneurysms are thought
to result from fundamental localized weakness
of the elastic lamina at the junction of the aortic
media and the annulus fibrosus (11,12). They may
be associated with an underlying deficiency of
normal elastic tissue, such as that found in Marfan
and Ehlers-Danlos syndromes (8,13–15). The
most common cardiac anomalies that occur with
Valsalva sinus aneurysms are ventricular septal defects (30%–60% of patients), aortic insufficiency
(20%–30%), bicuspid aortic valve (10%), and, less
frequently, coronary anomalies (16–20). Right coronary Valsalva sinus aneurysms commonly are associated with supracristal (type I) ventricular septal defects, whereas infracristal (type II) ventricular
septal defects are more common in patients with
an otherwise healthy heart (21). The association
between supracristal ventricular septal defects and
right coronary Valsalva sinus aneurysms is hypothesized to relate to incomplete fusion of the truncal
swellings at the time of the division of the common
truncus from the bulbar septum during the 5th
week of embryogenesis (12,22).
Acquired Valsalva sinus aneurysms often are
associated with conditions that compromise the
elastic connective tissue at the junction of the
aortic media and the annulus. Acquired aneurysms are most commonly caused by infectious
diseases such as bacterial endocarditis, syphilis,
and tuberculosis; degenerative conditions such
as atherosclerosis and cystic medial necrosis; and
injury from deceleration trauma (11,16). In addition, iatrogenic pseudoaneurysms of the sinus
have occurred due to hematoma formation after
aortic valve replacement or removal of aortic
valve calcifications (23,24).
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Table 1
Sites of Rupture among Patients with a Ruptured Valsalva Sinus Aneurysm
Type of Aneurysm
No. of Patients
Ruptured
Into the right ventricle
Into the right atrium
Into the left ventricle
Into the left atrium
Into the right ventricular outflow tract
Into the interventricular septum
Into the pericardium or pericardial space
Into the pulmonary artery
Nonruptured
529 (66)
458 (55.6)
249 (30.3)
17 (2.1)
9 (1.1)
70 (8.5)
13 (1.6)
5 (0.6)
2 (0.2)
266 (34)
Note.—The total number of patients with a ruptured aneurysm at presentation and in whom the sites of rupture were known was 823. Data in parentheses are percentages.
Clinical Features
Teaching
Point
Because they can be either congenital or acquired,
Valsalva sinus aneurysms may manifest at any age
(11). A Web-based search of all clinical and surgical cases of Valsalva sinus aneurysms published
in the English language over the past 10 years
yielded a total of 1121 patients studied in vivo
(7,8,25–75). Of these, 750 (67%) were male and
371 (33%) were female. The mean age at presentation was 35.4 years, and the age range was 4
days to 96 years. Aneurysms originated from the
right coronary sinus in 815 patients (72%), the
noncoronary sinus in 257 patients (22%), and the
left coronary sinus in 66 patients (6%).
The clinical manifestations of Valsalva sinus
aneurysms vary widely. When symptoms are present, they often are related to aneurysm rupture
or mass effect on adjacent cardiac structures
(16). Omitting surgical studies that dealt exclusively with ruptured Valsalva sinus aneurysms,
our search of the literature yielded a total of 795
cases. Of these, 529 (66%) were ruptured at
presentation and 266 (34%) were not (Table
1). Signs or symptoms at presentation were
documented in only 177 of the 795 cases; of
those 177 cases, 25 (14%) were asymptomatic.
Among the remaining 152 cases, the most common clinical sign was cardiac murmur, which
was seen in 86 cases (57%), and the most common symptom was dyspnea, which was present
in 85 cases (56%) (Table 2).
Depending on the size of the aneurysm, the
rapidity with which it ruptures, and the cardiac
chamber with which it communicates, the symptoms of a ruptured Valsalva sinus aneurysm at
presentation may include severe dyspnea, chest
pain, and hemodynamic compromise or insidiously progressive heart failure with fatigue, dyspnea, and volume overload (16,25). In general,
nonruptured aneurysms are asymptomatic more
often than ruptured aneurysms are, and they
may be incidentally found at imaging performed
to evaluate heart murmurs or abnormal cardiac
contours seen on radiographs (11,16,26–28).
Complications
Aortic regurgitation is a common complication
of both ruptured and nonruptured Valsalva sinus
aneurysms and occurs in 30%–50% of patients
(16). Nonruptured Valsalva sinus aneurysms also
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Table 2
Common Signs and Symptoms of Valsalva
Sinus Aneurysms
Sign or Symptom
Murmur
Dyspnea
Chest pain
Palpitations
No. of Patients
86 (57)
85 (56)
47 (31)
18 (12)
Note.—Excluding 28 patients in surgical studies
that dealt exclusively with ruptured aneurysms
and 25 patients who were asymptomatic, the total
number of patients with signs and symptoms of a
Valsalva sinus aneurysm at presentation was 152.
Data in parentheses are percentages.
Teaching
Point
may lead to impaired function of the tricuspid or
mitral valve, depending on the cardiac chamber
into which the aneurysm extends and the proximity of the aneurysm to the valve. Nonruptured
Valsalva sinus aneurysms, depending on their
size and the consequent mass effect on adjacent
cardiac structures, may lead to emergent complications. During the past decade, there were eight
published cases in which a nonruptured aneurysm manifested with myocardial ischemia or
infarct resulting from compression or occlusion
of a coronary artery. There were nine published
cases in which a nonruptured Valsalva sinus aneurysm had occluded or partially obstructed the
right ventricular outflow tract (29–37) and eight
in which a nonruptured Valsalva sinus aneurysm
had dissected into the interventricular septum
(26,38–44) at the time of manifestation.
When Valsalva sinus aneurysms rupture, they
most commonly rupture into the right ventricle,
followed by the right atrium; the consequent
aortocardiac shunt often leads to insidious heart
failure. Among the 1121 cases that we identified
in our general search, the aneurysm was ruptured and the site of rupture was described in
823 cases. Of these, the aneurysm had ruptured
into the right ventricle in 458 cases (55.6%), into
the right atrium in 249 (30.3%), and into the
right ventricular outflow tract in 70 (8.5%). The
aneurysms had ruptured into the left ventricle
in 17 cases (2.1%), the interventricular septum
in 13 (1.6%), and the left atrium in nine (1.1%)
(Table 1). Rupture into the extracardiac space
is rare, but it is generally associated with higher
mortality because it can lead to critical complications, such as cardiac tamponade (11).
Imaging Protocols
At imaging, the criteria for diagnosing a Valsalva
sinus aneurysm include an origin above the aortic
annulus, a saccular shape, and normal dimensions of the adjacent aortic root and ascending
aorta (1). Although angiography is considered the
reference standard for confirming the pres- ence
of a Valsalva sinus aneurysm, most are initially
seen at color Doppler echocardiography. Of 177
individual case reports of Valsalva sinus
aneurysms published over the past 10 years,
the diagnosis was made at echocardiography in
159 cases (90%). Angiography was performed
in 109 cases (62%), CT was performed in 36
cases (20%), and MR imaging was performed
in 19 cases (11%). Although in most cases CT
was performed to confirm echocardiographic
findings, the use of CT to help diagnose Valsalva
sinus aneurysms may become more common as it
becomes increasingly popular in urgent care settings. Das et al (45) described a case in which a
ruptured right coronary Valsalva sinus aneurysm
was missed at echocardiography and discovered
at follow-up imaging with ECG-gated multisection CT. There are two reported cases in which a
Valsalva sinus aneurysm was initially discovered
at chest CT performed to evaluate symptoms
such as dyspnea and atypical chest pain; acute
pulmonary thromboembolism and aortic dissection also were diagnostic considerations. In all
reported cases in which MR imaging was performed, it was to further define cardiac anatomy
in the setting of a known or suspected Valsalva
sinus aneurysm.
Teaching
Point
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Figure 3. Nonruptured
Valsalva sinus aneurysm in
a 53-year-old woman with
palpitation and dyspnea.
(a, b) Oblique coronal (a)
and short-axis oblique (b)
multiplanar reconstructions
obtained at contrast-enhanced
CT with retrospective ECG
gating show a partially thrombosed, nonruptured 2.5-cm
Valsalva sinus (SoV) aneurysm
(arrows) originating from the
noncoronary cusp and extending into the right ventricle
(RV), in close proximity to the
septal leaflet of the tricuspid
valve. (c, d) Axial (c) and
oblique coronal (d) balanced
steady-state free precession
(SSFP) MR images show the
nonruptured Valsalva sinus
(SoV) aneurysm (arrows).
RA = right atrium, RV = right
ventricle.
ECG-gated contrast material–enhanced multisection CT provides much better spatial resolution of cardiac structures than that attainable with
other imaging methods. It also provides detailed
anatomic depiction of Valsalva sinus aneurysms
and surrounding cardiac structures (Figs 3–7).
The high contrast resolution of CT also may make
it possible to delineate an aortocardiac shunt, if
present, and to identify a ruptured aneurysm by
depicting a jet of contrast material extending from
the aneurysm into the adjacent cardiac chamber.
Multiplanar MR imaging with sequences
such as balanced SSFP gradient-echo (“bright
blood” imaging) and half-Fourier acquisition
single-shot turbo spin-echo (“dark blood” imaging) also allows accurate assessment of the origin
and size of Valsalva sinus aneurysms and the
status of the surrounding cardiac and mediastinal anatomy (Figs 3, 4, 8). The advantages
of performing MR imaging in the setting of a
known or suspected Valsalva sinus aneurysm
include the ability to evaluate the left ventricular
hemodynamic pattern, identify aortic regurgitation, and quantify any aortocardiac shunt or turbulent or fistulous blood flow. ECG-gated cine
MR imaging can be performed during a single
breath hold and without exposing the patient to
ionizing radiation or iodinated contrast material,
which is a further fundamental advantage.
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Figure 4. Nonruptured
Valsalva sinus aneurysm in
an 80-year-old man with
chest pain and diaphoresis.
(a, b) Oblique coronal (a)
and oblique axial (b) balanced SSFP MR images show
a 6.7-cm aneurysm (arrows)
originating from the right
coronary cusp and extending
toward the right ventricle. (c,
d) Axial contrast-enhanced
ECG-gated CT images,
obtained with soft-tissue
window settings (c) and narrowed soft-tissue window
settings (d), 3 days after
patch repair, show a small
leak of contrast material (arrowhead in d) extending into
the aneurysm (arrows).
Figure 5. Nonruptured
Valsalva sinus aneurysm in
a 66-year-old man with angina. Oblique coronal (a) and
oblique axial (b) thin-section
MIP images obtained at
ECG-gated CT show a nonruptured 2-cm Valsalva sinus
aneurysm (arrows) arising
from the left coronary sinus.
In a, the left main coronary
artery is seen distal to the
aneurysm, at the level of the
sinotubular junction.
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Figure 6. Nonruptured Valsalva sinus aneurysm in
an asymptomatic 80-year-old man with heart murmur.
Oblique axial (a) and oblique coronal (b) thick-slab volumetric reconstructions obtained at ECG-gated CT and
oblique axial thin-section MIP image (c) obtained at the
level of the sinuses show a nonruptured 2.4-cm Valsalva
sinus aneurysm (arrows). In b, the aneurysm is seen
arising from the right coronary sinus and extending into
the right ventricle (RV). The origin of the right coronary
artery is seen distal to the aneurysm, which arises near
the level of the sinotubular junction (arrowhead in a and
b). LV = left ventricle.
Treatment Options
Teaching
Point
The mean duration of survival after diagnosis of
a ruptured Valsalva sinus aneurysm is 3.9 years
(11, 26). The mainstay of treatment for a ruptured
aneurysm is cardiopulmonary bypass surgery, and
the surgical approach is either via aortotomy, from
the chamber where the aneurysm terminates, or
a combination of the two (16). The aneurysm is
closed with a pericardial or polyester patch or with
sutures (Fig 4c, 4d). Recent surgical studies report
that patch repair may be more effective than primary repair, with fewer patients requiring reoperation for recurrent aortocardiac fistulas (46). The
risk of surgical treatment generally is low, with
perioperative mortality rates ranging from 1.9% to
11.8% and 10-year survival rates ranging from
90% to 95% (16,47). Reported perioperative
complications include low cardiac output, recur-
rent ventricular arrhythmia, and anticoagulation
treatment–related bleeding (47,48). Late complications include recurrent aortic regurgitation,
endocarditis, and para-aortic abscess (49–51).
Although a small amount of peripatch leakage may
be seen in the immediate postoperative period, few
patients require reoperation (Fig 4c, 4d).
Percutaneous transcatheter closure of ruptured
Valsalva sinus aneurysms also has been reported
to have good results and may be a less invasive alternative to open surgery. Four author groups described the use of an occlusion device (Amplatzer
Duct Occluder; AGA Medical Corp, Golden
Valley, Minn) to successfully close right Valsalva
sinus aneurysms that had ruptured into the right
ventricle and right atrium, with no periprocedural
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Bricker et al 107
Figure 7. Nonruptured Valsalva sinus aneurysm in a 46-year-old woman with atypical
chest pain. Oblique coronal (a) and oblique axial (b) thin-section MIP images obtained at
ECG-gated CT show a nonruptured right coronary Valsalva sinus aneurysm (white arrows)
that involves the entire sinus. The right coronary artery (black arrow in a) is seen arising
from the apex of the aneurysmal sinus.
Figure 8. Ruptured Valsalva sinus aneurysm in a 27-year-old man with shortness of breath. (a) Oblique
coronal balanced SSFP MR image obtained during systole shows a right coronary Valsalva sinus aneurysm that has ruptured into the right atrium (arrow). A dark jet of turbulent blood flow (arrowhead)
also is seen with signal dephasing. (b) Oblique axial balanced SSFP MR image obtained at the level of
the sinuses shows the aneurysm (arrow).
complications; all four patients were asymptomatic at follow-up evaluations (52–55). Rao et al
(56) described a case of successful transcatheter
coil occlusion of a right Valsalva sinus aneurysm
that had ruptured into the right ventricle, with no
reported periprocedural or late complications.
Complications of nonruptured Valsalva sinus
aneurysms, such as ventricular outflow tract
obstruction, arrhythmia, and interventricular
dissection, also are indications for surgical repair.
However, in asymptomatic patients, surgical
intervention for nonruptured aneurysms is controversial. In general, large aneurysms should be
repaired to avoid complications, whereas smaller
aneurysms may be monitored.
108 January-February 2010
Summary
Aneurysmal dilatation most often involves the
right coronary Valsalva sinus, followed by the
noncoronary and left coronary sinuses. The clinical manifestations of ruptured and nonruptured
Valsalva sinus aneurysms vary widely, ranging
from an asymptomatic heart murmur and insidiously progressive dyspnea to acute chest pain
and cardiac arrest. The mainstay of treatment is
surgical repair, although a few cases of successful, noninvasive transcatheter repair have recently
been described. Although both ruptured and
nonruptured Valsalva sinus aneurysms are associated with potentially fatal complications, after
treatment the prognosis is excellent; for this reason it is important to make a prompt and accurate diagnosis. Most Valsalva sinus aneurysms
are diagnosed on the basis of echocardiographic
findings, with or without angiography, but both
ECG-gated CT and MR imaging can provide
excellent anatomic depiction, and MR imaging
can provide valuable functional information.
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RG
Volume 30
Number 1
January-February 2010
Bricker et al
Valsalva Sinus Aneurysms: Findings at CT and MR Imaging
Aliye Ozsoyoglu Bricker, MD, et al
RadioGraphics 2010; 30:99–110 • Published online 10.1148/rg.301095719 • Content Codes:
Page 100
The Valsalva sinuses are three subtle dilatations of the aortic root wall that arise between the aortic
valve annulus and the sinotubular ridge. Each sinus is associated with a corresponding right, left, or
noncoronary aortic valve cusp.
Page 102
The clinical manifestations of Valsalva sinus aneurysms vary widely. When symptoms are present,
they often are related to aneurysm rupture or mass effect on adjacent cardiac structures.
Page 103
When Valsalva sinus aneurysms rupture, they most commonly rupture into the right ventricle,
followed by the right atrium; the consequent aortocardiac shunt often leads to insidious heart failure.
Page 103
At imaging, the criteria for diagnosing a Valsalva sinus aneurysm include an origin above the aortic
annulus, a saccular shape, and normal dimensions of the adjacent aortic root and ascending aorta.
Page 106
The mean duration of survival after diagnosis of a ruptured Valsalva sinus aneurysm is 3.9 years. The
mainstay of treatment for a ruptured aneurysm is cardiopulmonary bypass surgery, and the surgical
approach is either via aortotomy, from the chamber where the aneurysm terminates, or a combination
of the two.