SINUS OF VALSALVA

ANEURYSM
•

DR. KALPESH PATIL

DEPT OF CARDIOLOGY
• A 27‑year‑old female, resident of
Uttar Pradesh, and a homemaker • D/D
by occupation presented with the
chief complaints of
• Episode of sudden‑onset epigastric
pain 4 months back
• Progressive shortness of breath × 4
months
• Pedal edema and abdominal
distension × 4 months
• Exertional palpitations × 3 months.
 INTRODUCTION
• Usually Thin walled, saccular or tubular
outpouchings always in the right sinus or
adjacent half of the noncoronary sinus.
This defect may result from absence of
• Generally have an Intracardiac course normal elastic tissue at junction of the
aortic media and annulus fibrosus.

• May protrude into the pericardial space and

they may rupture into the right (or rarely left)
heart chambers to form ---
Aorta-cardiac fistula. Congenitally weak area enlarges under
aortic pressure to form aneurysm
• 5 times higher in Asian countries.

• Male preponderence – 4:1

• 0.15-1.5% surgeries correspond to SVA repair

 HISTORICAL ASPECT
• 1839 -1st description by Hope
• 1840- 1st important paper published by Thurman
• 1949- Jones and Langley -the subject of congenital and acquired
lesion .

• 1951- 1st diagnosis of rupture during life by Venning

• 1956- 1st. successful repair with CPB at Mayo Clinic using CPB.
• 1957-Morrow & colleagues –closed ruptured SOVA using mild
hypothermia
• SAKAKIBARA & KONNO
- Studied association with VSD & AR
- First to provide comprehensive classification
AORTIC ROOT- ANATOMY

Young
adults
AA>STJ

Adults
AA = STJ

Elderly
AA<STJ
EMBROYOLOGY

• Sinus of Valsalva aneurysms • There is also a relative

occur when there is a deficiency of elastic fibres in the
congenital defect in the aortic affected sinus that under the
media and an incomplete strain of aortic pressure
fusion of distal bulbar septum gradually weakens and dilates,
(primitive bulbus cordis) with causing the formation of an
truncal ridges resulting in mal- aneurysm.
fusion of the aortic media and
annulus fibrosus of the aortic
PATHOLOGY
valve.

Edward and Burchell postulated that the lack of fusion between the
aortic media and the annulus fibrosus of aortic valve results in a
congenital weak area in the aortic wall, which gradually becomes
thinned out and distended as it is subjected to high ejectile force of
LV. In some cases, the aneurysm may start developing from fetal life
 MORPHOLOGY
1 Separation of the aortic media of the
sinus from the media adjacent
to the hinge line of the AV valve
cusp .

Results from the absence of normal

aortic elastic tissue and media in two
region.

2 Congenitally weak area gradually

gives way under aortic pressure to
form an aneurysm.

3 The aneurysm appears an excavation

of the sinus which protrudes into the
underlying cardiac chamber.
 ROOT ANATOMY

.
[Link] sinuses function as a support structure
for the aortic valve
The 2 trigones underneath the 2. Provide space behind the valve leaflets
commissures of the noncoronary leaflet
when the leaflets are open so that they do not
are fibrous structures, whereas the other
underneath the commissure between the occlude the coronary ostia .
right and the left leaflets is mostly a
muscular structure.
Anatomic and Echocardiographic
Relationship Between the Components
of the Normal Aortic Root
 SINUS OF VALSALVA

• 3 sinuses named after- Antonio Valsalva.

• Provide space behind the open aortic leaflets so that the

leaflets do not occlude the coronary artery orifices.

• Secondly, this space favours the development of eddy

currents behind the leaflets when they are open.

• Magnetic resonance imaging has shown- in aiding leaflet

opening through the creation of a low-pressure system by
means of the Venturi effect.

• In valve sparing aortic valve surgery, maintenance or

recreation of the sinuses has been beneficial in terms of
normal leaflet movement and valve durability
 SINUS OF VALSALVA
ring.
ETIOLOGY

• Primary • Secondary
Congenital aneurysm of sinus Aneurysm of sinus of Valsalva
of Valsalva is the most common may be formed due to
form of such defect. It is atherosclerosis,
presumed to be caused by a syphilis,
spontaneous genetic mutation.
rarely tuberculosis,
cystic medial necrosis (e.g.
Marfan’s syndrome),
blunt or penetrating chest injury
infective endocarditis.
 CONGENITAL ACQUIRED

Connective tissue disorders- • Infectious diseases –

bacterial endocarditis,
syphilis, and tuberculosis;
• Rheumatoid arthritis,
• Ehlers-Danhlos syndrome, • Degenerative conditions
• Marfan’s syndrome, atherosclerosis
• Klippel Feil syndrome, cystic medial necrosis;
• Turner’s syndrome,
• Trisomies 13 and 15, • Injury from deceleration trauma.
• Loeys-Dietz syndrome,
• Iatrogenic pseudoaneurysms
• Arachnodactyly, hematoma formation after AVR
• Osteogenesis imperfecta. removal of aortic valve calcifcations
• Type I: the aneurysm originates in the left portion of the right sinus, protrudes forward and
ruptures into the right ventricle near the pulmonary valve.
The concurrent presence of VSD under the pulmonary valve is frequent.

• Type II: the aneurysm originates in the mid portion of the right sinus, protrudes and ruptures
in the right ventricle. A concurrent VSD is uncommon.

• Type III: the aneurysm originates in the mid portion of the right coronary leaflet and
protrudes towards the tricuspid valve. It often ruptures into the right atrium and sometimes
into the right ventricle, just below the septal leaflet of the tricuspid valve. VSD is rarely
encountered.

• Type IV: the aneurysm originates in the right portion of the non-coronary leaflet and
ruptures into the right atrium. A combined VSD is uncommon.
Congenital Heart Surgery Database Committee
of the Society of Thoracic Surgeons
 RSOVA-Variants
• Leftward portion of sinus

WINDSOCK projecting into the adjacent

RVOT just below the pulmonary valve.

• Arising CENTRALLY

project in the outlet portion of the

RV aspect of the ventricular septum
• RIGHTWARD

Entering RV beneath the parietal band in the

region of Membranous septum
 Non coronary sinus- VARIANTS
• Non coronary sinus

originate from –ANTERIOR PORTION

---- Project into RIGHT ATRIUM

---- Rarely into RV or RA+ RV, muscular
ventricular septum.

POSTERIOR PORTION

RUPTURE INTO PERICARDIUM

Associated abnormalities

• ventricular septal defects (30 60%),

• bicuspid aortic valve (15-20%),
• aortic regurgitation (40-50%)
• persistent left superior vena cava.
 Associated cardiac anomalies - VSD
• Prevalence – 30 to 50 %

• Incidence higher in [Link]

• Left third of right aortic sinus- JUXTA –

ARTERIAL

• RIGHT THIRD – conoventricular /peri

membranous

• Centrally – Juxta aortic / outlet portion of

the septum.
Supracristal VSD & RSOVA

• Hinge line of aortic leaflet separates

aneurysm from VSD
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
 ANEURYSM RUPTURE
• Ruptured aneurysms originate most
frequently from the right coronary
sinus (70–90%),

• Less frequently from the noncoronary

sinus (10–20%), and

• Rarely from the left coronary sinus

• ( < 5%)

• The right ventricle is the most

common receiving chamber (about
80–90%), due to rupture of either
right or noncoronary SVA
 RUPTURE
• RIGHT CORONARY SINUS
Develop localized “ WINDSOCK ”

Rupture into adjacent low pressure chamber

INTRA CARDIAC FISTULOUS PORTION

NIPPLE LIKE projection into cardiac chamber with one

or more points of rupture at its apex

Non coronary sinus origin - have no

WINDSOCK deformity , direct fistulous
communication between aortic sinus & heart

Left sinus origin – Extra cardiac aneurysm

 ANEURYSM RUPTURE
SITES OF RUPTURE ASIANS Non- Asians

RIGHT ATRIUM 13 35

RIGHT VENTRICLE 84 57
• occur.
RV + RA <1 <1

LEFT ATRIUM <1 <1

LEFT VENTRICLE <1 2

RA+ LA+ LV <1 <1

VENTRICULAR SEPTUM <1 <1

PULMONARY TRUNK <1 <1

RV + Pulmonary trunk <1 <1

PERICARDIUM <1 2
 Presentation
SOVA clinically presents based on

• Depending on the size of the aneurysm,

• the rapidity with which it ruptures,
• the cardiac chamber with which it communicates

RUPTURED SOVA

• 20% no symptoms develop.

• 45%- gradual onset of effort dyspnea
• 35% - acute symptoms

• sudden breathlessness & pain

• Pain- precordial/ epigastric
• Sudden death
• precipitated by – heavy exertion/ IE / Marfan syndrome.
Other effects of a sinus of
Valsalva
1. Aortic regurgitation by interfering with aortic leaflet coaptation,

2. Tricuspid regurgitation by entering the right atrium(RA)

3. Subpulmonary obstruction by entering the right ventricular

outflow tract ( RVOT).

4. Compression a proximal coronary artery

5. Dissection into the ventricular septum and cause complete heart

block.
• . A congenital aortic sinus
aneurysm that is the site of
infective endocarditis can be
difficult to distinguish from
aortic valve infective
endocarditis that caused the
aortic sinus to perforate.
• . A congenital sinus of Valsalva
aneurysm begins as a blind
pouch or diverticulum at a
localized site in an aortic sinus
and then protrudes as a finger-
like or nipple-like projection that
ruptures at its tip
 UNRUPTURED ANEURYSM
• - Tricuspid valve dysfunction

- RVOT obstruction

- Severe MI – by compressing
right or left coronary artery.
ST-elevation in leads V1–V3
- Conduction abnormalities

- Embolization from unruptured

aneurysm.
ARTERIAL PULSE

• All gradations of an aortic runoff are reflected in the arterial pulse,

irrespective of which chamber or side of the heart receives a ruptured
sinus of Valsalva aneurysm.

• The pulse pressure may not be wide immediately after perforation

because the left ventricle has not had time to adapt to the increase in
volume and because left ventricular end diastolic pressure is elevated.

• If survival permits, the arterial pulse becomes bounding with a rapid

rise, a rapid fall, a wide pulse pressure, and a bisferiens configuration

• When an aneurysm penetrates the base of the ventricular septum and

causes complete heart block, the arterial pulse is slow
JVP

• The height and waveform of the jugular venous pulse ----

depend on the size and rapidity of the rupture and
on the presence and degree of right ventricular failure.

elevated mean jugular venous pressure, and tall A and V waves -

large sudden rupture into the right atrium or right ventricle is
accompanied by congestive heart failure.

the V wave is selectively elevated- When an aortic sinus aneurysm

projects into the right atrium and causes tricuspid regurgitation
• A wave selectively increases- When an aneurysm results in
obstruction to right ventricular outflow.

• When an aneurysm causes obstruction of the superior vena cava,

the mean jugular venous pressure is elevated, but the wave forms
disappear.
PHYSICAL EXAMINATION

• An unruptured aneurysm that obstructs right ventricular outflow is

accompanied by an isolated right ventricular impulse a systolic thrill
generated across the obstruction.

• A continuous thrill that is more prominent in either systole or

diastole coincides with a loud coarse murmur and a large acute
rupture. This continuous thrill is very superficial and well palpable
on slightest touch.
• 1.A hallmark of acute rupture of an aortic sinus aneurysm into the
right side of the heart is the sudden appearance of a continuous
murmur in a previously healthy individual, usually a young male.
• 2. When the RA receives the rupture, the continuous murmur is
maximal along the right or left sternal border, or over the lower
sternum
• 3. When the rupture enters the body of the right ventricle, the
continuous murmur is maximal at the mid to lower left sternal
border

• 4. Rupture into the outflow tract of the right ventricle results in a

continuous murmur at the upper left sternal edge.
• 5. Rupture directly into the left ventricle results in an early diastolic
murmur of aortic regurgitation and in a short midsystolic murmur
from augmented flow into the aorta.

• 6. An unruptured aneurysm may cause an isolated diastolic murmur

of aortic regurgitation by compromising aortic cusp coaptation.
 Ruptured aneurysm
• The most characteristic finding of rupture of
sinus of Valsalva aneurysm is a continuous
murmur

• Sudden appearance of a continuous murmur

in an otherwise healthy individual.

• Heard at a maximum at the lower sternal

border or xiphoid.

• Diastolic accentuation of this murmur is an

important sign to differentiate ruptured
sinus from PDA or arteriovenous fistula.

• Systolic suppression of the murmur is caused

by both mechanical narrowing of the
fistulous tract during systole as well as the
probable

• Venturi effect created by the rapid ejection of

blood past the aortic origin of the fistula
• Tricuspid regurgitation, mitral • An aneurysm may protrude into
regurgitation, and ventricular the right ventricular outflow
septal defect result in tract and cause a longer
holosystolic murmurs. midsystolic murmur or may
com promise aortic cusp
coaptation and cause the
• Short midsystolic murmurs are murmur of aortic regurgitation.
generated by rapid ejection
across the aortic and
pulmonary valve
 SUDDEN CARDIAC DEATH
• Tamponade
• Myocardial ischemia,
• Conduction disturbances and/or
arrhythmias.

• Rupture into the pericardial space, a

very rare complication (2% of
noncoronary SVA ruptures), almost
invariably leads to fatal cardiac
tamponade

• Rupture causes compression of the

ostium of the left main coronary
artery, resulting in myocardial
ischemia and arrhythmic death
ECG CHANGES :

• The rhythm is normal sinus even when a large rupture is into the
right atrium.

• The PR interval tends to be prolonged .

• The QRS axis is normal or rightward, and occasionally leftward.

• Atrioventricular conduction defects, including complete heart block,

right or left bundle branch block, and bifascicular block, occur when
a ruptured or unruptured aneurysm penetrates the base of the
ventricular septum and injures the atrioventricular node or His
bundle.
ECG changes :

• A right atrial P wave abnormality

is generated when the right
atrium receives the rupture, or
when an aortic sinus aneurysm
causes tricuspid regurgitation.

• Increased flow through the left • Rupture into the right atrium or
atrium accounts for a left atrial P right ventricle results in volume
wave abnormality. overload of both ventricles, but
the electrocardiogram usually
shows left ventricular
hypertrophy by voltage criteria
and ST segment/T wave
abnormalities
 ECG

Compression of the His bundle occurs when the

ruptured SVA penetrates the base of the Ventricular tachycardias arising from the aortic
interventricular septum and results in sinus of Valsalva: An under-recognized variant
atriovenricular conduction defects and arrhythmias of left outflow tract ventricular tachycardia
X RAY
• It is uncommon to find the aneurysm
abnormality on x ray as they are
intracardiac.

• However, the evidence of aortic

atherosclerosis is a clue to the etiology as
evidenced in this patient.

• Rarely these aneurysms can cause heart

border abnormalities depending upon
the cusp involved.

• Marked cardiomegaly can be visualized if

aortic root dilation and aortic
insufficiency are present
CXR FINDINGS

• Large acute ruptures are

followed by pulmonary venous
congestion because of the steep
rise in end diastolic pressure in
an unprepared left ventricle.

• Increased pulmonary arterial

blood flow results in
enlargement of the pulmonary
trunk
• Moderate left atrial
enlargement is seen in the
lateral projection; and in the
anteroposterior view, a right
atrial convexity appears at the
right lower cardiac border and a
moderately dilated left
ventricle occupies the apex
IMAGING GUIDELINES
•
Echocardiographic criteria for a
sinus of Valsalva aneurysm
• (a) the root of the aneurysm be superior to the aortic annulus
• (b) the aneurysm be saccular in appearance and
• (c) the aortic root is of normal size.

• Aneurysmal dilatation of the sinus of Valsalva gives “Windsock”

appearance in more than 50 percent of case.
• Doppler and Color Flow Study Shows
• a. Continuous turbulence detected by pulsed wave Doppler just distal
to the area of rupture.
• b. Continuous turbulence at high velocities detected by continuous
wave Doppler.
• c. Color flow imaging show mosaic pattern indicating turbulence
across the ruptured aneurysm in real time
(A) Parasternal short axis view showing
(B) Parasternal short axis view
“windsock” aneurysm of the right aortic
showing aneurysm of right
sinus rupturing into RVOT
coronary sinus rupturing into RA

(C) Apical 5 chamber view showing

aneurysm of non-coronary sinus
rupturing into LV (A and B Courtesy:
Dr BK Mahala, Narayana Hrudayalaya,
Bangalore (C) Dr SK Sahoo, Cuttack
• All these features can be more clearly seen on trans eosophageal
echocardiography.

• Spectral Doppler interrogation helps in assessing RV outflow

obstruction, VSD, TR and severity of AR if present.
CATH STUDY

• Catheter study shows presence of a step up in oxygen saturation in

the right heart and filling of the right heart chambers by contrast
medium from the ascending aorta.

• The evolution in echocardiography has obviated the need for cardiac

catheterization in diagnosing and planning for the management of
rupture of sinus of Valsalva aneurysms
54
 AORTOGRAM
• Right-sided pressures were elevated with a
right atrial pressure of 12 mmHg,

• Pulmonary artery pressure of 47/31 mmHg

with a mean of 37 mmHg, a pulmonary
• occur. wedge pressure of 20 mmHg and a left
ventricular end-diastolic pressure is 24
mmHg.

• There was a significant 14% rise in oxygen

saturation between the right atrial and
vena caval oxygen saturations.

• A single injection was done at the aortic

root which demonstrated a communication
between the origin of the right sinus of
Valsalva into the right atrium (RA), with
eventual opacification of the right atrium,
right ventricle (RV) and pulmonary arteries . -
CT vs CMR
• The advantages of performing MR imaging in the
setting of a known or suspected Valsalva sinus
aneurysm include the

-evaluate the LV hemodynamic pattern,

- identify aortic regurgitation and quantify aorto-
cardiac shunt or fistulous blood flow.

• CT is less time consuming and the preferred

investigation compared to MRI in case of acute
setting of aneurysmal rupture

• Conventional angiography is the

gold standard and can be used for both
diagnostic and therapeutic purposes.
MANAGEMENT

• Small rupture repaired in timely fashion to prevent progression of

heart failure.

• UNRUPTURED RSOV – clear cut indication for surgery

IE / INTRCTABLE ARRYHTMIAS / CORONARY ARTERY COPMRESSION/
RVOT/LVOT obstruction

• Optimal management strategy for unruputured Asymptomatic SOV

remains unclear as natural history is not well defined.

• Surgical repairs remains mainstay of treatment for both ruptured and

indicated unruptured SOV
Algorithm for therapeutic approach
to aneurysm of the sinus of Valsalva
(ASV) by Vural et al.
 STEPS OF SURGERY

• Approach is by a median sternotomy using cardiopulmonary bypass.

• The arterial cannula is placed distally in the ascending aorta.

• Bicaval venous cannulation using right angle cannulas should be used.

• A moderate degree hypothermia,e.g. 28°C, is appropriate.

• CARDIOPLEGIA- Retrograde
• diagnosis of RSOV requires surgical intervention to prevent
progression of the disease resulting in death from right heart failure

• Initial preparation (CPB) is as for any open-heart procedure.

• After midsternotomy, pericardium is opened and an external
evaluation is done.
• There are no external evidence of aneurysm. The sac of aneurysm
may be palpated through the freewall of the right ventricle.
• Cardiopulmonary bypass is established after cannulation of
ascending aorta and direct caval cannulation.
• With mild-to moderate hypothermia, aorta is cross clamped, right
atrium opened and a vent suction introduced through foramen
ovale.
• Aorta is opened transversly, cardiac arrest achieved by infusing
antegrade cold cardioplegia through the coronary ostia directly.

• The orifice of the RSOV is visualized and the VSD if present is visualized
by lifting the aortic cusp.
• Redundancy or cusp prolapse is noted

• The thin windsock, which is the aneurysmal sac with a single opening or
multiple perforations is excised, creating a large defect in the right
sinus, which is down stream or cephalad to the VSD, separated by a
hinge line of the aortic cusp.
• Dacron or polytetrafluoroethylene (PTFE) patch is sewen to close the
VSD and the defect in the sinus of Valsalva, taking care to suture the
aortic cusp hinge to the patch at appropriate level
 Single/Double patch repair
• . Thedefect must be repaired
through the aortic root, using a
patch of autologous or bovine
pericardium to exclude the
aortic inlet into the aneurysm
• Primary closure predisposes to a higher risk of
recurrence (as high as 20%) or aortic valve
regurgitation from deformation of the root. -
CONDEMNED

• The ventricular or atrial aspect of the fistula

can be closed primarily,

• a patch should be used to incorporate closure

of a coexisting ventricular septal defect

• Great care should be taken in avoiding the

atrioventricular conduction system at the time
of VSD closure.
 RSOVA + VSD
• Aneurysm is in Right ward position of Right
sinus - Perimembranous VSD

• RIGHT ATRIAL APPROACH with detachment

of antr & septal leaflet of TV

• Leftward portion of the Right sinus-

• Vsd is juxta arterial - approach is through the

RV or pulmonary trunk.

• Combined Approach- Aortic & RV pulmonary

trunk or right atrial approach.
 RSOVA + VSD
•
•

Thinned out windsock containing one or Defect separated from VSD by hinge line of right
more perforation – resected creating a large aortic cusp.
defect in Rt. sinus of valsalva.
 RSOVA to RA ,No VSD
• Origin- usually non coronary sinus but
occasionally from right coronary sinus.

• Approach- Aorta alone or right atrium.

• Clamp placed across windsock.

• A coexisting VSD is always sought.

• Windsock is excised –based on hinge line

of valve cusp.

• Windsock is narrow & bordering edges are

of good quality- direct closure
 Unruptured sinus of valsalva
aneurysm
• Approach through ascending aorta.

• CPB- through single canula in RA.

• Venting catheter in LA

• Cardioplegia

• Aorta – opened transversely

• Site of origin of aneurysm defined.

• Orifice closed with pericardial / dacron patch

•
 Complications

• Most patients survive the early post op period.

• Hospital mortality – max. 5% reported.

• Severe AR with marked LV enlargement is a risk factor for premature death in

late postop period.

• Direct closure – 20 to 30 % prevalence for reoperation for reoperation for

recurrence of the fistula.

• Heart block occurs in 2 % to 3% of patients.

INDICATIONS FOR DEVICE CLOSURE
OF RSOV
• In the present era, ruptured aortic sinus aneurysm can be closed
percutaneously. with device after careful patient selection.

• Patient with left-to-right shunt with pulmonary to systemic flow

ratio of greater than 1.5:1

• with right ventricle volume overload greater than 1.5 cm/m2 and

• the margin of the defect at least 5 mm from the right coronary

ostia are suitable for device closure
 DEVICE CLOSURE
•
•THANK YOU