This document discusses sinus of Valsalva aneurysm (SVA), which is a rare cardiac anomaly where the wall of the sinus of Valsalva is weakened, forming a bulge or outpouching. SVAs can be congenital or acquired and most commonly originate from the right coronary sinus. Unruptured SVAs may be asymptomatic but can cause complications like heart failure. Ruptured SVAs often present with sudden chest pain and heart murmur, and can lead to cardiac tamponade, arrhythmias, or sudden death if ruptured into the pericardium. Echocardiography, CT, MRI and angiography can help in diagnosis. Surgery is the standard treatment but device closure is

1. SINUS OF VALSALVA ANEURYSM
Dr. KAZI ALAM NOWAZ
MD FINAL PART STUDENT CARDIOLOGY
NHFH & RI

2. Definition
• Thin walled, saccular or tubular
outpouchings usually always in the right
sinus or adjacent half of the noncoronary sinus.

3. • Sinus of Valsalva aneurysm (SVA) is an
uncommon cardiac anomaly that can be
congenital or acquired
• Account for only 1% of congenital cardiac
anomalies
• John Thurnam first described SVA in 1840.

4. • 5 times higher in Asian countries
• Male-to-female ratio is 4:1
• The prognosis is poor with progressive
aneurysmal dilatation or rupture unless early
surgical repair is performed.
• Actuarial survival rate for patients with
congenital SVA is 95% at 20 years, since most
SVAs do not rupture prior to age 20 years.

5. AORTIC ROOT- ANATOMY
Young
adults
AA>STJ
Adults
AA = STJ
Elderly
AA<STJ

6. .
The 2 trigones underneath the commissures of
the noncoronary leaflet are fibrous structures,
whereas the other underneath the commissure
between the right and the left leaflets is mostly
a muscular structure.
ROOT ANATOMY

7. Anatomic and Echocardiographic Relationship
Between the Components of the Normal Aortic
Root
Systole
Diastole
120 degree - LAX
ST junctionAnnulus
Tubular aortaSinuses

8. 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.
• It support coronary artery origin and help maintaining
continuous blood supply to heart both in systole &
diastole
• In valve sparing aortic valve surgery, maintenance or
recreation of the sinuses has been beneficial in terms of
normal leaflet movement and valve durability

9. Pathology
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.

11. Aetiology
Congenital Acquired
• Idiopathic
• Ehlers-Danhlos syndrome
• Marfan’s syndrome
• Klippel Feil syndrome
• Turner’s syndrome
• Trisomies 13 and 15
• Loeys-Dietz syndrome
• Arachnodactyly
• Osteogenesis imperfecta
• Infectious diseases –
bacterial endocarditis,
syphilis, and tuberculosis;
• Degenerative conditions
atherosclerosis
cystic medial necrosis;
• Injury from deceleration trauma.
• Iatrogenic pseudoaneurysms
hematoma formation after AVR
removal of aortic valve calcifcations

12. Association
• VSD:53.3%
• RVOT obstruction :7.5%
• Aortic valvular malformations:5.2%

13. Origin
• RCC:77%
• Non-CC:19%
• Multiple:2.4%
• left coronary sinus:0.5%

14. Complication
• Aortic regurgitation
• Heart failure
• Coronary artery flow compromise
• Atrioventricular conduction blocks
• Right ventricular outflow tract obstruction
• Infectious endocarditis
• Rupture into cardiac chambers
• Sudden cardiac death

15. Intact vs. rupture
• 71.7% ruptured
• Mean age of rupture 31 years

16. ANEURYSM RUPTURE
• Ruptured aneurysms
originate most
frequently from the
right coronary sinus
(65–85%),
• Less frequently from
the noncoronary sinus
(10–30%), and
• Rarely from the left
coronary sinus ( 5%)

17. Exit
• The right ventricle is the most common
receiving chamber (about 80–90%), due to
rupture of either right or noncoronary SVA
• Right atrium (10-20%)
• Other rare entry sites of rupture included the
left atrium, the left ventricle, the interatrial
• septum, the interventricular septum and the
pulmonary artery (0.5%–1.9%)

18. Exit

19. Sakakibara S, Konno S. Congenital aneurysm of
the sinus of Valsalva. Anatomy and
classification. Am Heart J 1962;63:405–24.
• 47.6% type I
• 33.5% type II
• 6.1% type IIIv
• 12.8% type IIIa

20. The SVAs arising from RCC by angiogram Sakakibara
and Konno
• Type I: left part of the sinus rupture or
protrusion into upper portion of RVOT
• Type II: central part of the sinus rupture or
protrusion into mid-portion of RVOT through
supraventricular crest
• Type IIIv: rupture or protrusion into right
ventricle near or at tricuspid annulus
• Type IIIa: rupture or protrusion into right
atrium

21. 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

22. 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

23. 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

24. UNRUPTURED ANEURYSM
• - Tricuspid valve dysfunction
- RVOT obstruction
- Severe MI – by compressing
right or left coronary artery.
- Conduction abnormalities
- Embolization from unruptured
aneurysm.
Compression of the His bundle occurs when the
unruptured SVA penetrates the base of the
interventricular septum and results in
atriovenricular conduction defects and arrhythmias

25. 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.

26. JVP in Ruptured aneurysm
• Rupture into the Right ventricle-
the severe diastolic ventricular volume-
overload causes
Obliteration of the y-descent
• Rupture into the right atrium
obliterates the x-descent.
• The high Right atrial pressure-
Early tricuspid opening
Premature v-wave,
High peaked a-wave
with a fourth heart sound.

27. Ruptured aneurysm
• Sudden appearance of a continuous or To and
Fro 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
• The apical impulse is hyper dynamic and the
pulse pressure is widened

28. Continuous murmur
• PDA (Loudest in left 2nd ICS)
• Coronary AV fistula (Lowdest in lower in
sternal border)
• Ruptured sinus of valsalva (Loudest in third or
fourth ICS near the sternal edge)

29. 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

30. ECG
•
ECG showing sinus tachycardia with 1st degree AV block and right bundle branch block.
Ventricular tachycardias arising from the aortic
sinus of Valsalva: An under-recognized variant of
left outflow tract ventricular tachycardiaST-elevation in leads V1–V3

31. 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

32. Echocardiography

35. CMR
The advantages of performing MRI
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.

36. CT SCAN
• CT is less time consuming and the preferred
investigation compared to MRI in case of
acute setting of aneurysmal rupture

37. CONTRAST ENHANCED CT

38. CT ANGIOGRAM

39. Conventional angiography

40. Medication Summary
• Depending on the clinical presentation
perioperative medical management consists
of
• Relieving heart failure symptoms
• Treating arrhythmia if present
• Treating endocarditis if present

41. Management
• Gold: Surgery
• Evolving: Transcathetor closure

42. Indications for surgery
• Enlargement beyond 5.5 cm,
• Progression of greater >1.0 cm/year.
• Aortic regurgitation from distraction of the commissural posts with ventricular
enlargement
• Unruptured aneurysms encroaching on nearby structures, causing myocardial
ischemia, or having the potential to rupture warrant repair.
• Family history of aortic dissection or rupture.
• Asymptomatic patients – Serial follow- up . If high likelihood of progressive
increase in size and the possibility of rupture or endocarditis.

43. SURGERY
•Usually successful( 95% survival after 25 years)
•Recurrence possible (16% reoperance rate)
•Techniques includes
1. Aortic root reconstruction or replacement
2. Aortic valve repair or replacement
3. Bentall procedure (valved conduit)
4. Ventricular septal defect repair (if present)
5. Atrial septal defect repair (if present)
6. Primary suture closures (pledget) and patch closures (if
rupture)

45. Complications
• Most patients survive the early post op period.
• Hospital mortality – max. 5%
• Severe AR with marked LV enlargement is a risk factor for
premature death in late postoperative period.
• Direct closure – 20 to 30 % prevalence for reoperation for
recurrence of the fistula.
• Heart block occurs in 2 % to 3% of patients.

46. Device closure of ruptured sinus of
valsalva
• Though ruptured sinuses of valsalva have
been traditionally managed surgically, they are
amenable to transcatheter closure by using
the Amplatzer duct occluder

47. DEVICE CLOSURE
•

48. DEVICE CLOSURE
•

49. Outcome
• Unruptured SVA has been observed in serial
monitoring up to several years after initial
diagnosis, but most unruptured SVAs have
been found to progress and rupture.
• Untreated SVAs may rupture, and patients
with ruptured SVAs die of heart failure (with
left-to-right shunting) or endocarditis within 1
year after onset of symptoms of ruptured SVA.

50. Conclusion
• A congenital SVA is usually clinically silent
• Rupture of SVA is the main cause of death and
rarely occurs before age 20 years in congenital
SVA.
• Cardiothoracic surgery consultation is urgent
in patients with ruptured SVA, because clinical
deterioration can be rapid

51. THANKS TO ALL