SINUS OF VALSALVA ANEURYSM

Moderator- Dr.RV KUMARPresenter- Dr.Jyotindra Singh

INTRODUCTIONThin walled, saccular or tubular outpouchings, usually always in the right sinus or adjacent half of the noncoronary sinus.

Generally have an - Intracardiac course

May protrude into the pericardial space and they may rupture into the right (or rarely left) heart chambers to form --- Aorta-cardiac fistula.

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

AdultsAA = STJ

ElderlyAA<STJ

.

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

Anatomic and Echocardiographic Relationship Between the Components of the Normal Aortic Root

Systole

Diastole

120 degree - LAX

ST junctionAnnulus

Tubular aortaSinuses

SINUS OF VALSALVAring.

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

MORPHOLOGY1 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.

CONGENITAL ACQUIRED

Connective tissue disorders-

Rheumatoid arthritis,

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

ACQUIRED

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-VariantsLeftward 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- VARIANTSNon 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 cardiac anomalies - VSD

Prevalence – 30 to 50 %

Incidence higher in Rt.sinus

Left third of right aortic sinus- JUXTA – ARTERIAL

RIGHT THIRD – conoventricular /peri membranous

Centrally – Juxta aortic / outlet portion of the septum.

Hinge line of aortic leaflet separates aneurysm from VSD

Supracristal VSD & RSOVA

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

VSD - CLASSIFICATION

Type-I - VSD is present immediately below the commissure of the left & right semilunar cusp of pulmonary valve .Aneurysm ruptures into the RVOT forming aortico –right ventricular fistula.

Type-II- VSD rests on the crista supraventricularis,not in contact with the tricuspid or pulmomary valves.

Type-III- VSD lies just below crista supraventricularis and the corresponding sinus of valsalva.

Type –IV – VSD rests on the paramembranacea

OTHER ANOMALIESAortic regurgitation results from prolapsed aortic cusp

Bicuspid aortic valve leading to AR.

Pulmonary stenosis

Aortic coarctation

PDA

ASD

Subaortic stenosis

TOF

ANEURYSM RUPTURERuptured 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%)

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

occur.

SITES OF RUPTURE ASIANS Non- Asians

RIGHT ATRIUM 13 35

RIGHT VENTRICLE 84 57

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.

UNRUPTURED ANEURYSM - Tricuspid valve dysfunction

- RVOT obstruction

- Severe MI – by compressing

right or left coronary artery.

- Conduction abnormalities

- Embolization from unruptured aneurysm.

ST-elevation in leads V1–V3

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.

JVP in Ruptured aneurysm

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

Ruptured aneurysm

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

SUDDEN CARDIAC DEATH

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 tachycardia

Compression of the His bundle occurs when the ruptured SVA penetrates the base of the interventricular septum and results in atriovenricular conduction defects and arrhythmias

X RAYIt 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

IMAGING GUIDELINES

 

35

AORTOGRAM

occur.

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 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 CMRThe 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.

Indications for Surgery

Indications for surgeryEnlargement 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.

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

STEPS OF SURGERY

A transverse aortotomy is performed, and the root anatomy assessed.

An oblique right atriotomy is performed next, allowing for identification of both ends of the aneurysm or of the fistulous tract in case of rupture .

In case of protrusion or rupture into the right ventricle, exposure can be obtained through a right atriotomy or a limited ventriculotomy .

When the fistulous tract or diverticulum is in the right ventricular infundibulum, the lesion can also be exposed through a transverse pulmonary arteriotomy.

STEPS OF SURGERY

The defect 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.

Single/Double patch repair

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

RSOVA + VSD

Thinned out windsock containing one or more perforation – resected creating a large defect in Rt. sinus of valsalva.

Defect separated from VSD by hinge line of right aortic cusp.

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

RSOVA to RA ,No VSD

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

Unruptured sinus of valsalva aneurysm

SOVA- RESULTS

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.

Complications

DEVICE CLOSURE

DEVICE CLOSURE