sinus valsalva aneurysm seoul national university hospital department of thoracic &...

Sinus Valsalva Aneurysm

Seoul National University HospitalDepartment of Thoracic & Cardiovascular Surgery


Definition• Thin walled, saccular or tubular outpouchings, usually

always in the right sinus or adjacent half of the noncoronary sinus.

• They generally have an intracardiac course, but may protrude into the pericardial space and they may rupture into the right (or rarely left) heart chambers to form an aorta-cardiac fistula.

• This defect may result from absence of normal elastic tissue and media in this region.

• Congenitally weak area gradually enlarges under aortic pressure to form an aneurysm, although the age at which this occurs is uncertain


History• 1st description by Hope in 1839

• 1st important paper published by Thurman in 1840

• Suggested ruptured as congenital by Abbott in 1919

• Reviewed the subject of congenital and acquired lesion

by Jones and Langley in 1949

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

• 1st. successful repair with CPB in 1956 at Mayo Clinic

& University of Minnesota using CPB

Aneurysm of Sinus ValsalvaClinical features1 Etiology ; congenital but other possibly acquired * Endocarditis, syphilis, Behcet’s disease, atherosclerosis, Cystic medial necrosis, penetrating injury * Incomplete fusion of proximal & distal bulbous chordous * Anatomic defect in the elastic tissue * Deficiency of the conal septum

2 Rupture or fistula 1) Incidence : rare ( 0.2 ~ 0.5% of open heart surgery), 75% ~ 80% are male 2) Site * Right coronary sinus to right ventricle : 65% * Noncoronary sinus to right atrium : 25% * Left coronary sinus to left atrium : rarely 3) Aortico-left ventricle tunnel : exceedingly rare form

Sinus Valsalva Aneurysm Etiology1 Separation of the aortic media of the sinus from the media adjacent to the hinge line of the AV valve cusp resulted 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.4 In Asians, the basic abnormality is sited leftward and toward the commissural area between Rt. and Lt. cusp.5 Acquired lesions caused by medionecrosis, syphilis, atherosclerosis, endocarditis, or penetrating injury are more diffuse, involving more of sinus or multiple and often ascending aorta, and projecting outside the heart.

Sinus Valsalva Aneurysm Pathophysiology

• Thinning of the aorta medial layer in the wall of a sinus of Valsalva results in an aneurysmal dilation, which may extend and rupture into a corresponding cardiac chamber, forming an aortocardiac fistula.

• Aneurysms usually arise from the right coronary sinus and extend into the right ventricle or right atrium.

• Aneurysmal rupture into the right heart results in a large left-to-right shunt , which, in turn, can lead to congestive heart failure.

• Unruptured aneurysms extending into the right heart may cause tricuspid valve stenosis/incompetence, right ventricular outflow tract obstruction, or complete heart block.


Unruptured aneurysm of right sinus Valsalva Aneurysm is walled by atrophic muscular tissue of RVOT

Histologic view


Associated cardiac anomalies• VSD occurs in 30 to 50%, but may be a little higher in

surgical patients.• Aortic valve abnormalities & incompetence are commo

n, and when VSD is present, AR usually results from a prolapsed cusp , and when VSD is not present , AR usually arises from other valve abnormalities

• Pulmonary stenosis is uncommon, but small gradients are common.

• Others are uncommonly, but any defects including COA, PDA, ASD, subaortic stenosis & TOF are present.


Natural History1 Unruptured aneurysms uncommonly cause symptoms, by protrusion into RA and RV, heart block as well as ventricular tachycardia may result.2 Rupture of aneurysm tends to take place in the 3rd or 4th decade of life.3 Once symptoms develop, the heart failure worsens and, without surgical treatment, most patient die within one year. Clinical presentation is usually within the 3rd decade of life

4 When a VSD coexists, AV is usually at least mildly incompetent, by the time 15 to 20 years, a fixed fibrous deformity of the prolapsed leaflet occurs.


Clinical features• The SVA produce TV dysfunction or RVOT obstruction.

• 80% of the persons with sinus Valsalva aneurysm are male.

• Rupture produces acute symptoms in about 35% and gradual

onset of effort dyspnea in 45% and no symptoms in 20%.

• In a few patients, death occurs within days, but in most there is

improvement, followed by recurrent symptoms.

• The frequency of symptoms may be related to the size of the

Fistula.

• Rupture is heralded not only by pain & dyspnea but also by

appearance of murmur, widened pulse pressure.


• Unruptured aneurysm of right sinus Valsalva with VSD

Daigram of aneurysm


• Ruptured right Sinus Valsalva Aneurysm with VSD

Daigram of aneurysm


Arrows indicate common sites of rupture of sinus of Valsalva aneurysm M ; membraneous septum NC ; noncoronary sinus V ; atrioventricular septum C ; conal septum

Rupture site


RVOT obstruction


Rupture1 The sinus of origin is the main determinant of the direction of projection and rupture.

2 Gradually develops a more localized windsock, in an unknown percent of cases ultimately rupture into an adjacent low pressure chamber and rarely outside chamber.

3 When the aneurysm coexists with a VSD(30-50%), the windsock usually projects into the RV.

4 In about one fourth, there is no windsock or any suggestion of aneurysm formation, but rather, a direct fistulous communication.

5 Typical windsock deformity may be more common from right sinus lesion, and a direct fistula in noncoronary sinus to RA lesion. .


Sites of rupture or fistula1 Aneurysm of the right sinus may originate more centrally and

project into the outlet of RV, but leftward portion into region of membranous septum.

2 Aneurysms from the noncoronary sinus usually originate from its anterior portion and rupture into the RA, but in rare cases

into RV, posterior portion may rupture into the pericardium.

3 Rarely, right or noncoronary sinus aneurysm rupture into LV.

4 Aneurysms from left coronary sinus rupture into the LA, LV, but rarely into LV due to thick wall and high pressure.

5 Aneurysms rupturing into areas adjacent to TV may be a cause of heart block or RBBB.


Indications for operation1 When ruptured or is associated with VSD or wi

th a VSD and AR, prompt operation is advisable.

2 Unruptured aneurysm that are producing hemodynamic derangements should be repaire

d.3 Small or moderate-sized unruptured aneurysm probably should not be repaired surgically.


Techniques of operation1 Ruptured aneurysm of right sinus Valsalva without VSD

2 Ruptured aneurysm of the sinus of Valsalva into the RA without VSD

3 Ruptured aneurysm of the right sinus of Valsalva

associated with VSD * Repair by excision of aneurysm and reconstruction

* Repair by closing the origin of aneurysm

* Repair the associated VSD and valve

Techniques of operation

• Repair of ruptured aneurysm of right sinus Valsalva with VSD



• Repair of unruptured aneurysm of right sinus Valsalva



• Noncoronary sinus

of Valsalva aneurysm

extending into the

right atrium


Techniques of operation• Noncoronary sinus

of Valsalva aneurysm

extending into the

right atrium

• VSD patch closure in

case of VSD



• David-V valve-sparing root replacement using a De Paulis Gelweave Valsalva graft



Results of operation1 Survival2 Risk factors for premature late death 1) severe aortic incompetence 2) left ventricular enlargement 3) aortic valve replacement

3 Functional status Persistent or worsening aortic valve incompetence

accounts for most of functional disability 4 Complications 1) Reoperation 2) Heart block

Aorta–right Atrial TunnelClinical features• Aorta–right atrial tunnel (ARAT) is a very rare abnormal tubula

r extracardiac communication between the ascending aorta and the right atrium.

• The first case was described in 1980 by Otero Coto and colleagues

• Embryologic background and cause for this anomaly are not clear.

• Probable cause seems to be a congenital deficiency of the elastic lamina in the aortic media

• The tunnel-like vascular extracardiac communication between the aortic root and the right atrium arose from any of the 3 sinuses of Valsalva.

Aorta–right Atrial TunnelClinical features• This aorto–right atrial communication behaves like a left-to-ri

ght shunt at the atrial level. • The most common symptoms were shortness of breath, palpit

ation, and recurrent respiratory tract infections. • On physical examination, all patients had a continuous murm

ur at the right parasternal border.

• The single diagnostic feature is demonstration of this distinct tunnel arising from one of the aortic sinuses of Valsalva and having an extracardiac course and entering into the right atrium

• Treatment options are simple ligation or ligation with implantation of coronary ostium or coil embolization.

sinus valsalva aneurysm seoul national university hospital department of thoracic &...

Documents

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cpb slide