1. Sinus Valsalva Aneurysm Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

2. Sinus Valsalva Aneurysm  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

3. Sinus Valsalva Aneurysm  History 1 st description by Hope in 1839 1 st 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 1 st diagnosis of rupture during life by Venning in 1951 1 st. successful repair with CPB in 1956 at Mayo Clinic & University of Minnesota using CPB

4. Aneurysm of Sinus Valsalva  Clinical features 1Etiology ; 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 2Rupture 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

5. Sinus Valsalva Aneurysm  Etiology 1Separation 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. 2Congenitally weak area gradually gives way under aortic pressure to form an aneurysm. 3The aneurysm appears an excavation of the sinus which protrudes into the underlying cardiac chamber. 4In Asians, the basic abnormality is sited leftward and toward the commissural area between Rt. and Lt. cusp. 5Acquired 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.

6. 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.

7. Sinus Valsalva Aneurysm Unruptured aneurysm of right sinus Valsalva Aneurysm is walled by atrophic muscular tissue of RVOT  Histologic view

8. Sinus Valsalva Aneurysm  Associated cardiac anomalies VSD occurs in 30 to 50%, but may be a little higher in surgical patients. Aortic valve abnormalities & incompetence are common, 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.

9. Sinus Valsalva Aneurysm  Natural History 1Unruptured aneurysms uncommonly cause symptoms, by protrusion into RA and RV, heart block as well as ventricular tachycardia may result. 2Rupture of aneurysm tends to take place in the 3rd or 4th decade of life. 3Once 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 4When 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.

10. Sinus Valsalva Aneurysm  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.

11. Sinus Valsalva Aneurysm Unruptured aneurysm of right sinus Valsalva with VSD  Daigram of aneurysm

12. Sinus Valsalva Aneurysm Ruptured right Sinus Valsalva Aneurysm with VSD  Daigram of aneurysm

13. Sinus Valsalva 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

14. Sinus Valsalva Aneurysm  RVOT obstruction

15. Sinus Valsalva Aneurysm  Rupture 1The sinus of origin is the main determinant of the direction of projection and rupture. 2Gradually develops a more localized windsock, in an unknown percent of cases ultimately rupture into an adjacent low pressure chamber and rarely outside chamber. 3When the aneurysm coexists with a VSD(30-50%), the windsock usually projects into the RV. 4In about one fourth, there is no windsock or any suggestion of aneurysm formation, but rather, a direct fistulous communication. 5Typical windsock deformity may be more common from right sinus lesion, and a direct fistula in noncoronary sinus to RA lesion..

16. Sinus Valsalva Aneurysm

18.  Sites of rupture or fistula 1Aneurysm of the right sinus may originate more centrally and project into the outlet of RV, but leftward portion into region of membranous septum. 2Aneurysms 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. 3Rarely, right or noncoronary sinus aneurysm rupture into LV. 4Aneurysms from left coronary sinus rupture into the LA, LV, but rarely into LV due to thick wall and high pressure. 5Aneurysms rupturing into areas adjacent to TV may be a cause of heart block or RBBB.

19. Sinus Valsalva Aneurysm  Indications for operation 1When ruptured or is associated with VSD or with a VSD and AR, prompt operation is advisable. 2Unruptured aneurysm that are producing hemodynamic derangements should be repaired. 3Small or moderate-sized unruptured aneurysm probably should not be repaired surgically.

20. Sinus Valsalva Aneurysm  Techniques of operation 1Ruptured aneurysm of right sinus Valsalva without VSD 2Ruptured aneurysm of the sinus of Valsalva into the RA without VSD 3Ruptured 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

21.  Techniques of operation Repair of ruptured aneurysm of right sinus Valsalva with VSD Sinus Valsalva Aneurysm

22.  Techniques of operation Repair of unruptured aneurysm of right sinus Valsalva Sinus Valsalva Aneurysm

23.  Techniques of operation Noncoronary sinus of Valsalva aneurysm extending into the right atrium Sinus Valsalva Aneurysm

24.  Techniques of operation Noncoronary sinus of Valsalva aneurysm extending into the right atrium VSD patch closure in case of VSD Sinus Valsalva Aneurysm

25. David-V valve-sparing root replacement using a De Paulis Gelweave Valsalva graft  Techniques of operation

26. Sinus Valsalva Aneurysm  Results of operation 1Survival 2 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

27. Aorta–right Atrial Tunnel  Clinical features Aorta–right atrial tunnel (ARAT) is a very rare abnormal tubular 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.

28. Aorta–right Atrial Tunnel  Clinical features This aorto–right atrial communication behaves like a left-to-right shunt at the atrial level. The most common symptoms were shortness of breath, palpitation, and recurrent respiratory tract infections. On physical examination, all patients had a continuous murmur 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.