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  1. 1. SINUS OF VALSALVA ANEURYSM Moderator- Dr.RV KUMAR Presenter- Dr.Jyotindra Singh
  2. 2. INTRODUCTION Thin 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
  3. 3. 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
  4. 4. AORTIC ROOT- ANATOMY Young adults AA>STJ Adults AA = STJ Elderly AA<STJ
  5. 5. ROOT ANATOMY . 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.
  6. 6. Anatomic and Echocardiographic Relationship Between the Components of the Normal Aortic Root 120 degree - LAX Annulus Sinuses Systole ST junction Tubular aorta Diastole
  7. 7. SINUS OF VALSALVA ring.
  8. 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. Magnetic resonance imaging has shown- in aiding leaflet opening through the creation of a lowpressure 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
  9. 9. 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.
  10. 10. CONGENITAL Connective tissue disordersRheumatoid arthritis, Ehlers-Danhlos syndrome, Marfan’s syndrome, Klippel Feil syndrome, Turner’s syndrome, Trisomies 13 and 15, Loeys-Dietz syndrome, Arachnodactyly, Osteogenesis imperfecta. ACQUIRED 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
  11. 11. ACQUIRED
  12. 12. 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.
  13. 13. Congenital Heart Surgery Database Committee of the Society of Thoracic Surgeons
  14. 14. 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
  15. 15. 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
  16. 16. Associated cardiac anomalies - VSD Prevalence – 30 to 50 % Incidence higher in Rt.sinus Left third of right aortic sinusJUXTA – 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
  17. 17. 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
  18. 18. OTHER ANOMALIES Aortic regurgitation results from prolapsed aortic cusp Bicuspid aortic valve leading to AR. Pulmonary stenosis Aortic coarctation PDA ASD Subaortic stenosis TOF
  19. 19. 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%) The right ventricle is the most common receiving chamber (about 80–90%), due to rupture of either right or noncoronary SVA
  20. 20. 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 aneurysm – Extra cardiac
  22. 22. 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.
  23. 23. 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
  24. 24. JVP in Ruptured aneurysm Rupture into the Right ventriclethe severe diastolic ventricular volume-overload causes Obliteration of the y-descent Rupture into the right atrium obliterates the x-descent. The high Right atrial pressureEarly tricuspid opening Premature v-wave, High peaked a-wave with a fourth heart sound.
  25. 25. 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
  26. 26. 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
  27. 27. ECG ECG showing sinus tachycardia with 1st degree AV block and right bundle branch block. Compression of the His bundle occurs when the ruptured SVA penetrates the base of the interventricular septum and results in atriovenricular Ventricular tachycardias arising from the aortic sinus of Valsalva: An under-recognized variant of left outflow tract ventricular tachycardia
  28. 28. 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
  30. 30. 35
  31. 31. AORTOGRAM Right-sided pressures were elevated with a right atrial pressure of 12 mmHg, occur. 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. -
  32. 32. 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.
  33. 33. Indications for Surgery
  34. 34. 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 followup . If high likelihood of progressive increase in size and the possibility of rupture or endocarditis.
  35. 35. 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
  36. 36. 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.
  37. 37. Single/Double patch repair 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.
  38. 38. 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.
  39. 39. 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.
  40. 40. 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
  41. 41. 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
  42. 42. SOVA- RESULTS
  43. 43. 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.