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Sva
- 1. SINUS OF VALSALVAANEURYSM 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 ofValsalva 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 timeshigher 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 trigonesunderneath 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 EchocardiographicRelationship 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 ofthe 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% • RVOTobstruction :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 • Rupturedaneurysms 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 rightventricle 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%)
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- 19. Sakakibara S, KonnoS. 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 arisingfrom 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 portionof 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 presentsbased 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 Rupturedaneurysm • 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 • Suddenappearance 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 sinustachycardia 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 • Itis 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
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- 35. CMR The advantages ofperforming 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 • CTis less time consuming and the preferred investigation compared to MRI in case of acute setting of aneurysmal rupture
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- 40. Medication Summary • Dependingon 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 patientssurvive 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 ofruptured 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
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- 49. Outcome • Unruptured SVAhas 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 congenitalSVA 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
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