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Aneurysm of sinus of Valsalva(SVA)

MedGen UID:
853730
•Concept ID:
C2239253
•
Disease or Syndrome
Synonyms: Sinus of Valsalva aneurysm; SVA
SNOMED CT: Sinus of Valsalva aneurysm (54160000)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0015197
Orphanet: ORPHA1054

Definition

A rare congenital heart malformation of one or more of the aortic sinuses, consisting of a dilation that when unruptured is usually asymptomatic but when ruptured presents with progressive exertional dyspnea, fatigue, chest pain and that can lead to congestive heart failure if left untreated. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAneurysm of sinus of Valsalva

Professional guidelines

PubMed

Aorta-right atrial tunnel: clinical presentation, diagnostic criteria, and surgical options.
Gajjar T, Voleti C, Matta R, Iyer R, Dash PK, Desai N
J Thorac Cardiovasc Surg 2005 Nov;130(5):1287-92. doi: 10.1016/j.jtcvs.2005.07.021. PMID: 16256780
Diagnosis and surgical treatment of ruptured aneurysm in sinus of Valsalva.
Zhao G, Seng J, Yan B, Wei H, Qiao C, Zhao S, Zhao W, Zhi X
Chin Med J (Engl) 2003 Jul;116(7):1047-50. PMID: 12890381
Ruptured sinus of valsalva aneurysm: management options.
Hijazi ZM
Catheter Cardiovasc Interv 2003 Jan;58(1):135-6. doi: 10.1002/ccd.10421. PMID: 12508217

Recent clinical studies

Etiology

Surgical correction of ruptured aneurysms of the sinus of Valsalva using on-pump beating-heart technique.
Mo A, Lin H
J Cardiothorac Surg 2010 May 14;5:37. doi: 10.1186/1749-8090-5-37. PMID: 20470371Free PMC Article
Ruptured congenital sinus of valsalva aneurysms.
Lin CY, Hong GJ, Lee KC, Tsai YT, Tsai CS
J Card Surg 2004 Mar-Apr;19(2):99-102. doi: 10.1111/j.0886-0440.2004.04022.x. PMID: 15016044
Aneurysm of sinus of Valsalva dissecting into interventricular septum.
Choudhary SK, Bhan A, Reddy SC, Sharma R, Murari V, Airan B, Kumar AS, Venugopal P
Ann Thorac Surg 1998 Mar;65(3):735-40. doi: 10.1016/s0003-4975(97)01432-x. PMID: 9527204
Sinus of Valsalva aneurysms: 20 years' experience.
Choudhary SK, Bhan A, Sharma R, Airan B, Kumar AS, Venugopal P
J Card Surg 1997 Sep-Oct;12(5):300-8. doi: 10.1111/j.1540-8191.1997.tb00143.x. PMID: 9635267
Aneurysms of the sinus of Valsalva.
Yilmaz AT, Demirkiliç U, Ozal E, Tatar H, Oztürk OY
J Cardiovasc Surg (Torino) 1997 Apr;38(2):119-24. PMID: 9201120

Diagnosis

A giant aneurysm of sinus of Valsalva presenting with frequent syncopal attacks.
Zanganehfar ME, Shahandashti FJ, Shriati A, Tayebi A, Saedi S
Asian Cardiovasc Thorac Ann 2022 Nov;30(9):1023-1024. Epub 2022 Sep 7 doi: 10.1177/02184923221123900. PMID: 36071598
Diagnostic paradox: Ruptured aneurysm of sinus of Valsalva simulating tricuspid valve endocarditis.
Hoda M, Verma A, Alapati S, Alapati S, Yarrabolu TR
Echocardiography 2017 Mar;34(3):465-467. Epub 2017 Feb 18 doi: 10.1111/echo.13479. PMID: 28213924
Aneurysm of sinus of Valsalva.
Tipoo Sultan FA, Basir N, Fatimi S
J Coll Physicians Surg Pak 2011 Mar;21(3):173-5. PMID: 21419027
Aneurysm of sinus of Valsalva dissecting into interventricular septum.
Choudhary SK, Bhan A, Reddy SC, Sharma R, Murari V, Airan B, Kumar AS, Venugopal P
Ann Thorac Surg 1998 Mar;65(3):735-40. doi: 10.1016/s0003-4975(97)01432-x. PMID: 9527204
Sinus of Valsalva aneurysms: 20 years' experience.
Choudhary SK, Bhan A, Sharma R, Airan B, Kumar AS, Venugopal P
J Card Surg 1997 Sep-Oct;12(5):300-8. doi: 10.1111/j.1540-8191.1997.tb00143.x. PMID: 9635267

Therapy

Severe aortic regurgitation after the closure of a ruptured congenital aneurysm of sinus of Valsalva with an Amplatzer device.
Dobarro D, Sánchez Recalde A, Martín Reyes R
EuroIntervention 2010 Aug;6(3):426-7. doi: 10.4244/EIJV6I3A71. PMID: 20884426
Coexistent congenital aortic defects, aneurysm of sinus of valsalva, atrial septal defect and infective endocarditis: a case report.
Mundo-Sagardía JA, Johnson C, Calderón R, Quintana C
P R Health Sci J 2006 Sep;25(3):273-8. PMID: 17203799
Aneurysm of sinus of Valsalva dissecting into interventricular septum: a late complication of aortic valve replacement.
Güler N, Eryonucu B, Tuncer M, Aşker M
Echocardiography 2004 Oct;21(7):645-8. doi: 10.1111/j.0742-2822.2004.03128.x. PMID: 15488095
Aortic valve replacement in children: are mechanical prostheses a good option?
Alexiou C, McDonald A, Langley SM, Dalrymple-Hay MJ, Haw MP, Monro JL
Eur J Cardiothorac Surg 2000 Feb;17(2):125-33. doi: 10.1016/s1010-7940(00)00324-9. PMID: 10731647
Traumatic hemolysis with rupture of aneurysm of sinus of Valsalva.
Ellman L, Knox-Macauley H
Arch Intern Med 1970 Dec;126(6):1019-21. PMID: 5483597

Prognosis

Unusual clinical course after surgical repair of unruptured aneurysm of sinus of Valsalva.
Sato Y, Kawasaki T, Yamano M, Honda S, Harimoto K, Miki S, Kamitani T, Matoba S
J Med Ultrason (2001) 2016 Oct;43(4):523-6. Epub 2016 May 21 doi: 10.1007/s10396-016-0721-1. PMID: 27209286
Ruptured congenital sinus of valsalva aneurysms.
Lin CY, Hong GJ, Lee KC, Tsai YT, Tsai CS
J Card Surg 2004 Mar-Apr;19(2):99-102. doi: 10.1111/j.0886-0440.2004.04022.x. PMID: 15016044
Aneurysm of sinus of Valsalva dissecting into interventricular septum.
Choudhary SK, Bhan A, Reddy SC, Sharma R, Murari V, Airan B, Kumar AS, Venugopal P
Ann Thorac Surg 1998 Mar;65(3):735-40. doi: 10.1016/s0003-4975(97)01432-x. PMID: 9527204
Aneurysms of the sinus of Valsalva.
Yilmaz AT, Demirkiliç U, Ozal E, Tatar H, Oztürk OY
J Cardiovasc Surg (Torino) 1997 Apr;38(2):119-24. PMID: 9201120
Ruptured aneurysm of sinus of Valsalva. Long-term postoperative follow-up.
Mattila SP, Kupari M, Harjula AL, Ventilä M, Meurala H, Maamies TJ, Aho P
Scand J Thorac Cardiovasc Surg 1987;21(3):233-8. doi: 10.3109/14017438709106031. PMID: 3438719

Clinical prediction guides

Supracristal ventricular septal defect in adults: Is it time for a paradigm shift?
Egbe AC, Poterucha JT, Dearani JA, Warnes CA
Int J Cardiol 2015 Nov 1;198:9-14. Epub 2015 Jul 2 doi: 10.1016/j.ijcard.2015.06.100. PMID: 26149330
Prognostic implications of initial echocardiographic findings in adolescents and adults with supracristal ventricular septal defects.
Cho MS, Jang SJ, Sun BJ, Jang JY, Ahn JM, Kim DH, Song JM, Kang DH, Song JK
J Am Soc Echocardiogr 2014 Sep;27(9):965-71. Epub 2014 Jul 1 doi: 10.1016/j.echo.2014.05.016. PMID: 24996557
Surgical correction of ruptured aneurysms of the sinus of Valsalva using on-pump beating-heart technique.
Mo A, Lin H
J Cardiothorac Surg 2010 May 14;5:37. doi: 10.1186/1749-8090-5-37. PMID: 20470371Free PMC Article
Aortic root replacement: twenty years of experience.
Casali G, Luciani GB, Pugliese P, Mazzucco A
G Ital Cardiol 1998 Jul;28(7):745-53. PMID: 9773298
Echocardiography diagnosis of ruptured aneurysm of sinus of Valsalva. Report of two cases.
Matsumoto M, Matsuo H, Beppu S, Yoshioka Y, Kawashima Y, Nimure Y, Abe H
Circulation 1976 Feb;53(2):382-9. doi: 10.1161/01.cir.53.2.382. PMID: 1245046

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