Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Sinus of Valsalva Aneurysm Clinical Presentation

  • Author: Edward J Bayne, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Feb 06, 2015
 

History

Children with sinus of Valsalva aneurysm are most often asymptomatic. Symptoms typically present in young adulthood (usually in patients < 30 y) either due to enlargement of the aortic root and compression of surrounding structures or due to manifestations of a ruptured aneurysm. Three clinical pictures may be associated with sinus of Valsalva aneurysm, as follows:

  • Sudden massive rupture may occur after strenuous exertion and may be signaled by acute chest or epigastric pain with dyspnea. Symptoms may be confused with those of acute myocardial infarction. [3]
  • Patients with a smaller insidious rupture may be asymptomatic, but small ruptures also may be associated with progressive symptoms of exertional dyspnea and/or chest discomfort from advancing heart failure. [9]
  • Patients with unruptured aneurysms may be asymptomatic. Angina may occur secondary to coronary compression resulting from an unruptured aneurysm. Syncope or dizziness may be caused by aneurysm compression of the conduction system, with associated heart block (Adams-Stokes syndrome).
Next

Physical

Positive physical findings may be absent in a patient with an unruptured sinus of Valsalva aneurysm. Physical signs of a ruptured aneurysm vary, depending on the location of the shunt, and may mimic signs observed in a patient with a sizable coronary arteriovenous (AV) fistula. Physical signs may include the following[10] :

  • A loud continuous murmur, accentuated in the diastole, occurs with aneurysm rupture into the right ventricle or right atrium. The systolic component of the continuous murmur is usually heard best higher in the chest, whereas the diastolic component may be best heard lower along the sternal border.
  • A parasternal thrill is heard from associated ventricular septal defect with large volume of runoff or, possibly, outflow obstruction.
  • Bounding pulses occur as a result of aortic runoff into lower-pressure chambers.
  • Shunt from the aortic root to the left ventricle may produce a diastolic murmur similar to that of aortic insufficiency.
  • Pulmonary rales may be present from progressive left heart failure.
  • With an unruptured aneurysm partially obstructing the right ventricular outflow tract, an ejection murmur may be heard at the left base radiating into the back.
Previous
Next

Causes

Sinus of Valsalva aneurysm is presumed to be caused by a spontaneous genetic mutation. Although the defect is inherited, no distinct pattern of inheritance has been noted. Frank aneurysmal dilatation is rarely seen at birth.

Subpulmonic ventricular septal defect may be an important contributing factor in the progression of sinus of Valsalva aneurysms.

Tertiary syphilis is of historical significance as a cause for aortic aneurysms.

Traumatic injury to the aortic root (usually from direct chest compression) may rarely cause rupture of the aortic root.

A number of generalized disorders may be associated with dilatation and/or distortion of the aortic root, including Marfan syndrome, Ehlers-Danlos syndrome, Turner syndrome, Williams syndrome, aortic valve, bicuspid, and osteogenesis imperfecta.

Previous
 
 
Contributor Information and Disclosures
Author

Edward J Bayne, MD Assistant Professor, Division of Pediatric Cardiology, Emory University School of Medicine

Edward J Bayne, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Echocardiography, American College of Cardiology, American Heart Association

Disclosure: Nothing to disclose.

Coauthor(s)

Lynn Cronin, MD Clinical Cardiology Fellow, Department of Pediatrics, Division of Cardiology, William Beaumont Hospital

Lynn Cronin, MD is a member of the following medical societies: American College of Physicians, American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

References
  1. Ring WS. Congenital Heart Surgery Nomenclature and Database Project: aortic aneurysm, sinus of Valsalva aneurysm, and aortic dissection. Ann Thorac Surg. 2000 Apr. 69(4 Suppl):S147-63. [Medline].

  2. Magee R. A Cardiac Clinico-Pathological Conference in 1882 an historical vignette. Heart Lung Circ. 2004 Sep. 13(3):322-5. [Medline].

  3. Braunwald E, ed. Heart Disease: A Textbook of Cardiovascular Medicine. Philadelphia, PA: WB Saunders Co; 1996.

  4. Abad C. Congenital aneurysm of the sinus of Valsalva dissecting into the interventricular septum. Cardiovasc Surg. 1995 Oct. 3(5):563-4. [Medline].

  5. Fazio G, Zito R, Dioco DD, Mussagy C, et al. Rupture of a left sinus of Valsalva aneurysm into the pulmonary artery. Eur J Echocardiogr. 2006 Jun. 7(3):230-2. [Medline].

  6. Choudhary SK, Bhan A, Sharma R, et al. Sinus of Valsalva aneurysms: 20 years' experience. J Card Surg. 1997 Sep-Oct. 12(5):300-8. [Medline].

  7. Dong C, Wu QY, Tang Y. Ruptured sinus of valsalva aneurysm: a Beijing experience. Ann Thorac Surg. 2002 Nov. 74(5):1621-4. [Medline].

  8. Lin CY, Hong GJ, Lee KC, Tsai YT, Tsai CS. Ruptured congenital sinus of valsalva aneurysms. J Card Surg. 2004 Mar-Apr. 19(2):99-102. [Medline].

  9. Perloff JK, Child JS. Congenital Heart Disease in Adults. 2nd ed. Philadelphia, PA: WB Saunders Co; 1997.

  10. Perloff JK. The Clinical Recognition of Congenital Heart Disease. 4th ed. Philadelphia, PA: WB Saunders Co; 1994.

  11. Pamulapati M, Teague S, Stelzer P, Thadani U. Successful surgical repair of a ruptured aneurysm of the sinus of Valsalva in early pregnancy. Ann Intern Med. 1991 Dec 1. 115(11):880-2. [Medline].

  12. Thankavel PP, Lemler MS, Ramaciotti C. Unruptured sinus of valsalva aneurysm in a neonate with hypoplastic left heart syndrome: echocardiographic diagnosis and features. Echocardiography. 2014 Mar. 31(3):E85-7. [Medline].

  13. Cho MS, Jang SJ, Sun BJ, Jang JY, Ahn JM, Kim DH, et al. Prognostic implications of initial echocardiographic findings in adolescents and adults with supracristal ventricular septal defects. J Am Soc Echocardiogr. 2014 Sep. 27(9):965-71. [Medline].

  14. Tipoo Sultan FA, Basir N, Fatimi S. Aneurysm of sinus of Valsalva. J Coll Physicians Surg Pak. 2011 Mar. 21(3):173-5. [Medline].

  15. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007 Oct 9. 116(15):1736-54. [Medline]. [Full Text].

  16. Yacoub MH, Khan H, Stavri G, et al. Anatomic correction of the syndrome of prolapsing right coronary aortic cusp, dilatation of the sinus of Valsalva, and ventricular septal defect. J Thorac Cardiovasc Surg. 1997 Feb. 113(2):253-60; discussion 261. [Medline].

  17. Zikri MA, Stewart RW, Cosgrove DM. Surgical correction for sinus of Valsalva aneurysm. J Cardiovasc Surg (Torino). 1999 Dec. 40(6):787-91. [Medline].

  18. Stroeh K, Schreiber C, Henze R, Lange R. Surgical correction of a congenital coronary arterial fistula and a massive sinus of valsalva aneurysm. Interact Cardiovasc Thorac Surg. 2012 Aug 7. [Medline].

  19. Kuriakose EM, Bhatla P, McElhinney DB. Comparison of reported outcomes with percutaneous versus surgical closure of ruptured sinus of Valsalva aneurysm. Am J Cardiol. 2015 Feb 1. 115(3):392-8. [Medline].

  20. Cullen S, Somerville J, Redington A. Transcatheter closure of a ruptured aneurysm of the sinus of Valsalva. Br Heart J. 1994 May. 71(5):479-80. [Medline].

  21. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an Amplatzer occluder device. Heart. 2005 Feb. 91(2):244. [Medline].

  22. Arora R, Trehan V, Rangasetty UM. Transcatheter closure of ruptured sinus of valsalva aneurysm. J Interv Cardiol. 2004 Feb. 17(1):53-8. [Medline].

  23. Jean WH, Kang TJ, Liu CM, et al. Transcatheter occlusion of ruptured sinus of Valsalva aneurysm guided by three-dimensional transesophageal echocardiography. J Formos Med Assoc. 2004 Dec. 103(12):948-51. [Medline].

  24. Rao PS, Bromberg BI, Jureidini SB, Fiore AC. Transcatheter occlusion of ruptured sinus of valsalva aneurysm: innovative use of available technology. Catheter Cardiovasc Interv. 2003 Jan. 58(1):130-4. [Medline].

  25. Narin N, Ozyurt A, Baykan A, Uzüm K. Transcatheter closure of ruptured sinus Valsalva aneurysm with retrograde approach. Turk Kardiyol Dern Ars. 2014 Apr. 42(3):299-301. [Medline].

  26. Shaddy RE, Tani LY, Gidding SS, et al. Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children: a multi-institutional experience. J Heart Lung Transplant. 1999 Mar. 18(3):269-74. [Medline].

  27. McMahon CJ, Ayres N, Pignatelli RH, et al. Echocardiographic presentations of endocarditis, and risk factors for rupture of a sinus of Valsalva in childhood. Cardiol Young. 2003 Apr. 13(2):168-72. [Medline].

  28. El Hattaoui M, Charei N, Boumzebra D, Chraibi S, Bennis A. A large aneurysm of a left sinus of Valsalva invading the interventricular septum--a rare cause of syncope. Can J Cardiol. 2008 May. 24(5):e28-9. [Medline].

  29. Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg. 1999 Nov. 68(5):1573-7. [Medline].

  30. Li ZQ, Liu AJ, Li XF, Zhu YB, Liu YL. Progression of aortic regurgitation in Asian patients with congenital sinus of valsalva aneurysm. Heart Surg Forum. 2013 Aug 1. 16(4):E219-24. [Medline].

  31. Bricker AO, Avutu B, Mohammed TL, Williamson EE, Syed IS, Julsrud PR, et al. Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics. 2010 Jan-Feb. 30(1):99-110. [Medline].

 
Previous
Next
 
Sinus of Valsalva aneurysm. Color-flow Doppler ultrasonography is performed in the right ventricle through a supracristal ventricular septal defect with fingerlike prolapse of the right coronary sinus wall (arrow).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.