Sinus of Valsalva Aneurysm Clinical Presentation
- Author: Anuradha Tunuguntla, MD, FACC; Chief Editor: Park W Willis IV, MD more...
Approximately 25% of reported cases of sinus of Valsalva aneurysm (SVA) are clinically asymptomatic, unruptured SVA detected by routine 2-dimensional echocardiography. Rupture of the aneurysmal sac may occur spontaneously, precipitated by exertion, trauma, or cardiac catheterization.
A ruptured SVA progresses in 3 stages as described by Blackshear and colleagues, as follows:
- Acute chest or right upper quadrant pain
- Subacute dyspnea on exertion or at rest (heart failure syndrome) with progressive or acute onset
- Progressive cough, dyspnea, edema, and oliguria
Atypically, SVA presents with infective endocarditis, which may originate at the edges of the aneurysm.
Palpitations or syncope may present secondary to obstruction of the left or right ventricular outflow tract.
Dyspnea is by far the most common presenting symptom.
Unruptured SVA is often asymptomatic and has almost no physical signs. When SVA ruptures, few specific signs of left-to-right shunting may become apparent, and these are often indistinguishable from coronary arteriovenous fistula. Clinical suspicion followed by prompt echocardiographic confirmation is key to diagnosis.
Signs include the following:
- A loud, superficial, "machine-type" continuous murmur is accentuated in diastole in as many as 40% of patients.
- A palpable thrill along the right or left lower parasternal border is occasionally noticeable.
- Bounding pulses are occasionally present.
- In one series, approximately 44% of the patients had associated aortic regurgitation.
Primary causes are congenital.
Secondary causes include the following:
- Cystic medial necrosis or Marfan syndrome
- Blunt or penetrating chest injury
- Infective endocarditis
Associated congenital defects include the following:
- Ventricular septal defect
- Aortic insufficiency
Ring WS. Congenital Heart Surgery Nomenclature and Database Project: Aortic Aneurysm, Sinus of Valsalva Aneurysm, and Aortic Dissection. Ann Thorac Surg. 2000. 69:S147-S163. [Medline].
Meier JH, Seward JB, Miller FA, et al. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr. 1998 Jul. 11(7):729-45. [Medline].
Wang KY, St John Sutton M, Ho HY, Ting CT. Congenital sinus of Valsalva aneurysm: a multiplane transesophageal echocardiographic experience. J Am Soc Echocardiogr. 1997 Nov-Dec. 10(9):956-63. [Medline].
Prian GW, Diethrich EB. Sinus of Valsalva abnormalities. A specific differentiation between aneurysms of an aneurysms involving the sinuses of Valsalva. Vasc Surg. 1973 May-Jun. 7(3):155-64. [Medline].
Raslan S, Nanda NC, Lloyd L, Khairnar P, Reilly SD, Holman WL. Incremental value of live/real time three-dimensional transesophageal echocardiography over the two-dimensional technique in the assessment of sinus of valsalva aneurysm rupture. Echocardiography. 2011 Sep. 28(8):918-20. [Medline].
Chu SH, Hung CR, How SS, Chang H, Wang SS, Tsai CH, et al. Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg. 1990 Feb. 99(2):288-98. [Medline].
Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, et al. Sinus of Valsalva aneurysms--47 years of a single center experience and systematic overview of published reports. Am J Cardiol. Apr 2007. 99:1159-64. [Medline].
Blackshear JL, Safford RE, Lane GE, Freeman WK, Schaff HV. Unruptured noncoronary sinus of Valsalva aneurysm: preoperative characterization by transesophageal echocardiography. J Am Soc Echocardiogr. 1991 Sep-Oct. 4(5):485-90. [Medline].
Ferreira AC, de Marchena E, Mayor M, Bolooki H. Sinus of Valsalva aneurysm presenting as myocardial infarction during dobutamine stress test. Cathet Cardiovasc Diagn. 1996 Dec. 39(4):400-2. [Medline].
Shahrabani RM, Jairaj PS. Unruptured aneurysm of the sinus of Valsalva: a potential source of cerebrovascular embolism. Br Heart J. 1993 Mar. 69(3):266-7. [Medline].
Dev V, Goswami KC, Shrivastava S, Bahl VK, Saxena A. Echocardiographic diagnosis of aneurysm of the sinus of Valsalva. Am Heart J. 1993 Oct. 126(4):930-6. [Medline].
Thankavel PP, Lemler MS, Ramaciotti C. Unruptured Sinus of Valsalva Aneurysm in a Neonate with Hypoplastic Left Heart Syndrome: Echocardiographic Diagnosis and Features. Echocardiography. 2013 Dec 23. [Medline].
Vatankulu MA, Tasal A, Erdogan E, Sonmez O, Goktekin O. The role of three-dimensional echocardiography in diagnosis and management of ruptured sinus of valsalva aneurysm. Echocardiography. 2013 Sep. 30(8):E260-2. [Medline].
Fedson S, Jolly N, Lang RM, Hijazi ZM. Percutaneous closure of a ruptured sinus of Valsalva aneurysm using the Amplatzer Duct Occluder. Catheter Cardiovasc Interv. 2003 Mar. 58(3):406-11. [Medline].
Gioia G, Zheng J, Ray A, Gioia M. Perforated Sinus of Valsalva (PSOV) aneurysm closure with a muscular VSD occluder. Cardiovasc Revasc Med. 2013 Nov 8. [Medline].
Zhong L, Tong SF, Zhang Q, Zhang ZH, Yao Q, Li YH, et al. Clinical efficacy and safety of transcatheter closure of ruptured sinus of valsalva aneurysm. Catheter Cardiovasc Interv. 2013 Dec 18. [Medline].
Flynn MS, Castello R, McBride LW, Labovitz AJ. Ruptured congenital aneurysm of the sinus of Valsalva with persistent left superior vena cava imaged by intraoperative transesophageal echocardiography. Am Heart J. 1993 Apr. 125(4):1185-7. [Medline].
Mayer ED, Ruffmann K, Saggau W, Butzmann B, Bernhardt-Mayer K, Schatton N. Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg. 1986 Jul. 42(1):81-5. [Medline].
Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg. 1999. 68(5):1573-7. [Medline].
Harkness JR, Fitton TP, Barreiro CJ, et al. A 32-year experience with surgical repair of sinus of valsalva aneurysms. J Card Surg. 2005 Mar-Apr. 20(2):198-204. [Medline].
Zikri MA, Stewart RW, Cosgrove DM. Surgical correction for sinus of Valsalva aneurysm. J Cardiovasc Surg (Torino). 1999. 40(6):787-91. [Medline].
Menon S, Kottayil B, Panicker V, Pillai V, Karunakaran J. Ruptured sinus of Valsalva aneurysm: 10-year Indian surgical experience. Asian Cardiovasc Thorac Ann. 2011 Oct. 19(5):320-3. [Medline].
Altekin RE, Karakas MS, Er A, Yanikoglu A, Ozbek S, Yilmaz H. Percutaneous closure of ruptured sinus of Valsalva aneursym with Amplatzer ductal occluder. Acta Cardiol. 2011 Oct. 66(5):657-60. [Medline].
Kloppenburg GT, Sonker U, Post MC, Yilmaz A, Morshuis WJ. Emergency surgery for ruptured sinus of valsalva aneurysms. Scand Cardiovasc J. 2011 Dec. 45(6):374-8. [Medline].