AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

John Thurnam first described sinus of Valsalva aneurysm (SVA) in 1840. Hope further described it in 1939. SVA is usually referred to as a rare congenital anomaly. A congenital SVA is usually clinically silent but may vary from a mild, asymptomatic dilatation detected in routine 2-dimensional echocardiography to symptomatic presentations related to the compression of adjacent structures or intracardiac shunting caused by rupture of the SVA into the right side of the heart. Approximately 65-85% of SVAs originate from the right sinus of Valsalva, while SVAs originating from noncoronary (10-30%) and left sinuses (<5%) are exceedingly rare.

Pathophysiology

Congenital SVA is caused by a dilation, usually of a single sinus of Valsalva, from a separation between the aortic media and the annulus fibrosus. A deficiency of normal elastic tissue and abnormal development of the bulbus cordis have been associated with the development of SVA. Other disease processes that involve the aortic root (eg, atherosclerotic aneurysms, syphilis, endocarditis, cystic medial necrosis, chest trauma) may also produce SVA, although this usually involves multiple sinuses. Rupture of the dilated sinus may lead to intracardiac shunting when a communication is established with the right atrium (Gerbode defect [10%]) or directly into the right ventricle (60-90%). Cardiac tamponade may occur if the rupture involves the pericardial space.

Frequency

United States

SVA was present in 0.09% of cadavers in a large autopsy series and ranged to 0.14-0.23% in a Western surgical series. Two-dimensional echocardiography is likely to determine a higher incidence of SVA.

International

SVA is more prevalent in Asian surgical series (0.46-3.5%) and correlates with more supracristal ventricular septal defects (~60%).

Mortality/Morbidity

The true natural history of SVA is unclear. Clinical complications from SVA are often the initial presentation of SVA (see Complications).

• Associated structural defects in congenital SVAs included supracristal or perimembranous ventricular septal defect (30-60%), bicuspid aortic valve (15-20%) and aortic regurgitation (44-50%). Approximately 10% of patients with Marfan syndrome have some form of SVA. Less commonly observed anomalies include pulmonary stenosis, coarctation, and atrial septal defects.
• Rupture of SVA (with progressive heart failure and left-to-right shunting or endocarditis) is the main cause of death and rarely occurs before age 20 years in congenital SVA.

Race

Race differences in SVA are unclear, although a higher frequency was observed in the Asian surgical series.

Sex

Male-to-female ratio is 4:1, including frequencies of both ruptured and unruptured SVA.

Age

• Unruptured SVA is usually asymptomatic and is often detected serendipitously by routine 2-dimensional echocardiography, even in patients older than 60 years.
• Most ruptured SVAs occur from puberty to age 30 years and are often diagnosed or presented clinically at this age.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

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:
• • 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.

Physical

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.

• 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.

Causes

• Primary causes - Congenital
• Secondary causes
• • Atherosclerosis
  • Syphilis
  • Cystic medial necrosis or Marfan syndrome
  • Blunt or penetrating chest injury
  • Infective endocarditis
• Associated congenital defects
• • Ventricular septal defect
  • Aortic insufficiency
  • Coarctation

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Chest pain
Arrhythmia/syncope

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Imaging Studies

• Cine magnetic resonance imaging (MRI) is the criterion standard.
• Multiplane transesophageal echocardiography (TEE) provides conclusive information regarding sinus of Valsalva aneurysm (SVA) and allows precise identification of structural anomalies and shunt locations for perioperative assessment.
• Two-dimensional transthoracic echocardiography (TTE) may detect as many as 75% of all patients with SVA. Usually TEE or MRI is needed to confirm the diagnosis and for perioperative assessment. Features of TTE include the following:
• • Generalized single sinus enlargement
  • "Wind-sock" extension of sinus from body and/or apex of otherwise normal aortic sinus when ruptured
  • Lack of associated lesions such as ventricular septal defect

Other Tests

• Electrocardiogram usually presents with sinus tachycardia, although conduction defects may occur.

Procedures

• Although rarely necessary, the definitive diagnosis can be confirmed by performing a retrograde thoracic aortography or cardiac catheterization. Left-to-right shunting also can be demonstrated if SVA is ruptured.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

• Medical management usually involves stabilization (eg, optimizing medications for heart failure syndrome) and perioperative assessment and management.
• Transcatheter closure of ruptured sinus of Valsalva aneurysm (SVA) has been successfully performed using Amplatzer devices.

Surgical Care

Urgent surgical repair is recommended in all patients with ruptured SVA, especially with intracardiac shunting. Aggressive surgical correction of unruptured SVA is often recommended because of its association with increased morbidity and mortality.

• Aortic root reconstruction or replacement
• Aortic valve repair or replacement
• Bentall procedure (valved conduit)
• Ventricular septal defect repair (if present)
• Atrial septal defect repair (if present)
• Primary suture closures (pledget) and patch closures (if ruptured)

Consultations

Cardiothoracic surgery consultation is urgent in patients with ruptured SVA because clinical deterioration can be rapid.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Surgery is the definitive therapy for sinus of Valsalva aneurysm. Depending on the clinical presentation, perioperative medical management consists of (1) relieving heart failure symptoms, (2) treating arrhythmia if present, and (3) treating endocarditis if present. The reader is encouraged to read corresponding articles for appropriate medication therapy.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Complications

• Myocardial infarction (from coronary arterial compression by adjacent unruptured sinus of Valsalva aneurysm [SVA])
• Complete heart block (from compression of conduction tissues by adjacent unruptured SVA)
• Right ventricular outflow tract obstruction
• Sudden cardiac death
• Infective endocarditis
• Tamponade if ruptured into the pericardium
• Rarely, a potential source of cerebrovascular emboli

Prognosis

• 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.
• Unruptured SVA has 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.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• A possible source of medicolegal trouble is failure to consider sinus of Valsalva aneurysm (SVA) rupture in young patients (especially those with minimal cardiac risk factors) who have signs and symptoms of myocardial ischemia, heart failure, or cardiac conduction defects. Definitive treatment is available, yet the prognosis is poor if rapid diagnosis cannot be made.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Blackshear JL, Safford RE, Lane GE, et al. Unruptured noncoronary sinus of Valsalva aneurysm: preoperative characterization by transesophageal echocardiography. J Am Soc Echocardiogr. 1991;4:485-90.
• Chu HS, Hung CR, How SS, et al. Ruptured aneurysms of the sinus of Valsalva in Oriental Patients. J Thorac Cardiovasc Surg. 1990;99:288-298.
• Dev V, Goswami KC, Shrivastava S, et al. Echocardiographic diagnosis of aneurysm of the sinus of Valsalva. Am Heart J. 1993;126:930-6.
• 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. Mar 2003;58(3):406-11. [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;39:400.
• 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;125:1185.
• Harkness JR, Fitton TP, Barreiro CJ, et al. A 32-year experience with surgical repair of sinus of valsalva aneurysms. J Card Surg. Mar-Apr 2005;20(2):198-204. [Medline].
• Mayer ED, Ruffmann K, Saggau W, et al. Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg. 1986;42:81-5.
• 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. Jul 1998;11(7):729-45. [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;7:155.
• 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].
• Shahrabani RM, Jairaj PS. Unruptured aneurysm of the sinus of Valsalva: a potential source of cerebrovascular embolism. Br Heart J. 1993;69:266.
• 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].
• Wang KY, Sutton MSJ, Ho HY, Ting CT. Congenital Sinus of Valsalva Anuerysm: A Multiplane Transesophageal Echocardiographic Experience. J Am Soc Echocardiogr. 1997;10:956-963.
• Zikri MA, Stewart RW, Cosgrove DM. Surgical correction for sinus of Valsalva aneurysm. J Cardiovasc Surg (Torino). 1999;40(6):787-91. [Medline].

Article Last Updated: Feb 2, 2006