eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Sinus of Valsalva Aneurysm

Wai Hong Wilson Tang, MD, Assistant Professor of Medicine, Section of Heart Failure and Cardiac Transplantation Medicine, Cleveland Clinic Foundation
William J Stewart, MD, Associate Professor, Department of Cardiology, The Cleveland Clinic Foundation and Ohio State University

Updated: Jan 28, 2009

Introduction

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.¹ 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.²

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.³ 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.¹

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.⁴ 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%).⁵

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.
A retrospective review of an institutional database identified 86 patients who underwent SVA repair from 1956-2003 found the median age to be 45 years (range 5-80 y).⁶

Clinical

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:⁹
- 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. Clinical suspicion followed by prompt echocardiographic confirmation is key to diagnosis.

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.⁶

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

Differential Diagnoses

Aortic Stenosis	Isolated Coronary Artery Anomalies
Atrial Septal Defect	Ventricular Septal Defect
Atrioventricular Block
Cardiomyopathy, Dilated
Cardiomyopathy, Hypertrophic

Workup

Imaging Studies

Cine magnetic resonance imaging (MRI) is the criterion standard.
Multiplane transesophageal echocardiography (TEE) provides conclusive information regarding SVA and allows precise identification of structural anomalies and shunt locations for perioperative assessment.^9,3
Two-dimensional transthoracic echocardiography (TTE) may detect as many as 75% of all patients with SVA.¹⁰ 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
Three-dimensional TTE is becoming a valuable tool for prompt bedside diagnosis of SVA, especially in ruptured situations.

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

Medical Care

Medical management usually involves stabilization (eg, optimizing medications for heart failure syndrome) and perioperative assessment and management.⁹
Transcatheter closure of ruptured sinus of Valsalva aneurysm (SVA) has been successfully performed using Amplatzer devices.¹¹

Surgical Care

Urgent surgical repair is recommended in all patients with ruptured SVA, especially with intracardiac shunting.^12,13,14 Aggressive surgical correction of unruptured SVA is often recommended because of its association with increased morbidity and mortality.^15,16,6

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

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

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.¹⁶
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.
In a series of 86 patients who underwent SVA repair, ruptures occurred in 34%. Six (7%) died perioperatively; the actuarial 10-year survival rate was 63%. These patients often required concomitant surgical repair of associated ventricular septal defect, atrial septal defect, and the aortic valve.⁶

Miscellaneous

Medicolegal 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

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. Jul 1998;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. Nov-Dec 1997;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. May-Jun 1973;7(3):155-64. [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. Feb 1990;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].
Ferreira AC, de Marchena E, Mayor M, Bolooki H. Sinus of Valsalva aneurysm presenting as myocardial infarction during dobutamine stress test. Cathet Cardiovasc Diagn. Dec 1996;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. Mar 1993;69(3):266-7. [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. Sep-Oct 1991;4(5):485-90. [Medline].
Dev V, Goswami KC, Shrivastava S, Bahl VK, Saxena A. Echocardiographic diagnosis of aneurysm of the sinus of Valsalva. Am Heart J. Oct 1993;126(4):930-6. [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. Mar 2003;58(3):406-11. [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. Apr 1993;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. Jul 1986;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. Mar-Apr 2005;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].

Keywords

sinus of Valsalva aneurysm, SVA, Marfan syndrome, supracristal perimembranous ventricular septal defect, perimembranous ventricular septal defect, bicuspid aortic valve, aortic regurgitation, pulmonary stenosis, coarctation, atrial septal defects, Gerbode defect, cardiac tamponade, aortic media, annulus fibrosus, atherosclerotic aneurysm, syphilis, endocarditis, cystic medial necrosis, chest trauma, deficiency of normal elastic tissue, abnormal development of the bulbus cordis, heart failure syndrome, infective endocarditis, atherosclerosis, ventricular septal defect, aortic insufficiency, coarctation

Contributor Information and Disclosures

Author

Wai Hong Wilson Tang, MD, Assistant Professor of Medicine, Section of Heart Failure and Cardiac Transplantation Medicine, Cleveland Clinic Foundation
Wai Hong Wilson Tang, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, Heart Failure Society of America, and International Society for Heart and Lung Transplantation
Disclosure: Nothing to disclose.

Coauthor(s)

William J Stewart, MD, Associate Professor, Department of Cardiology, The Cleveland Clinic Foundation and Ohio State University
William J Stewart, MD is a member of the following medical societies: American College of Cardiology and American Society of Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Alan D Forker, MD, Professor of Medicine, Program Director of Cardiovascular Fellowship, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research Center, MidAmerica Heart Institute of St Luke's Hospital
Alan D Forker, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Society of Hypertension, and Phi Beta Kappa
Disclosure: Research Grant Grant/research funds Hospital contracts to do research; I am a hospital employee with no personal profit; Speakers Bureau Honoraria Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steven J Compton, MD, FACC, FACP, Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals
Steven J Compton, MD, FACC, FACP is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, and Heart Rhythm Society
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

Further Reading

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].

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].