Sinus of Valsalva Aneurysm

Updated: Dec 14, 2020
  • Author: Arnold S Baas, MD, FACC, FACP; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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Overview

Background

Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac anomaly that can be congenital or acquired. John Thurnam first described this condition in 1840. James 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 on routine two-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, whereas SVAs originating from noncoronary (10-30%) and left sinuses (< 5%) are exceedingly rare. [1]  SVA is commonly associated with aortic valve regurgitation and/or ventricular septal defect. [2]

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Pathophysiology

Congenital sinus of Valsalva aneurysm (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. [4]

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Etiology

Primary causes of sinus of Valsalva aneurysm (SVA) are congenital.

Secondary causes include the following:

  • Atherosclerosis
  • Syphilis
  • Cystic medial necrosis or Marfan syndrome
  • Blunt or penetrating chest injury
  • Infective endocarditis

Associated congenital defects include the following:

  • Ventricular septal defect
  • Aortic insufficiency
  • Coarctation
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Epidemiology

United States data

Sinus of Valsalva aneurysm (SVA) was present in 0.09% of cadavers in a large autopsy series and ranged from 0.14% to 0.23% in a Western surgical series. [5] Two-dimensional echocardiography is likely to determine a higher incidence of SVA, although researchers note the incremental value of three-dimensional echocardiography. [6]

International data

SVA is more prevalent in Asian surgical series (0.46-3.5%) and correlates with more supracristal ventricular septal defects (about 60%). [7]

Race-, sex-, and age-related demographics

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

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

Unruptured SVA is usually asymptomatic and is often detected incidentally by routine two-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 that identified 86 patients who underwent SVA repair from 1956 to 2003 found the median age to be 45 years (range 5-80 years). [8]

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Prognosis

The prognosis for patients with sinus of Valsalva aneurysm (SVA) is poor with progressive aneurysmal dilatation or rupture unless early surgical repair is performed. [9]

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/or rupture.

In a series of 86 patients who underwent SVA repair, ruptures occurred in 34%. [8] 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. [8]

Another series reported comparable outcomes after surgical repair. [10] Of 65 patients aged 5-50 years undergoing surgical repair of SVA, the 30-day postoperative survival was 92%. Of the remaining patients, all survived to 1 year and two had complications: one with septic shock due to endocarditis and one with paraprosthetic regurgitation. [10]

In a series of 25 patients undergoing transcatheter closure of ruptured SVA, procedural success was 84%. [11]  One closure device embolized, two patients had residual leak, and three were referred to surgery.

Morbidity/mortality

The true natural history of SVA remains unclear. Clinical complications from SVA are often the initial presentation of this condition.

Complications of SVA include the following:

  • Potential risks of expansion, rupture, cardiac failure, and sudden death.
  • Myocardial infarction (from coronary arterial compression by adjacent unruptured SVA)
  • Complete heart block (from compression of conduction tissues by adjacent unruptured SVA)
  • Right ventricular outflow tract obstruction
  • Infective endocarditis
  • Tamponade if ruptured into the pericardium
  • Potential source of cerebrovascular emboli (rare)

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%). [12, 13] 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.

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