Sinus of Valsalva Aneurysm

Updated: Jul 15, 2014
  • Author: Anuradha Tunuguntla, MD, FACC; Chief Editor: Park W Willis IV, MD  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 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]

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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]

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Epidemiology

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, although researchers note the incremental value of 3-dimensional echocardiography. [5]

International

SVA is more prevalent in Asian surgical series (0.46-3.5%) and correlates with more supracristal ventricular septal defects (~60%). [6]

Mortality/Morbidity

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

Complications of sinus of Valsalva aneurysm include the following:

  • Sinus of Valsalva aneurysm (SVA), even if asymptomatic, has 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
  • Rarely, a potential source of cerebrovascular emboli

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). [7]

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