Pediatric Sinus of Valsalva Aneurysm

Updated: Feb 06, 2015
  • Author: Edward J Bayne, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Overview

Background

Congenital sinus of Valsalva aneurysm [1] was first described by Hope in 1839. [2] This first published account describes rupture of a sinus of Valsalva, which is the most feared complication. Soon afterwards, clinicians described other cases of unruptured aneurysms and applied anatomic descriptions. The condition may have been clinically described for the first time in 1883. [2]

The 3 sinuses of Valsalva are located in the most proximal portion of the aorta, just above the cusps of the aortic valve. The sinuses correspond to the individual cusps of the aortic valve. These structures contained within the pericardium are easily revealed using aortography and echocardiography as distinct but subtle out-pouchings of the aortic wall just above the valve. The sinuses end in the area of the sinotubular junction, and the tubular portion of the aorta begins here.

Aneurysm of a sinus of Valsalva is a rare congenital cardiac defect that can rupture, causing heart failure or other catastrophic cardiac events. If the aneurysm remains unruptured, it occasionally causes obstruction of cardiac flow resulting from compression of normal structures. Dissection of the aneurysm into the cardiac tissues may occur, causing obstruction or destruction of local structures.

Acquired aneurysmal dilatation of the sinuses of Valsalva may occur because of Marfan syndrome, syphilitic aortitis, or as a function of aging. These entities are not discussed in this article. See the image below.

Sinus of Valsalva aneurysm. Color-flow Doppler ult Sinus of Valsalva aneurysm. Color-flow Doppler ultrasonography is performed in the right ventricle through a supracristal ventricular septal defect with fingerlike prolapse of the right coronary sinus wall (arrow).
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Pathophysiology

Aneurysmal dilatation of the sinuses of Valsalva occurs when the aortic media is defective, allowing separation of the media from the aortic annulus fibrosus. [3] The defect is inherited, but frank aneurysmal dilatation is rarely seen at birth. Sinus of Valsalva aneurysm is associated with a ventricular septal defect in approximately 40% of patients.

Aneurysms typically develop as a discrete flaw in the aortic media within one of the sinuses of Valsalva. Aneurysms most often involve the right aortic sinus (67-85% of patients, often associated with a supracristal ventricular septal defect), followed by the noncoronary sinus, whereas an aneurysm of the left sinus is rare. [3] Under the strain of aortic pressure, the sinus gradually weakens and dilates, causing the formation of an aneurysm. Lack of supporting tissue (eg, ventricular septal defect) may contribute to instability and progressive distortion of the aortic sinus, often with associated aortic insufficiency. Deficiency of the aortic media where it attaches to the aortic annulus produces dilation of the aortic sinuses, usually over many years.

Distortion and prolapse of the sinus and aortic valve tissue can lead to progressive aortic valve insufficiency. Unruptured aneurysm may cause distortion and obstruction in the right ventricular outflow tract. Distortion and compression may also cause myocardial ischemia (by coronary artery compression) and, possibly, heart block (by compressing the conduction system). Rupture may occur into any chamber, although rupture most commonly occurs into the aortic right ventricular communication. Rupture into the right atrium is the second most common, in association with a noncoronary cusp aneurysm. Rupture may occur less commonly into the left-sided chambers, [4] the pulmonary artery, [5] and rarely extends into the pericardium.

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Epidemiology

Frequency

United States

Sinus of Valsalva aneurysm comprises approximately 0.1-3.5% of all congenital cardiac anomalies. Discovery in the pediatric age group is unusual.

International

Incidence in the Western Hemisphere (outside of the United States) is approximately the same as in the United States. An increase in prevalence is observed in Asians because of the higher incidence of supracristal (subpulmonic) ventricular septal defects.

Mortality/Morbidity

Morbidity and mortality are most often associated with acute severe aortic valve insufficiency resulting from aneurysm rupture. The mortality rate in patients with a sinus of Valsalva aneurysm in whom surgery is not performed is high within the first year after rupture. [3, 6, 7]

Causes of sudden death from sinus of Valsalva aneurysm most commonly involve rupture of the aneurysm with the acute onset of overwhelming congestive heart failure, cardiac tamponade, dysrhythmia, or coronary ischemia, depending on the location of the aneurysm and the subsequent flow disturbance. Size and location of the shunt are the major determinants of presentation and prognosis. Late death usually occurs within 1 year after rupture if the shunt is substantial; longer survival may occur if the shunt is small. [7, 8]

Major cardiac morbidity and late mortality in this condition also depend on the magnitude and location of shunts. In some instances, an unruptured aneurysm also causes major symptoms and hemodynamic alterations because of obstruction of valves or impingement upon cardiac structures (eg, coronary arteries, conduction system).

The surgical mortality rate is estimated to be 5% or less.

Race

Increased incidence in the Asian population has been described, which is the result of increased incidence of supracristal ventricular septal defect in Asian persons. These ventricular defects may contribute to instability of the aortic sinuses, particularly the right aortic sinus.

Sex

Male-to-female ratio is 4:1.

Age

Average age of patients with rupture of a sinus of Valsalva aneurysm is approximately 30 years, with a range of 11-67 years in one series. Problems in infancy and childhood are uncommon.

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