Pediatric Sinus of Valsalva Aneurysm Clinical Presentation
- Author: Edward J Bayne, MD; Chief Editor: Howard S Weber, MD, FSCAI more...
Children with sinus of Valsalva aneurysm are most often asymptomatic. Symptoms typically present in young adulthood (usually in patients < 30 y) either due to enlargement of the aortic root and compression of surrounding structures or due to manifestations of a ruptured aneurysm. Three clinical pictures may be associated with sinus of Valsalva aneurysm, as follows:
Sudden massive rupture may occur after strenuous exertion and may be signaled by acute chest or epigastric pain with dyspnea. Symptoms may be confused with those of acute myocardial infarction. 
Patients with a smaller insidious rupture may be asymptomatic, but small ruptures also may be associated with progressive symptoms of exertional dyspnea and/or chest discomfort from advancing heart failure. 
Patients with unruptured aneurysms may be asymptomatic. Angina may occur secondary to coronary compression resulting from an unruptured aneurysm. Syncope or dizziness may be caused by aneurysm compression of the conduction system, with associated heart block (Adams-Stokes syndrome).
Positive physical findings may be absent in a patient with an unruptured sinus of Valsalva aneurysm. Physical signs of a ruptured aneurysm vary, depending on the location of the shunt, and may mimic signs observed in a patient with a sizable coronary arteriovenous (AV) fistula. Physical signs may include the following :
A loud continuous murmur, accentuated in the diastole, occurs with aneurysm rupture into the right ventricle or right atrium. The systolic component of the continuous murmur is usually heard best higher in the chest, whereas the diastolic component may be best heard lower along the sternal border.
A parasternal thrill is heard from associated ventricular septal defect with large volume of runoff or, possibly, outflow obstruction.
Bounding pulses occur as a result of aortic runoff into lower-pressure chambers.
Shunt from the aortic root to the left ventricle may produce a diastolic murmur similar to that of aortic insufficiency.
Pulmonary rales may be present from progressive left heart failure.
With an unruptured aneurysm partially obstructing the right ventricular outflow tract, an ejection murmur may be heard at the left base radiating into the back.
Sinus of Valsalva aneurysm is presumed to be caused by a spontaneous genetic mutation. Although the defect is inherited, no distinct pattern of inheritance has been noted. Frank aneurysmal dilatation is rarely seen at birth.
Subpulmonic ventricular septal defect may be an important contributing factor in the progression of sinus of Valsalva aneurysms.
Tertiary syphilis is of historical significance as a cause for aortic aneurysms.
Traumatic injury to the aortic root (usually from direct chest compression) may rarely cause rupture of the aortic root.
A number of generalized disorders may be associated with dilatation and/or distortion of the aortic root, including Marfan syndrome, Ehlers-Danlos syndrome, Turner syndrome, Williams syndrome, aortic valve, bicuspid, and osteogenesis imperfecta.
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