History
Nonruptured sinus of Valsalva aneurysms (SVAs) are most often asymptomatic (25%) and found incidentally in routine two-dimensional echocardiography. However, patients can eventually develop nonspecific complaints, such as dyspnea, chest pain, palpitations, or loss of consciousness. Dyspnea is by far the most common presenting symptom.
When aneurysms are large enough, they can compress on neighboring structures, thereby causing arrhythmias, or they can obstruct the left or right ventricular outflow tracts, causing palpitations or syncope. Also, a thrombus can develop within the aneurism and occlude the coronary ostia and present as acute coronary syndrome, or it can dislodge and embolize to the brain and cause a stroke.
When the dilated sinus does rupture (precipitated by exertion, trauma, or cardiac catheterization), consequences depend on the location of the aneurysm. Patients may present dramatically with abrupt hemodynamic collapse, or the presentation may have a subtle onset. Most right coronary sinus aneurysms rupture into the right ventricular outflow tract, although an aorta-to-right atrium communication may also result. Left sinus aneurysms usually rupture into the left atrium. Rupture of noncoronary sinus aneurysms normally result in communication between the aorta and the right atrium or the right ventricle, thus creating a left-to-right shunt, which can lead to right ventricular overload and eventual right-sided heart failure. SVA rupture into the interventricular septum, with consequent left ventricular outflow tract obstruction, has also been reported. [2] Cardiac tamponade is a feared complication that may occur with any of the three types of aneurysms if the rupture involves the pericardial space. [4]
A ruptured SVA progresses in the following three stages, as described by Blackshear and colleagues [14] :
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Acute chest or right upper quadrant pain
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Subacute dyspnea on exertion or at rest (heart failure syndrome) with progressive or acute onset
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Progressive cough, dyspnea, edema, and oliguria
Atypically, SVA presents with infective endocarditis, which may originate at the edges of the aneurysm.
Physical Examination
Unruptured sinus of Valsalva aneurysms (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.
Signs of SVA include the following:
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A loud, superficial, "machine-type" continuous murmur is accentuated in diastole in as many as 40% of patients.
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A palpable thrill along the right or left lower parasternal border is occasionally noticeable.
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Bounding pulses are occasionally present.
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In one series, approximately 44% of the patients had associated aortic regurgitation. [8]
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Sinus of Valsalva Aneurysm. An axial slice from an electrocardiography (ECG)-gated computed tomographic angiogram (CTA) demonstrates an aneurysm of the right sinus of Valsalva (arrow). There is a filling defect within the aneurysm consistent with a thrombus.
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Sinus of Valsalva Aneurysm. A parasagittal slice from an electrocardiography (ECG)-gated computed tomographic angiogram (CTA) demonstrates a right sinus of Valsalva aneurysm (arrow). There is a filling defect within the aneurysm consistent with a thrombus.
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Sinus of Valsalva Aneurysm. A three-dimensional reconstruction of a computed tomographic (CT) aortogram is shown, demonstrating a right sinus of Valsalva aneurysm (arrow).
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Sinus of Valsalva Aneurysm. A mid-esophageal short-axis echocardiogram demonstrates a ruptured right sinus of Valsalva aneurysm. Color doppler demonstrates flow into the right ventricle.
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Sinus of Valsalva Aneurysm. A mid-esophageal long-axis echocardiogram demonstrates a ruptured right sinus of Valsalva aneurysm. Color doppler demonstrates flow into the right ventricle.