(Radiology. 2001;219:82-85.)
© RSNA, 2001
Case 33: Sinus of Valsalva Aneurysm1
Charles S. White, MD and
Gary D. Plotnick, MD
1 From the Department of Diagnostic Radiology (C.S.W.) and the Division of Cardiology, Department of Internal Medicine (G.D.P.), University of Maryland Medical Center, 22 S Greene St, Baltimore, MD 21201. Received May 6, 1999; revision requested July 16; revision received August 16; accepted August 26. Address correspondence to C.S.W. (e-mail: cwhite@umm.edu).
Index terms: Aneurysm, aortic, 941.73 • Aneurysm, CT, 941.12916 • Aneurysm, MR, 941.12941 • Aorta, injuries, 941.41 • Diagnosis Please • Sinus of Valsalva
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HISTORY
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A 44-year-old woman presented to our trauma center after being thrown from a horse. Radiographs of the lumbar spine revealed a burst fracture of the first lumbar vertebral body. The patient underwent spinal stabilization with placement of spinal rods between T10 and L2. A chest radiograph was obtained following surgery (Fig 1). During the postoperative evaluation, a sinus tachycardia was noted, and an electrocardiogram showed depressions in the S-T segment. Transthoracic echocardiography was performed at that time (Fig 2), and cardiac magnetic resonance (MR) imaging was performed several weeks later (Fig 3).
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Figure 1. Anteroposterior chest radiograph obtained after surgery for lumbar spinal stabilization. Widespread opacity reflects postoperative pulmonary edema and bilateral posteriorly layering pleural effusions. Fixation rods are evident in the lumbar spine. An abnormal convexity (arrow) is present along the upper left border of the heart.
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Figure 2. Transthoracic echocardiogram obtained in the parasagittal long-axis view after an abnormal electrocardiogram (not shown) shows a rounded nearly echo-free lesion (V) posterior to the aortic root (A) and anterior to the left atrium (L).
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Figure 3a. Electrocardiographically gated spin-echo MR images (508/30 [repetition time msec/ echo time msec]) obtained in the (a) transverse and (b, c) coronal planes show a rounded 6-cm structure (large solid arrows) extending to the left of the ascending aorta near the aortic root. In a, the lesion contains a small amount of signal intensity. In b and c, more signal intensity is present that suggests swirling flow. In both planes, a connection (small solid arrows) is visible between the lesion and the aortic root. A moderate pericardial effusion (open arrows in b and c) is of medium signal intensity. a = ascending aorta.
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Figure 3b. Electrocardiographically gated spin-echo MR images (508/30 [repetition time msec/ echo time msec]) obtained in the (a) transverse and (b, c) coronal planes show a rounded 6-cm structure (large solid arrows) extending to the left of the ascending aorta near the aortic root. In a, the lesion contains a small amount of signal intensity. In b and c, more signal intensity is present that suggests swirling flow. In both planes, a connection (small solid arrows) is visible between the lesion and the aortic root. A moderate pericardial effusion (open arrows in b and c) is of medium signal intensity. a = ascending aorta.
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Figure 3c. Electrocardiographically gated spin-echo MR images (508/30 [repetition time msec/ echo time msec]) obtained in the (a) transverse and (b, c) coronal planes show a rounded 6-cm structure (large solid arrows) extending to the left of the ascending aorta near the aortic root. In a, the lesion contains a small amount of signal intensity. In b and c, more signal intensity is present that suggests swirling flow. In both planes, a connection (small solid arrows) is visible between the lesion and the aortic root. A moderate pericardial effusion (open arrows in b and c) is of medium signal intensity. a = ascending aorta.
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IMAGING FINDINGS
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The frontal radiograph obtained with the patient supine showed widespread hazy opacity consistent with postoperative pulmonary edema and bilateral posteriorly layering pleural effusions. Lumbar spinal-fixation rods were consistent with the recent stabilization surgery. An abnormal convexity was present along the upper left border of the heart (Fig 1).
A transthoracic echocardiogram obtained in a parasagittal long-axis view showed a rounded nearly echo-free structure posterior to the aorta and anterior to the left atrium (Fig 2).
T1-weighted MR images obtained in transverse and coronal planes showed a rounded 6-cm structure extending to the left of the ascending aorta, superior to the left ventricle, and inferior to the main pulmonary artery. On the transverse image, the epicenter of the lesion was adjacent to the upper part of the aortic root. The lesion contained little or no signal intensity. On the coronal images, some signal intensity was present that suggested the presence of swirling flow. In both planes, a small connection was visible between the lesion and the aortic root (Fig 3). Cine sequences (not shown) helped confirm flow within the lesion.
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DISCUSSION
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The imaging findings in this case suggest an aneurysm related to the heart or proximal aorta. The major types of aneurysms in this region are left ventricular aneurysms, septic pseudoaneurysms, ascending aortic aneurysms, left coronary artery aneurysms, and sinus of Valsalva aneurysms. Most true left ventricular aneurysms originate from the apex or the lateral or inferior wall. A true aneurysm of the left ventricle is often caused by ischemic heart disease and typically has a wide mouth; its connection with the ventricle is evident (1). A false aneurysm may be caused by ischemia or trauma and has a narrow mouth. A connection between the aneurysm and the ventricular cavity may not always be visible. However, most false aneurysms are located posteroinferiorly, and a left ventricular aneurysm of either type is unusual at the base of the heart (1).
A septic pseudoaneurysm is a lesion caused by extension of infective endocarditis into the myocardium. Most septic pseudoaneurysms are small and are located in the myocardium adjacent to the affected cardiac valve (2). The main complication is interference with the conducting system of the heart, which causes heart block. The aneurysm in the test case does not appear to be confined to the myocardium (2).
An ascending aortic aneurysm could potentially produce an appearance similar to that found in this case. Ascending aortic aneurysms may be part of the complex of annuloaortic ectasia, in which the root of the aorta dilates, the sinotubular junction is effaced, and aortic regurgitation results. Marfan syndrome and other connective tissue diseases are frequent causes (3). In as many as one-third of cases, annuloaortic ectasia is idiopathic. Ascending aortic aneurysms may also be caused by poststenotic dilatation in aortic stenosis, and less commonly by syphilis, other infections, trauma, and atherosclerosis. Most ascending aortic aneurysms are fusiform, unlike the eccentric morphology of the aneurysm in the present case (3).
A proximal left main coronary artery aneurysm might also lead to an appearance like that shown in this case. However, several factors decrease the likelihood of this diagnosis. First, the epicenter of the lesion is somewhat cephalic to the expected location of a left coronary artery as demonstrated by its relationship to the ascending aorta on the transverse MR image. In addition, a left main coronary artery aneurysm of this size is a rare lesion and would most likely contain thrombus (4). The pattern of the minimal signal intensity found on the transverse MR image suggests swirling flow, not thrombus. Finally, the MR images show a direct connection between the lesion and the aortic root. If this lesion were a left coronary artery aneurysm, an intervening structure representing a normal or compressed sinus of Valsalva might be expected. Thus, the location and characteristics of the aneurysm near the aortic root as shown on both the echocardiogram and MR images is most consistent with an aneurysm of the left sinus of Valsalva.
The sinus of Valsalva aneurysm (aortic sinus aneurysm) is most often congenital and accounts for 0.1%–3.5% of congenital heart disease (5). A 4:1 male predominance is reported (5). The incidence appears to be higher in countries in the Far East (5). The aneurysm is thought to arise from incomplete fusion of the distal bulbar septum that divides the aorta and pulmonary artery and attaches to the anulus fibrosus of the aortic valve (5). It is postulated that after exposure to long-standing high pressures, this area, which forms part of the sinuses of Valsalva, weakens and becomes aneurysmal. Most series have shown a strikingly higher prevalence of right sinus of Valsalva involvement (75%–90%) (5). The noncoronary sinus is affected in 10%–25% of patients. The left sinus, which is rarely affected by congenital lesions, does not arise from the bulbar septum (5).
Unruptured sinus of Valsalva aneurysms may remain clinically silent for many years. Symptoms occasionally are caused by mechanical obstruction. Compression of the conducting system is an unusual cause of heart block; extrinsic narrowing of the coronary artery by an aneurysm may lead to ischemia in rare cases (6). Intracardiac rupture occurs most commonly in the 3rd or 4th decade of life and causes a dramatic presentation with chest pain and heart failure. The rupture typically results in communication of the aortic sinus with the right side of the heart, especially the right ventricle. If the aneurysm affects the noncoronary cusp, a fistula with the right atrium is more frequent (5).
Although congenital sinus of Valsalva aneurysms are often isolated occurrences, an association with ventricular septal defect, aortic regurgitation, or both is reported. Aortic regurgitation, such as occurred in our case, is presumably due to deformation of the affected leaflet by the aneurysm. Secondary causes of sinus of Valsalva aneurysms are less common than congenital lesions and more frequently affect the left sinus. Secondary causes include bacterial endocarditis, trauma, syphilis, tuberculosis, and Behçet disease (5,7).
The sinus of Valsalva of aneurysm is often not apparent at chest radiography (8). The lesion occasionally manifests as an abnormal convexity of the upper right or, less commonly, the upper left border of the heart. More often, radiographic abnormalities are due to the hemodynamic consequences of the aneurysm, such as aortic insufficiency or rupture. In these cases, chest radiography may demonstrate an enlarged heart or pulmonary venous congestion. Angiography shows a smooth or slightly lobulated contour of the aneurysm. The site of rupture is often depicted as contrast material that flows into the receiving chamber (8).
More recently, echocardiography, computed tomography (CT), and MR imaging have shown value in the diagnosis of sinus of Valsalva aneurysm (9–12). Echocardiography has re-placed angiography as the principal technique to diagnose the aneurysm. Transthoracic echocardiography with Doppler ultrasonography (US) shows the wall of the aneurysm and turbulent flow within the unruptured aneurysm. Rupture is diagnosed if echo discontinuity occurs at the edge of the aneurysm. Color Doppler flow US imaging reveals flow between the aneurysm and receiving chamber. The most commonly associated abnormalities, aortic regurgitation and ventricular septal lesion, are well depicted (9). Transesophageal echocardiography offers the potential for more accurate characterization of the aneurysm (13).
In patients who have suboptimal evaluation at echocardiography, CT and MR imaging are useful to delineate sinus of Valsalva aneurysm (Fig 4). Both show the structural appearance of the aneurysm, but MR imaging offers direct multiplanar depiction without the need for injection of contrast material (10–12). MR imaging shows turbulence within the aneurysm, as in the test case, and gradient-echo images may document aortic regurgitation. Cine phase-contrast MR imaging delineates the site of fistula formation and can be used to determine the extent of shunting caused by rupture of the aneurysm (12).
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Figure 4. Abnormal right border of the heart, as identified on a chest radiograph (not shown), in a 56-year-old man with chest pain. Enhanced transverse chest CT scan reveals a rounded structure (v) that is closely related to the aortic root (a) and projects rightward and anteriorly. An echocardiogram (not shown) was used to confirm a right sinus of Valsalva aneurysm.
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The aneurysm in the test case occurred in the left sinus of Valsalva, which is an unusual location for a congenital lesion. MR imaging was performed prior to surgery to clarify the findings at echocardiography, which was technically difficult. At surgery, it was thought that the injury was of recent origin and likely related to the patient’s traumatic injury.
Our congratulations to the 41 individuals who submitted the most likely diagnosis (sinus of Valsalva aneurysm) for Diagnosis Please, Case 33. The names and locations of the individuals, as submitted, are as follows:
- Gholamali Afshang, MD, Tinley Park, Ill
- Edward L. Baker, MD, São Paulo, Brazil
- Fábio Bayona, Santa Marcelina, São Paulo, Brazil
- A. Joseph Borelli, Jr, MD, Hilton Head, SC
- Marianne Braunschweig, Biel, Switzerland
- Eric L. Bressler, MD, Minnetonka, Minn
- Douglas C. Brown, MD, Virginia Beach, Va
- Júlio Dabdab, Santa Marcelina, São Paulo, Brazil
- Marc G. de Baets, MD, Lugano, Switzerland
- Marco Antônio de Melo Medeiros, Santa Marcelina, São Paulo, Brazil
- Eugênio de Sá Coutinho Neto, Santa Marcelina, São Paulo, Brazil
- Kemal Demir, MD, Istanbul, Turkey
- Mario Finazzo, Palermo, Italy
- Richard Friedland, Poughkeepsie, NY
- Athanassios D. Gouliamos, Athens, Greece
- Flavius Guglielmo, Basking Ridge, NJ
- Alberto Iaia, MD, Wilmington, Del
- Masako Kataoka, Sakyo, Kyoto, Japan
- Eduardo Lassalle, MD, Quilmes, Argentina
- Edward A. Lebowitz, MD, San Jose, Calif
- Joseph H. Lock, Jr, MD, Mankato, Minn
- Dieter Lungenschmid, MD, Innsbruck, Austria
- Kathlyn Marsot-Dupuch, Paris, France
- Edward Menges, MD, Aptos, Calif
- Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina
- Júlio Neves Junior, Santa Marcelina, São Paulo, Brazil
- Vung D. Nguyen, MD, San Antonio, Tex
- Mizuki Nishino, MD, Kyoto, Japan
- Jose Novoa, MD, Salem, Ore
- Sanford M. Ornstein, MD, Phoenix, Ariz
- David M. Panicek, MD, New York, NY
- Shawn P. Quillin, MD, Charlotte, NC
- Maria Margarete Ruthes, Santa Marcelina, São Paulo, Brazil
- Luís José Sales Campos, Santa Marcelina, São Paulo, Brazil
- Matt Shapiro, MD, Lowell, Mass
- Paolo Siotto, MD, Cagliari, Italy
- Cícero J. Torres A. Silva, Santa Marcelina, São Paulo, Brazil
- Danielle van Westen, Malmö, Sweden
- J. Edson F. Viana, Santa Marcelina, São Paulo, Brazil
- Joe Yut, Olathe, Kan
- Walter Zoletti, Jr, Santa Marcelina, São Paulo, Brazil
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FOOTNOTES
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Part 1 of this case appears 4 months previously and may contain larger images.
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Gharzuddine WS, Sawaya JI, Kazma HK, Obeid MY. Traumatic pseudoaneurysm of the left sinus of Valsalva: a case report. J Am Soc Echocardiogr 1997; 10:377-380.[Medline]
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Xu Q, Peng Z, Rahko PS. Doppler echocardiographic characteristics of sinus of Valsalva aneurysms. Am Heart J 1995; 130:1265-1269.[Medline]
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Ogawa T, Iwama Y, Hashimoto H, Ito T, Satake T. Noninvasive methods in the diagnosis of ruptured aneurysm of Valsalva: usefulness of magnetic resonance imaging and Doppler echocardiography. Chest 1991; 100:579-581.[Abstract/Free Full Text]
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Becker C, von Smekal A, Knez A, Wintersperger BJ, Behr J, Reiser M. Demonstration by spiral CT of an aneurysm of the left sinus of Valsalva. Rofo Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr 1997; 167:427-429[German].[Medline]
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Ho VB, Kinney JB, Sahn DJ. Ruptured sinus of Valsalva aneurysm: cine phase-contrast MR characterization. J Comput Assist Tomogr 1995; 19:652-656.[Medline]
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Wang KY, St John Sutton M, Ho HY, Ting CT. Congenital sinus of Valsalva aneurysm: a multiplane transesophageal echocardiographic experience. J Am Soc Echocardiogr 1997; 10:956-963.[Medline]
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TOP
HISTORY
IMAGING FINDINGS
