|Year : 2022 | Volume
| Issue : 3 | Page : 222-226
Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm: Role of Echocardiography
Munesh Tomar1, Roli Srivastava2
1 Department of Pediatrics, LLRM Medical College, Meerut, Uttar Pradesh, India
2 Department of Cardiology, Medanta Hospital Lucknow, Lucknow, Uttar Pradesh, India
|Date of Submission||08-May-2022|
|Date of Acceptance||10-May-2022|
|Date of Web Publication||12-Nov-2022|
Dr. Munesh Tomar
LLRM Medical College, Meerut, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Ruptured sinus of Valsalva (RSOV) aneurysm is a rare but well-known entity. It is a form of left-to-right shunt due to the rupture of aortic sinus into cardiac chambers, more commonly right sided. Hemodynamic effects are acute and severe. Surgical repair of RSOV aneurysm is an established treatment option and is being done with excellent results. Over the last two decades, there are case reports and case seriers describing percutaneous closure of RSOV aneurysm with promising results. Before planning percutaneous closure of RSOV aneurysm, it is imperative to know the detailed anatomy of the defect and its hemodynamic effects and to rule out associated defects requiring surgical intervention. Echocardiography plays a crucial role in defining the lesion in detail and helps in decision-making.
Keywords: Device closure, echocardiography, ruptured sinus of Valsalva
|How to cite this article:|
Tomar M, Srivastava R. Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm: Role of Echocardiography. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:222-6
|How to cite this URL:|
Tomar M, Srivastava R. Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm: Role of Echocardiography. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Feb 4];6:222-6. Available from: https://jiaecho.org/text.asp?2022/6/3/222/361060
| Introduction|| |
Aortic root is the segment of the aorta between the aortic annulus and sinotubular junction, and sinus of Valsalva (SOV) aneurysm is an abnormal dilatation of the aortic root. It is a rare cardiac anomaly. Rupture of SOV aneurysm is a potentially fatal complication and needs an urgent referral for closure by surgical repair or percutaneous device closure.
| Etiology|| |
Ruptured sinus of Valsalva (RSOV) aneurysm occurs more frequently in males than females (3:1) and in patients of Asian and Indian origin., It can be congenital or may occur as an acquired lesion. Patients with connective tissue disorders such as Marfan syndrome, Ehlers–Danlos syndrome, annuloaortic ectasia, and ankylosing spondylitis are at high risk for the development of RSOV aneurysm. Other causes for the development of RSOV aneurysm are aortic valve prolapse with ventricular septal defect (VSD), endocarditis, and trauma. In patients with connective tissue disorders and aortic valve prolapse, the weakened wall of SOV progressively dilates under systemic pressure, usually producing a windsock deformity. This dilated root may eventually rupture into a low-pressure cardiac chamber, right atrium (RA), such as right ventricle (RV), left atrium (LA), pulmonary artery (PA), interventricular septum (IVS), or rarely into the pericardium.[2-5] Any of the aortic sinuses may be involved, and there could be involvement of more than one sinus. The most commonly involved coronary sinus is right (73%), followed by noncoronary (20%), while left sinus (6%) is the least commonly involved. Enlargement of SOV aneurysm occurs in the direction of least resistance, so most commonly, it bulges into the RA or RV. Right sinus can rupture into RV (most common), RA, PA, left ventricle (LV), or into the pericardium while aneurysm/rupture of noncoronary sinus can extend to RA (most commonly), RV, LA, LV, or pericardium. Left coronary sinus aneurysm/rupture can extend into LA, RA, LV, RV, PA, or pericardium. IVS can be eroded by the aneurysm of right and nonfacing sinuses. Sakakibara and Konno classification has classified RSOV aneurysm into four types (I–IV) depending on the sinus involved and the site of rupture [Table 1] and [Figure 1].
|Table 1: Sakakibara and Konno classification of ruptured sinus of Valsalva|
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|Figure 1: Line diagram depicting Sakakibara and Konno classification of RSOV aneurysm. LCC: Left coronary cusp, NCC: Noncoronary cusp, RA: Right atrium, RCC: Right coronary cusp, RV: Right ventricle, RVOT: RV outflow tract|
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Aneurysm of SOV is a disease of aortic root and so aortic regurgitation (AR) is a frequent occurrence. The reason cited is loss of support of the aortic annulus and sinus. In addition, ruptured aneurysm leads to aortic runoff, which produces Bernoulli's effect and the cusp is pulled away from its line of apposition, leading to the development of AR.
Associated anomalies are common, most common being VSD, reported in about 50% of patients. Other associated anomalies are atrial septal defect, bicuspid aortic valve, coarctation of the aorta, pulmonary stenosis, tetralogy of Fallot, and left superior vena cava. There is a higher association of AR when SOV aneurysm is associated with VSD (43.3%) in comparison to intact ventricular septum (25.9%).
| Echocardiography Imaging|| |
Transthoracic echocardiography is the initial diagnostic tool to diagnose the anomaly, cusp involved, site of rupture, its hemodynamic effects, including shunt quantitation, chamber(s) volume overload, pulmonary arterial pressure, AR, and associated structural defects. A recess at the apex of the involved aneurysmal sinus may be seen in two-third of the patients, which has been classically described as “windsock deformity.” In grown-up patients with poor acoustic windows, transesophageal echocardiography (TEE) is needed for detailed definition.
Echocardiographic evaluation of RSOV aneurysm includes:
- Sinus involved and details of aneurysm including “windsock deformity”
- Site of rupture and cavity involved
- Hemodynamic effects (direction of shunt and volume overload, chamber dilatation, PA pressure, ventricular function, and pericardial effusion)
- Associated defects if any.
The key planes for the diagnosis include:
- Parasternal long-axis view
- Parasternal short-axis view of the aortic root
- Four-chamber views; apical-four-chamber and subcostal (coronal)
- Modified, nonstandard views showing the aortic root
- Suprasternal view: Need to rule out coarctation of the aorta. Color flow mapping across arch also showed diastolic flow reversal in the aorta due to aortic runoff [Video 1].
[Additional file 1]
Video 1: Suprasternal long-axis view with color flow mapping showing pandiastolic flow reversal in descending thoracic aorta in a patient with ruptured sinus of Valsalva aneurysm.
Parasternal long-axis view
In the parasternal long-axis (PLAX) view, right SOV occupies the most anterior position while noncoronary sinus is located posteriorly. Any dilatation and rupture of right or noncoronary sinus is seen in this view [Figure 2]a and [Figure 2]b. Posterior tilt in the PLAX view shows RV inflow while anterior tilt will show RV outflow. RSOV aneurysm from right sinus to RV inflow or outflow can be profiled from PLAX view with anterior or posterior tilt, respectively. Color flow mapping demonstrates the flow from ruptured aortic sinus to the cavity into which it opens [Figure 2]b. Rupture into RA, LA, RV, or PA will result in a continuous flow from the aortic sinus to the receiving cavity [Figure 2]c and [Figure 2]d], while with rupture into LV, there will be only diastolic flow. The presence of AR and its severity should also be noted using color flow mapping [Video 2].
|Figure 2: Echocardiography of a 26-year-old male with RSOV aneurysm from RCC to RV inflow: (a) Parasternal long-axis view with slight posterior tilt showing RSOV aneurysm from RCC to RV. (b) Same view with color flow mapping showing left to right continuous flow across RSOV aneurysm from RCC to RV. (c) M-mode interrogation with color flow mapping showing continuous flow across the RSOV aneurysm. (d) Continuous wave Doppler interrogation of RSOV aneurysm showing continuous flow across the communication. RCC: Right coronary cusp, RSOV: Ruptured sinus of Valsalva, RV: Right ventricle|
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[Additional file 2]
Video 2: Transthoracic echocardiography, parasternal longaxis view with color flow mapping in a patient with ruptured sinus of Valsalva aneurysm showing no aortic regurgitation.
M-mode interrogation of the flow across RSOV aneurysm is the best way to demonstrate the direction of shunt in relation to the cardiac cycle. With RSOV aneurysm, a continuous flow from the aorta to receiving chamber can be demonstrated [Figure 2]c.
Parasternal short-axis view
In the parasternal short-axis (PSAX) view, aorta at the level of sinus is the central structure surrounded by RA, RV with outflow, main PA, and LA. This view defines the anatomy with the aortic sinus protruding into the surrounding structure [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. Anatomical details of the aneurysm and rupture, including “windsock deformity,” size of communication at aortic and communicating site, and the length of communication should also be defined [Figure 3]c.
|Figure 3: (a) In parasternal short-axis view at the level of great vessels, right coronary cusp occupies the most anterior position, left coronary sinus left and posterior position while noncoronary sinus occupies right and posterior position. The grayscale image shows the defect- RSOV aneurysm from RCC to RV. (b) Parasternal short-axis view with color flow mapping showing left to right continuous flow (mosaic pattern) across RSOV aneurysm (arrow) from RCC to RV. (c) Parasternal short-axis view (modified) showing aneurysm (arrow) protruding into right atrium from RCC (RSOV aneurysm from RCC to RA). Measurement is being taken at the aortic end of RSOV aneurysm as shown in the picture for device closure of RSOV aneurysm. (d) Parasternal short-axis view with color flow mapping showing continuous flow (arrow) across RSOV aneurysm from RCC to RA. LA: Left atrium, LCC: Left coronary cusp, NCC: Noncoronary cusp, RA: Right atrium, RCC: Right coronary cusp, RSOV: Ruptured sinus of Valsalva, RV: Right ventricle|
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Apical four-chamber view
Anterior sweep from the apical four-chamber view (5-C view) shows the aorta arising from LV with right and left coronary sinuses on respective sides. Any dilatation with or without rupture of these sinuses will be seen in the 5-C view [Figure 4]a. The color jet of RSOV aneurysm of the right aortic sinus to RA can be mistaken with a tricuspid regurgitation jet [Figure 4]b. Both the jets will appear blue, but the TR jet will be systolic, while the RSOV aneurysm jet will give a continuous signal.
|Figure 4: Transthoracic echocardiography of a 32-year-old male with ruptured sinus of Valsalva from right coronary sinus to right atrium: (a) Apical four-chamber view showing windsock aneurysm from the right coronary cusp, protruding into the right atrium (arrow points to site of rupture). (b) Same view with color flow mapping showing turbulent flow entering the right atrium (arrow). LA: Left atrium, LV: Left ventricle, RA: Right atrium, RV: Right ventricle|
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In grown-up patients with RSOV aneurysm, as transthoracic imaging is not adequate, TEE may be required for the detailed definition of the lesion and to look for associated defects such as VSD and for assessment of AR. Basal short-axis view (mid-esophageal, at 40° angle) defines the aortic sinus involved, presence of aneurysm, and rupture of aortic sinus to the neighboring chamber such as RA, RV, or LA [Figure 5]a and [Figure 5]b, [Figure 6] [Video 3] and [Video 4]a, [Video 4]B, [Video 4]c. Mid-esophageal (0°) apical four-chamber view, modified with anteflexion of transducer, and long-axis view of the left ventricular outflow tract (mid-esophageal at 120°) can define RSOV aneurysm into RA, to ventricular septum, or into the LV.
|Figure 5: (a) Transesophageal echocardiography of an adult patient with RSOV aneurysm. Short-axis view at mid-esophageal level showing aneurysm (windsock) from NCC protruding into the right atrium (arrow). (b) The same view with color flow mapping showing turbulent flow across RSOV aneurysm (NCC to RA communication). Ao: Aorta, LA: Left atrium, NCC: Non-coronary cusp, RA: Right atrium, RSOV: Ruptured sinus of Valsalva, RV: Right ventricle|
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|Figure 6: Transesophageal echocardiography of an adult patient with RSOV aneurysm. Short-axis view at mid-esophageal level with color flow mapping showing RSOV aneurysm of the right coronary cusp into the RV inflow (arrow). Ao: Aorta, LA: Left atrium, RA: Right atrium, RSOV: Ruptured sinus of Valsalva, RV: Right ventricle|
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[Additional file 3]
Video 3: Transesophageal echocardiography, short-axis view at mid-esophageal level with color flow mapping showing left to right continuous flow across ruptured sinus of Valsalva aneurysm from right coronary sinus to right ventricle.
Video 4: [Additional file 4] (a) Transesophageal echocardiography of an adult patient with a ruptured sinus of Valsalva aneurysm. The short-axis view at mid-esophageal level is shown depicting aneurysm (windsock) from the non-coronary cusp protruding into the right atrium. [Additional file 5](b) Transesophageal echocardiography with modified long-axis view at mid-esophageal level from the same patient showing aneurysm (windsock) from non-coronary cusp protruding into the right atrium. [Additional file 6](c) Transesophageal echocardiography, short-axis view at the mid-esophageal level with color flow mapping showing left to right continuous flow across the ruptured sinus of Valsalva aneurysm from the noncoronary cusp to the right atrium.
| Role of Echocardiography Imaging in Transcatheter Closure of RSOV|| |
Cullen et al. published the first report of device closure of RSOV aneurysm in 1994 using the Rashkind umbrella. Since then, there have seen case reports and case series of percutaneous closure of RSOV aneurysm using Amplatzer devices.,,,,, Echocardiography is essential in planning the intervention and as a guidance during the procedure. Some operators do the procedure under fluoroscopy with the help of transthoracic echocardiography, while majority use TEE as a guide during device closure of RSOV aneurysm. Detailed TEE evaluation should be done prior to proceeding with the hemodynamic study and angiography. This includes assessment of the maximum diameter of the aortic end of the RSOV aneurysm, the minimum diameter and the length of the windsock, the distance of the aortic end of the RSOV aneurysm from the coronary ostium, and assessment of AR. The presence of VSD or other structural defects needing surgery should also be ruled out. Majority of operators choose device size as per echocardiography/angiography measurements and not by balloon sizing of the defect.,,
TEE also helps in limiting angiography and avoiding contrast load in critically-ill patients. Three-dimensional TEE provides a more accurate measurement of the defect size and position and should be used if available.
Amplatzer duct occluder (ADO) is the preferred device for percutaneous device closure of RSOV aneurysm, though there are reports of the use of atrial septal occluder, muscular VSD device, vascular plugs, and coils. Majority use an ADO 2–4 mm larger than the aortic end of the RSOV aneurysm, as measured on TEE/angiography. Sometimes, the operator needs to upsize the device when the margins of the defect are flimsy (”windsock like”) [Figure 3]c, [Figure 4]a and [Figure 5]a. Attempts should be made to close the defect as far as possible near the aortic end to avoid leaving any uncovered residual aneurysm with a potential to rupture at another site. Critical assessment of AR should be done, during and after the device deployment because the presence of AR at discharge is the single most important determinant of long-term outcomes following surgical repair of RSOV aneurysm. Encroachment of the device on the coronary arteries has never been reported because the site of RSOV aneurysm is at the bottom of coronary cusp and is away from coronary artery origin but should also be checked carefully.
At the end of the procedure and during follow-up, the following should be checked on echocardiography:
- Device position, stability of device [Video 5]
- Any residual shunt
- AR and comparison to previous AR
- Ventricular function
- PA pressure
- Pericardial effusion.
[Additional file 7]
Video 5: Parasternal short-axis view following device closure of a ruptured sinus of Valsalva aneurysm from the right coronary cusp to right atrium. The closure device is seen in situ, with no residual flow and no tricuspid or aortic regurgitation.
| Conclusion|| |
RSOV aneurysm is a relatively rare cardiac anomaly with presentation varying from asymptomatic findings to acute heart failure, which can rapidly worsen to cause death. Although surgery is the gold standard treatment, percutaneous transcatheter closure has now become equally efficacious with fewer complications. Echocardiography plays a crucial role in the preprocedure evaluation of the patients to define the anatomy, hemodynamic effects, and the degree of AR and to rule out associated lesion(s). Furthermore, echocardiography, especially TEE, is crucial during percutaneous closure of the defect and on follow-up.
Financial support and sponsorship
Conflicts of interest
Munesh Tomar is an editorial board member of the Journal of The Indian Academy of Echocardiography and Cardiovascular Imaging. The article was subject to the journal's standard procedures, with peer review handled independently of this editor and their research groups.
There are no other conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]