A Rare Case of Giant Unruptured Sinus of Valsalva Aneurysm

Biswaranjan Mishra; Harsha Pattnaik

doi:10.4103/jiae.jiae_56_21

INTERESTING CASE REPORT

Year : 2022 | Volume : 6 | Issue : 2 | Page : 146-150

A Rare Case of Giant Unruptured Sinus of Valsalva Aneurysm

Biswaranjan Mishra¹, Harsha Pattnaik²
¹ Senior Consultant Cardiologist, Max Diagnostic, Cuttack, Odisha, India
² MBBS (Student), Lady Hardinge Medical College, New Delhi, India

Date of Submission	15-Sep-2021
Date of Acceptance	21-Nov-2021
Date of Web Publication	23-Aug-2022

Correspondence Address:
Dr. Biswaranjan Mishra
201, Chandralok Apartment, Professorpada, Cuttack - 753 003, Odisha
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_56_21

Abstract

Sinus of Valsalva aneurysm is a rare congenital disorder that usually presents with rupture of the aneurysm into adjacent cardiac structures. It usually arises from the right sinus of Valsalva which ruptures into the right ventricle or right atrium (RA). Aneurysms may also arise from the noncoronary cusp that rupture into RA. Rarely, an unruptured aneurysm may compress or dissect into the adjacent structures and produce obstruction to either left or right ventricular outflow or produce conduction blocks. It may also intrude upon valves affecting valvular function. Here is a case of an unruptured giant aneurysm arising from the right sinus of Valsalva burrowing into the interventricular septum (IVS) causing conduction defect in the form of left bundle branch block with first degree atrioventricular block. Through IVS, it protruded into the left ventricular (LV) outflow tract (LVOT) giving rise to a cystic appearance causing mild LVOT obstruction and mild-to-moderate aortic regurgitation. The aneurysm also affected mitral valve function causing severe mitral regurgitation (MR) including characteristic diastolic MR due to prolonged PR interval. There was no regional wall motion abnormality indicating the absence of coronary arterial obstruction by the aneurysm. Left atrial and LV dilatation was present along with LV systolic dysfunction. Pulmonary arterial systolic pressure was approximately 48 mmHg as calculated from tricuspid regurgitation (TR) gradient. Like MR, TR also showed diastolic TR.

Keywords: Diastolic mitral regurgitation, sinus of Valsalva aneurysm, unruptured sinus of Valsalva aneurysm

How to cite this article:
Mishra B, Pattnaik H. A Rare Case of Giant Unruptured Sinus of Valsalva Aneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:146-50

How to cite this URL:
Mishra B, Pattnaik H. A Rare Case of Giant Unruptured Sinus of Valsalva Aneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Sep 27];6:146-50. Available from: https://jiaecho.org/text.asp?2022/6/2/146/354322

Introduction

Sinus of Valsalva aneurysm (SVA) is a rare disorder due to congenital weakness of the aortic media causing deficiency of elastic fiber in the aortic sinuses. The incidence of SVA is reported to be 0.1%–3.5% of all congenital cardiac anomalies.^[1] Rarely, it may be acquired secondary to infective endocarditis or trauma. SVAs commonly accompany a ventricular septal defect and have sex preponderance for males in a ratio of 4:1. SVA appears like a windsock with a relatively narrow base and an elongated body. It commonly arises from the right sinus of Valsalva in about 80%–85% of cases. The rest majority of SVAs arises from the noncoronary sinus which accounts for 5%–15% of cases. Aneurysm of the left sinus is uncommon. On rare occasion, multiple sinuses may be involved.^[2]

SVAs typically present with rupture into a cardiac chamber, commonly into a right-sided chamber, in young adults. On rare occasion, rupture occurs into pericardial or pleural cavity, pulmonary trunk, left atrium (LA) or into superior vena cava.^[3] Unruptured aneurysms may present with obstruction to either left or right ventricular outflow tracts.^[4] Rare presentation may be due to compression of a coronary artery causing myocardial ischemia.^[5] Dissection into ventricular septum causing conduction disturbances,^[6] intrusion upon valve apparatus causing valvular regurgitation or dissection into atrial septum or ventricular free wall may also occur.^[7] Wide availability of imaging, particularly echocardiography has now made possible detection of unruptured asymptomatic aneurysms more frequently. Transthoracic echocardiography (TTE) is the imaging mode of choice for diagnosing SVA. However, other imaging modalities such as transesophagial echocardiography (TEE), three-dimensional (3D) echocrdiography, computed tomography (CT), and cardiac magnetic resonance imaging are often required for defining the exact anatomy of the SVA, particularly before surgery.

Case Report

A 43-year-old male presented with progressive dyspnea for approximately a year. He was a nondiabetic, nonhypertensive with no history of smoking. Physical examination revealed pulse rate of 90 beats/min which was regular. Blood pressure was 140/60 mm of Hg, jugular venous pressure was not raised. Apex was displaced laterally and inferiorly; auscultation revealed the presence of left ventricular (LV) third heart sound, a pansystolic murmur grade III/VI at apex and an early-diastolic murmur at left sternal border. The patient was already on torsemide 10 mg daily.

Electrocardiogram showed sinus rhythm, complete left bundle branch block (LBBB) and first degree atrio-ventricular block with PR interval of 320 ms [Figure 1]. Chest X-ray showed cardiomegaly, LV contour, and signs of pulmonary venous hypertension [Figure 2].

Figure 1: Electrocardiogram showing first degree atrioventricular block with PR interval of 320 ms and complete left bundle branch block. Arrows indicate P-waves

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Figure 2: Chest X-ray showing cardiomegaly, left ventricular contour, and signs of pulmonary venous hypertension

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Two-dimensional TTE in the parasternal long-axis view showed a cystic structure in the LV outflow tract (LVOT); an echo-free space was also noted in the proximal interventricular septum (IVS) [Figure 3]a. The cystic structure was collapsing in systole and expanding in diastole [Video 1] and [Figure 3]b. Similar findings were also noted in the apical five-chamber view [Video 2] and [Figure 3]c. Slight rotation of the probe showed that the echo-free space in the IVS was in communication with the aortic root [Video 3] indicating a right SVA burrowing into the IVS. Further tilting of the probe revealed that the cystic space in the LVOT, echo-free space in the proximal IVS and the aortic root in the region of right sinus of Valsalva were in continuity implying a giant SVA which was burrowing into IVS and also protruding into the LVOT [Video 4] and [Figure 3]d. Color Doppler interrogation showed flow from the aortic root to the aneurysm [Video 5]. Continuous wave (CW) Doppler showed a to-and-fro flow into the aneurysm from the aortic root differentiating it from aortico-LV tunnel and sub-aortic aneurysm [Figure 4]a. Color Doppler interrogation in the apical five-chamber view showed moderate aortic regurgitation (AR) [Video 6]. CW Doppler interrogation showed LVOT peak velocity 2.57 m/s, peak gradient 26.43 mmHg, and AR pressure half time of 597 ms [Figure 4]b. CW Doppler interrogation of the right ventricular outflow tract (RVOT) did not show any obstruction, but there was a high-velocity pulmonary regurgitation jet indicating the presence of pulmonary hypertension [Figure 4]c. Short-axis view at the base showed the giant aneurysm measuring 4.5 cm × 2.0 cm occupying the RVOT and also protruding into the right atrium (RA) [Figure 5] and [Video 7]. Color Doppler did not reveal any evidence of rupture [Video 8]. Examination in the apical four-chamber view showed severe mitral regurgitation (MR) with an eccentric jet having vena contracta of 8 mm [Video 9] and [Figure 6]a. The mechanism of MR was assumed to be the infringement on the valve apparatus as the aneurysm abutted the anterior mitral leaflet and the attached chordae during early systole before it collapsed in the later part of systole [Figure 6]b. The posterior direction of the MR jet, absence of leaflet thickening, and the lack of any other mechanism of MR also corroborated with this. Interestingly, there was diastolic MR also, due to prolonged PR interval [Figure 6]c. LA and LV were dilated without regional wall motion abnormality (RWMA) indicating absence of any coronary arterial compression by the aneurysm. Inferior vena cava dimension and respiratory variation were normal. Tricuspid regurgitation (TR) peak velocity was 3.2 m/sec, yielding a peak gradient of 43 mmHg, with calculated pulmonary artery systolic pressure being 48 mmHg. Diastolic TR was also noted, similar to diastolic MR, due to prolongation of PR interval [Figure 6]d.

Figure 3: (a) Two dimensional echocardiogram in the parasternal long-axis view showing a cystic structure in the left ventricular outflow tract (horizontal arrow), an echo-free space was also noted in the proximal interventricular septum (vertical arrow). (b) The cystic structure (C) was collapsing in systole (yellow arrow); white arrow shows the echo free space in the proximal ventricular septum. (c) Apical five-chamber view shows the cystic structure (arrow) in the left ventricular outflow tract. Echo free space in the proximal ventricular septum is also seen (E). (d) Modified parasternal long-axis view showing the echo-free space from the aortic root (horizontal white arrow) in continuity with the echo free space in the proximal interventricular septum (vertical arrow) coming from right sinus of Valsalva (open yellow arrow). Ao: Aorta, LA: Left atrium, LV: Left ventricle

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Figure 4: Continuous wave Doppler showing (a) To-and-fro flow into the aneurysm from the aortic root. (b) Left ventricular outflow tract peak systolic velocity of 2.57 m/s, peak gradient of 26.4 mmHg and aortic regurgitation pressure half time of 597 ms. (c) No obstruction to right ventricular outflow, but there is a high velocity pulmonary regurgitation jet indicating the presence of pulmonary hypertension

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Figure 5: Short-axis view at the base showing the giant aneurysm (A) measuring 4.5 cm × 2.0 cm (double headed arrows) occupying the right ventricular outflow tract and protruded into the right atrium. White arrow indicates aorta in the short-axis. Ao: Aorta, LA: Left atrium, RA: Right atrium, RVOT: Right ventricular outflow tract

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Figure 6: (a) Two-dimensional echocardiogram in the apical four-chamber view shows an eccentric jet of mitral regurgitation having vena contracta of 8 mm. (b) Parasternal long-axis view, early systolic frame shows that the aneurysm (A) abuts the anterior mitral leaflet (white arrow) and its chordae (yellow arrow). (c) Continuous-wave Doppler mitral regurgitation spectrum shows diastolic mitral regurgitation (arrows) and prolonged PR interval in the electrocardiography. (d) Continuous-wave Doppler tricuspid regurgitation peak systolic velocity is 3.2 m/s and peak systolic gradient is 43 mmHg; diastolic tricuspid regurgitation is also noted (arrows) due to PR prolongation. Ao: Aorta, Diast: Diastolic, MR: Mitral regurgitation, LA: Left atrium, LV: Left ventricle, TR: Tricuspid regurgitation

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[Additional file 1]

Video 1: Two-dimensional echocardiography in the parasternal long-axis view showing a cystic structure in the left ventricular outflow tract which collapses in systole and expands in diastole. An echo-free space is also seen in the proximal interventricular septum.

[Additional file 2]

Video 2: Two-dimensional echocardiography in the apical five-chamber view showing a cystic structure in the left ventricular outflow tract which collapses in systole and expands in diastole. An echo-free space was also noted in the proximal interventricular septum

[Additional file 3]

Video 3: Slight rotation of the probe in the parasternal long-axis view showing that the echo-free space in the proximal interventricular septum is in communication with the aortic root.

[Additional file 4]

Video 4: Further tilting of the probe shows that the cystic space in the left ventricular outflow tract, echo-free space in the proximal interventricular septum and the aortic root in the region of right sinus of Valsalva are in continuity indicating that a giant sinus of Valsalva aneurysm is burrowing into the interventricular septum and is also protruding into the left ventricular outflow tract

[Additional file 5]

Video 5: Color Doppler interrogation shows color flow from the aortic root into the aneurysm

[Additional file 6]

Video 6: Color Doppler interrogation in the apical five-chamber view shows moderate aortic regurgitation

[Additional file 7]

Video 7: Short-axis view at the base shows that the giant aneurysm occupies the right ventricular outflow tract and also protrudes into the right atrium

[Additional file 8]

Video 8: Colour Doppler interrogation of the aneurysm did not reveal rupture into any of the adjacent chambers

[Additional file 9]

Video 9: Two-dimensional echocardiography in the apical four-chamber view shows the presence of severe mitral regurgitation.

Final diagnosis based on the complete assessment was a giant unruptured right SVA, burrowing into IVS and protruding into LVOT, with mild LVOT obstruction and mild to moderate AR, severe MR including diastolic MR, LA and LV dilatation, LV systolic dysfunction, no RWMA, PA systolic pressure approximately 48 mmHg, sinus rhythm, LBBB and first degree atrio-ventricular block, with New York Heart Association functional class II.

Discussion

SVAs may be formed in one or more of the sinuses of Valsalva and are thin-walled outpouchings that project into adjacent cardiac structures in the shape of a “windsock” caused by the weakness of the elastic and muscular tissues of the aortic root between the annulus and the sino-tubular junction. Although SVAs are mostly congenital, they may also occur secondary to connective tissue disorder, infection, and trauma. Rupture of the aneurysms is the usual mode of presentation that causes a fistulous connection between the aneurysm and the adjacent cardiac chamber. Unruptured aneurysms may remain asymptomatic, to be detected incidentally, or may produce symptom due to compression, dissection, or encroachment of the adjacent structures.^[8]

The present case depicted a giant SVA that had burrowed through the IVS and caused conduction disturbance, protruded into the LVOT causing LVOT obstruction, AR and also MR due to encroachment on the mitral apparatus. The cyst-like structure was differentiated from a true cyst by its collapsibility in systole; true cysts would have been stable throughout the cardiac cycle. The color and Doppler flow pattern into and out of the aneurysm from aortic root differentiated it from a sub-aortic aneurysm or aortico-LV tunnel. In a sub-aortic aneurysm, the flow occurs from LV whereas in an aortico-LV tunnel, the flow resembles AR. Interestingly, diastolic MR was noted which was explained by the prolonged PR interval. Mitral valve opens during atrial systole, but prolonged PR interval delays initiation of systole, during which period the LV pressure exceeds that of left atrium causing a low velocity MR.^[9]

Yang et al. recently described echocardiographic features from 270 patients with SVAs treated over 18 years between 1995 and 2013.^[10] In their series, 22 out of 270 cases had rare patterns which included a rare origin, a rare extending position or a rare course. The three most common aneurysmal complications were severe AR, obstruction of the ventricular outflow tract or valvular orifice, and conduction disturbance. The authors recommend combining the conventional echocardiography with different imaging techniques, such as TEE, 3D echocardiography, CT, and aortic angiography.

However, in our case, the diagnosis was apparent with TTE itself, which is the imaging modality of choice for diagnosis. Cheng et al. described echocardiographic features of SVAs in 212 surgically treated patients over 17 years from 1995 to 2012. They emphasized the importance of echocardiography in the diagnosis of SVA. When confirmed by the surgical findings, the sensitivity, specificity, and accuracy of echocardiographic diagnosis of SVAs were 93.9%, 99.9%, and 99.8%, respectively.^[11]

Conclusion

SVA is a rare disorder but easy to identify during echocardiography. Giant unruptured aneurysms often present a diagnostic challenge. They obstruct, protrude, and dissect into the adjacent cardiac structures causing various clinical, electrocardiographic and echocardiographic abnormalities. The identification of the aneurysm, its origin, location, and course is essential from the management point of view. Although TTE is the method of choice for diagnosis, other imaging modalities may also be needed to exactly delineate this disorder.

Declaration of patient consent

All appropriate patient consent has been obtained for her images and other clinical information to be reported in the journal. The patient understood that her name and initials will not be published and due efforts are taken to conceal her identity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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