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| INTERESTING CASE REPORT |
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| Year : 2023 | Volume
: 7
| Issue : 2 | Page : 193-195 |
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Aneurysm of Sinus of Valsalva with Fistula to the Coronary Sinus
Madhu Shukla, Jagdish Chander Mohan
Department of Cardiology, Institute of Heart and Vascular Diseases, Jaipur Golden Hospital, Delhi, India
| Date of Submission | 03-Feb-2023 |
| Date of Decision | 10-Mar-2023 |
| Date of Acceptance | 13-Mar-2023 |
| Date of Web Publication | 03-May-2023 |
Correspondence Address:
Prof. Jagdish Chander Mohan
A51, Hauz Khas, New Delhi - 110 016
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jiae.jiae_6_23
A 63-year-old female with hypertension, evaluated for atypical chest pain and continuous precordial murmur, was found to have an insignificant stenosis in the proximal right coronary artery with a large tortuous fistula originating from the right sinus of Valsalva to the coronary sinus draining into the right atrium with an unusual course parallel to but distinct from the right coronary artery. Multidetector computed tomographic coronary angiography and cardiac catheterization confirmed the diagnosis. Such an anomaly has not been reported earlier.
Keywords: Arteriovenous fistula, coronary sinus, sinus of Valsalva
How to cite this article:
Shukla M, Mohan JC. Aneurysm of Sinus of Valsalva with Fistula to the Coronary Sinus. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:193-5 |
How to cite this URL:
Shukla M, Mohan JC. Aneurysm of Sinus of Valsalva with Fistula to the Coronary Sinus. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 Sep 27];7:193-5. Available from: https://jiaecho.org/text.asp?2023/7/2/193/375667 |
| Introduction | |  |
Sinus of Valsalva aneurysm (SVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction. This occurs as a consequence of the weakness of the elastic lamina at the junction of the aortic media and annulus fibrosa. Aneurysm of the sinus of Valsalva can be congenital or acquired. Acquired causes include connective tissue disorders, infective endocarditis, trauma, vasculitis, iatrogenic injury, atherosclerosis, medial cystic necrosis, and tuberculosis.[1] Embryogenesis underlying congenital etiopathogenesis includes incomplete fusion of the aortopulmonary and interventricular septa.[2] The right sinus of Valsalva lies close to the tricuspid valve and right ventricular outflow tract, and hence, its fistulous communications with the right ventricle (60%) or the right atrium (30%) may occur.[2] Congenital outpouching of the aortic sinus may also be associated with other defects such as ventricular septal defect and bicuspid aortic valve. Fistulous communication between any sinus of Valsalva with venous coronary sinus has not been reported so far in any of the largest series,[3],[4] although one of 270 cases reported did have a tortuous fistula opening in the right ventricle. This report describes a tortuous fistula communicating the right sinus of Valsalva with the coronary sinus draining into the right atrium and tracking the right atrioventricular groove.
| Case Report | | |
This 63-year-old female was diagnosed to have a continuous precordial murmur in 2017, when she presented with atypical chest pain and was detected to have hypertension. Multidetector computed tomographic (CT) angiography showed a large fistulous communication between the right sinus of Valsalva and the right atrium, and a noncritical stenosis in the proximal right coronary artery. She was put on conservative treatment. She presented in December 2022 with fever, dyspnea, and precordial pain, and was diagnosed to have acute myopericarditis in view of leukocytosis, moderate pericardial effusion, and a B-type natriuretic peptide level of 231 pg/mL. Once fever subsided, she underwent a selective invasive coronary angiogram in light of an abnormal electrocardiogram [Figure 1], which showed a chronic total occlusion of the proximal right coronary artery and a large aneurysm arising from the right sinus of Valsalva with a tortuous course. Her echocardiographic examination showed nondilated aortic sinuses, a fistulous track arising from the right aortic sinus, coursing anterior to the right ventricular outflow tract and the right atrium along the right atrioventricular groove, and reaching the junction of the posterior interventricular groove, wherein it joined the coronary sinus by two different tributaries [Figure 2], [Figure 3], [Figure 4], [Figure 5] and [Video 1], [Video 2], [Video 3]. The coronary sinus was dilated (18 mm). The right ventricular chambers were of normal size with normal motion of the interventricular septum. Normal biventricular function was noted with no other congenital abnormalities. The aortic valve was tricuspid, and there was no aortic regurgitation. [Figure 6] shows the comparative structures and course on CT angiogram versus catheter angiogram. The patient declined any intervention, and is on aspirin, rosuvastatin, telmisartan, and amlodipine. | Figure 1: 12-lead electrocardiogram showing T-wave inversion in the inferolateral leads with tiny Q-waves
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 | Figure 2: (a) Transthoracic echocardiographic short-axis view showing the neck of the fistula originating from the aortic root and opening in an aneurysm which courses anterior to the right ventricular outflow tract. (b) Contrast injection from the right coronary catheter into the neck opacifying the aneurysmal fistula and its entire course as three distinct communicating cavities due to tortuosity of the fistula (1,2,3). LA: Left atrium, LAO: Left anterior oblique, RA: Right atrium, RV: Right ventricle
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 | Figure 3: Transthoracic apical 4-chamber view focusing on the right-sided chambers. In the left panel, the nondilated right atrium and the right ventricle with an ovoid intrapericardial cystic structure (3.5 cm × 3.0 cm, yellow arrows) projecting into the right atrium near the atrioventricular groove are shown. Continuous flow in this structure is shown by continuous-wave Doppler in the right panel. LA: Left atrium, LV: Left ventricle, RA: Right atrium, RV: Right ventricle
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 | Figure 4: (a) Dilated coronary sinus and its tributaries with the fistula tracking along the inferior wall of the left ventricle (yellow arrows). (b) Continuous flow by color flow mapping in the fistula (yellow arrows). LA: Left atrium, LV: Left ventricle
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 | Figure 5: Modified transthoracic echocardiographic 4-chamber view with the posterior tilt showing opening of the dilated coronary sinus into the right atrium without (a) and with color Doppler flow (b). Multiple openings into the coronary sinus are shown (yellow arrows). CS: Coronary sinus, LV: Left ventricle, RA: Right atrium, RV: Right ventricle
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 | Figure 6: Computed tomography angiogram in sagittal view showing the normal origin of the coronary arteries and the right coronary artery coursing anterior to the fistula, which originates from the right sinus of Valsalva and goes posteriorly (a). Its opening into the coronary sinus cannot be seen. (b) Invasive catheter angiogram of the fistula; its termination is not clear, but the right atrium is filling. AA: Ascending aorta, DTA: Descending thoracic aorta, RCA: Right coronary artery
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[Additional file 1]
Video 1: Transthoracic echocardiographic short-axis view showing the origin of the fistula with aneurysm from the aortic root.
[Additional file 2]
Video 2: Modified apical 4-chamber view showing the large coronary sinus with continuous flow draining into the right atrium and two tributaries perpendicular to the sinus.
[Additional file 3]
Video 3: Large intrapericardial cystic mass in the right atrioventricular groove with diastolic expansion.
| Discussion | | |
SVA is a rare congenital or acquired anomaly that is relatively more common among Asians.[1] Incomplete fusion of aortopulmonary and interventricular septa in fetal life may produce inherent weakness at the junction of the aortic annulus with the aortic media, and is possibly the underlying mechanism for SVA.[2] The aorta-right atrium fistula falls within abnormal vascular connections of the aorta named aorto-cameral fistulae. The diagnosis is usually made by transthoracic or transesophageal echocardiography, cardiac catheterization with aortography, or CT angiography. This anomaly may lead to heart failure, infectious endocarditis, and/or arrhythmia.[1] Spontaneous closure is rare and surgical or percutaneous transcatheter closure is usually required.[3],[4] Although the sinus of Valsalva communication with other cardiac chambers (mostly the right ventricle), pulmonary artery, interventricular septum, or pericardium due to slow erosion or rupture has been described,[3],[4] there is no report describing its opening into the coronary sinus.
Aortic root and the sinus of Valsalva develop from the cephalic part of the primitive heart tube called truncus arteriosus. The coronary sinus develops from the left horn of the sinus venosus (and the left common cardinal vein), which is the most caudal part of the primitive heart tube. During the 4th week of embryonic life, looping of the primitive heart tube occurs, and the cephalic and caudal parts come close to each other.[5] It is possible that at this stage of cardiogenesis, communication may develop between the aortic root and the coronary sinus due to some inherent defects which progressively lengthen over time.
The clinical symptoms and age at the manifestation of a congenital SVA depend on the underlying anatomy and the size of the fistulous connection to the right side of the heart. The patients can be asymptomatic for a long time, as in our case. The long-standing left-to-right shunt can produce a significant volume overload, with progressive dilatation of both the tricuspid and mitral valve annuli, with consequent mitral and/or tricuspid valve regurgitation. Our patient did not have this complication due to the small size of the left-to-right shunt, despite a very big fistula size and competent aortic valve. Longevity depends on the magnitude of flow through the fistulous communication, and the chamber or vascular bed that receives the fistula, and the presence or absence of aortic regurgitation. Our patient had all the favorable factors for a benign course.
| Conclusion | | |
This case exemplifies the rare occurrence of fistulous connection between the right sinus of Valsalva and the coronary sinus, possibly of congenital origin.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to her conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | |
| 2. | Ring WS. Congenital heart surgery nomenclature and database project: Aortic aneurysm, sinus of Valsalva aneurysm, and aortic dissection. Ann Thorac Surg 2000;69:S147-63.  |
| 3. | Cheng TO, Yang YL, Xie MX, Wang XF, Dong NG, Su W, et al. Echocardiographic diagnosis of sinus of Valsalva aneurysm: A 17-year (1995-2012) experience of 212 surgically treated patients from one single medical center in China. Int J Cardiol 2014;173:33-9. |
| 4. | Yang Y, Zhang L, Wang X, Lü Q, He L, Wang J, et al. Echocardiographic diagnosis of rare pathological patterns of sinus of Valsalva aneurysm. PLoS One 2017;12:e0173122. |
| 5. | |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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