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| Discussion |
Case Report: A 23-year-old man was admitted with dyspnea on exertion (NYHA grade 3) since one month. There was history of palpitations on and off. Physical examination and laboratory tests were within normal limits.
A 2D echocardiogram was suggestive of a sinus of Valsalva aneurysm (Figs1A and1B).
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Fig: 1 A |
Fig: 1B |
The sinus of Valsalva aneurysm appeared to be partially thrombosed and unruptered and appeared to arise from the left aortic cusp.
Plain and contrast enhanced Cardiac MRI was performed.
Fig. 2A. Haste; axial image (black blood) shows the lumen of the aneurysm as also the thrombosed part.
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Fig: 2 A |
Fig. 2B TruFisp coronal image (white blood) shows the aneurysm to be arising above the left aortic cusp.
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Fig: 2 B |
Figs. 3A and 3B Post contrast images axial and coronal.
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Fig: 3 A |
Fig: 3 B |
Patient then underwent a preoperative coronary angiogram.
Figs. 4A and 4B show opacification of the aneurysm and its communication.
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Fig: 4 A |
Fig: 4 B |
DISCUSSION:
Thurman first described a Sinus of Valsalva aneurysm (SVA) in 1840. SVA is a rare congenital anomaly accounting for 0.5-3% of all congenital cardiac anomalies.
SVA is usually clinically silent but sometimes may present with compression of the adjacent structures or intracardiac shunting caused by rupture of the aneurysm. Approximately 65-85% of SVAs arises from the right sinus of Valsalva followed by the non coronary (10-30%) and left coronary (less than 5%) cusps.
Anatomy:- :- Sinuses of Valsalva are spaces bounded medially by the aortic valve cusps and laterally by the wall of the aorta. There are three sinuses - right, left and noncoronary. The right coronary sinus is adjacent to the pulmonary tract, the crista ventricularis of right ventricle and the anterior portion of the right ventricle. The left coronary sinus is adjacent to the anterior wall of the left ventricle. The non coronary sinus is adjacent to the right atrium near the atrio-ventricular node and bundle of His.
Age:- Unruptured SVAs are detected insidiously on 2D ECHO even in patients older than 60 years of age. Most ruptured aneurysms are seen in the young individuals in the second or third decades.
Sex:-
Males are affected more than the female. (M: F-4:1).
Presentation:
Approximately one quarter of patients with SVAs are asymptomatic.
Dyspnea is the most common presenting symptoms.
Rupture of the aneurysm may occur spontaneously or be precipitated by trauma, exertion or cardiac catheterization. Ruptured SVAs present with specific signs of left to right shunting and they are indistinguishable from coronary arterio-venous fistulas. Those signs are machine type continuous murmurs, bounding pulse, palpable thrill along right or left sternal border.
Ruptured SVAs progress in three stages described by Blackshear.
1) Right chest or right upper quadrant pain
2) Sub acute dyspnea on exertion or at rest
3) Progressive dyspnea, cough, edema and oliguria.
Etiology:-
Primary - Congenital (Idiopathic).
Secondary-
Atherosclerosis
Syphilis.
Cystic medial necrosis; Marfan syndrome.
Blunt or penetrating chest injuries.
Infective endocarditis.
Sinus of Valsalva aneurysms are associated with ventricular septal defect, aortic insufficiency and coarctation of aorta.
Pathophysiology:-
The aneurysm is thought to arise from incomplete fusion of the distal bulbar septum that divides the aorta and the pulmonary artery. It attaches to the annulus fibrous of the aortic valve. It is postulated that after exposure to long standing high pressures, this is the part that forms the sinuses of Valsalva -weakens and becomes aneuysmal.
Imaging:-
1) Plain radiographic features: Since the aortic root is intracardiac, the aneurysm is usually not visible. Rarely, the left aortic sinus aneurysm may bulge in the region of left atrial appendage.
2) Multiplanar transesophageal echocardiography provides precise diagnosis of the aneurysm. It also helps in the identification of the structural anomalies and shunt location for preoperative assessment.
3) 2D ECHO may detect as many as 75% of the SVAs. It may show
- the origin of the sinus
- extension of the sinus.
- associated cardiac conditions.
4) Cardiac MRI-Multiplanar imaging combined with contrast images helps in better delineation of the site and extent of the aneurysm for preoperative assessment.
Treatment:-
Medical management-It usually involves stabilization and preoperative assessment of the patient.
Transcatheter closer of the sinus of Valsalva is done by using the Amplatzer device.
Surgical treatment-It is mainly for a patient with a ruptured aneurysm.
The surgical procedure includes
- Aortic root reconstructions or replacement.
- Aortic valve replacement of repair.
- Bentall procedure (valve conduit)
- VSD or ASD repair.
- Primary suture closures and patch closures.
Complications:-
1) Myocardial infarction due to coronary artery compression from the aneurysm.
2) Complete heart block due to compression of the conduction tissue by the aneurysm.
3) Right ventricular outflow obstruction.
4) Sudden cardiac death.
5) Infective endocarditis.
6) Tamponade if rupture occurs into the pericardium.
Outcome:-
The prognosis depends upon size of aneurysm and whether it is ruptured or not. Unruptured SVAs need to be followed up to monitor increase in size.
Most of the SVAs increase in the size and rupture. Patients with ruptured SVAs die of heart failure or endocarditis within one year of the onset of symptoms.