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 Table of Contents  
INTERESTING CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 2  |  Page : 151-154

Clinical Presentation of Acute Ruptured Sinus of Valsalva Aneurysm as a Mimic of Acute Coronary Syndrome


Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, Chennai, India

Date of Submission16-Jan-2022
Date of Decision10-Feb-2022
Date of Acceptance12-Feb-2022
Date of Web Publication23-Aug-2022

Correspondence Address:
Dr. Kothandam Sivakumar
Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, 4A, Dr. J. J. Nagar, Mogappair, Chennai - 600 037, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_5_22

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  Abstract 

Acute ruptured sinus of Valsalva aneurysm may present with chest pain and rapidly worsening dyspnea. A 41-year-old male without known atherosclerotic risk factors presented with ST-segment elevation in leads aVR and V1 on electrocardiogram and elevated cardiac troponins and was diagnosed as acute coronary syndrome. After antiplatelet loading, statin, and heparin, he was referred due to worsening hypotension and tachycardia. Clinical examination and echocardiogram was challenging in the acutely dyspneic restless patient. Transesophageal echocardiography delineated the ruptured aneurysm and assisted in its management. Importance of physical examination and echocardiography in the emergency room is highlighted as manifestations may be varied.

Keywords: Device closure, electrocardiogram, myocardial ischemia, noncoronary aortic sinus, transesophageal echocardiogram, unstable angina


How to cite this article:
Jain G, Sivakumar K, Mumtaz ZA. Clinical Presentation of Acute Ruptured Sinus of Valsalva Aneurysm as a Mimic of Acute Coronary Syndrome. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:151-4

How to cite this URL:
Jain G, Sivakumar K, Mumtaz ZA. Clinical Presentation of Acute Ruptured Sinus of Valsalva Aneurysm as a Mimic of Acute Coronary Syndrome. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2022 Oct 3];6:151-4. Available from: https://jiaecho.org/text.asp?2022/6/2/151/354320


  Introduction Top


Ruptured sinus of Valsalva aneurysm (RSOVA) is a rare but potentially life-threatening clinical entity, which requires immediate recognition and intervention. The presentation may range from an asymptomatic continuous murmur to acute cardiogenic shock and death. Rupture typically occurs in young males usually before 30 years, with a male-to-female ratio of 4:1.[1] RSOVA is five times more common in Asians as compared to the Western population.[2] Acute RSOVA may present with sudden chest pain that resolves after some time, often followed by progressive right heart failure. Only one-third of the patients present with chest pain or severe dyspnea of acute onset, and the rest usually develop progressive breathlessness that worsens with time.[3]


  Case Report Top


A 41-year-old male without any known atherosclerotic risk factors developed acute chest pain and additional autonomic disturbances, such as vomiting and intestinal hurry on an early morning. Initial electrocardiogram showed ST-segment elevation in leads aVR and V1 and global ST-segment depression in anteroinferior leads [Figure 1]. The point-of-care troponin was 0.357 ng/mL (normal– up to 0.034 ng/mL). As the findings were suggestive of proximal left coronary artery occlusion, loading dose of aspirin (325 mg) and clopidogrel (600 mg), statin, and heparin were administered in the emergency room. Rapid worsening to cardiogenic shock with systolic pressures falling below 80 mmHg despite norepinephrine infusions led to referral to our unit. At the time of presentation, the blood pressure was 80/40 mmHg and pulse was rapid and thready with low volume. There was grade II continuous murmur in upper left parasternal area along with bilateral basal crepitations. The audibility of the murmur was affected by the rapid and deep respirations and restlessness of the patient.
Figure 1: 12-lead electrocardiogram in emergency room (a) showing ST-segment elevation in aVR and lead V1 with generalized ST-segment depression in other leads. The changes including sinus tachycardia fully normalized (b) within 24 h of the intervention

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Even though bedside echocardiogram was suboptimal due to deep and rapid breathing, it showed RSOVA from noncoronary cusp to right atrium. After intubation because of pulmonary edema, transesophageal echocardiogram confirmed a large 20 mm aneurysm tapering to a windsock exit of 14 mm near the septal tricuspid annular attachment [Figure 2]. Three-dimensional echocardiography demonstrated normal coaptation of the trileaflet aortic valve and the location of the windsock sac [Figure 3]. Transcatheter closure was planned after informed consent. Contrast angiogram was avoided due to anuria and creatinine levels of 2.3 mg/dL. The mean right atrial pressure was elevated at 18 mmHg, and the pulmonary artery pressure was 50/20 (34) mmHg compared to aortic pressure of 100/40 (65) mmHg. After crossing the aneurysm with an arterial guidewire, an arteriovenous circuit was used to advance a 12F long venous sheath into the aorta. The aneurysm was closed with a 22–20 HeartR duct occluder (Lifetech Scientific, Shenzhen, PRC) under transesophageal echocardiographic guidance using a guidewire maintaining technique [Figure 4]. A limited coronary angiogram using minimal contrast showed normal epicardial coronaries. The improved blood pressure after closure allowed weaning of inotropes, improved urine output and led to rapid clinical recovery. He was discharged home after 48 h without any medications. There was no residual shunt or aortic regurgitation on 1-month follow-up [Figure 5].
Figure 2: Short-axis view of the aortic root on transesophageal echocardiogram (a) with color flows (b) and three-dimensional images in diastole (c) and systole (d) showing sinus of Valsalva aneurysm protruding as a sac into the RA above the TV as a sac from the N. The other two L and R sinuses are not dilated. L: Left coronary sinus, LA: Left atrium, N: Non-coronary sinus, R: Right coronary sinus, RA: Right atrium, RVOT: Right ventricular outflow tract, TV: Tricuspid valve

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Figure 3: A long venous sheath is advanced through the aneurysmal sac into the ascending aorta (a) through which the aortic retention skirt of a duct occluder device is extruded in the ascending aorta (b) before further withdrawal of the sheath to position it in the aneurysm (c) and deploy it (d). L: Left coronary sinus, LA: Left atrium, LAA: Left atrial appendage, LV: Left ventricle, MV: Mitral valve, N: Non-coronary sinus, R: Right coronary sinus, RA: Right atrium, RV: Right ventricle, RVOT: Right ventricular outflow tract

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Figure 4: A guidewire is advanced from arterial catheter through the aneurysm into the right atrium and snared from the superior vena cava (a). A long venous sheath is advanced into the ascending aorta through this circuit to advance a duct occluder device (b) while maintaining the guidewire. After full deployment of the device (c), the guidewire is removed before device release (d). RA: Right atrium

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Figure 5: Three-dimensional echocardiogram of the aortic root showing the final device position in diastole (a) and systole (b). L: Left coronary sinus, N: Non-coronary sinus, R: Right coronary sinus

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  Discussion Top


A congenital defect in the ventriculoaortic junction accounting for <1% of all congenital defects progresses to form a sinus of Valsalva aneurysm.[4],[5] It may project as a blind pouch into the right atrium or ventricle and identified before rupture in 20% of cases.[6] These aneurysms commonly originate from right sinus (70%–90%), noncoronary sinus (10%–25%), and rarely involve left sinus (<5%).[7] Aneurysms from right sinus may rupture into the right ventricle or right atrium, whereas aneurysms of noncoronary sinus rupture into the right atrium.

RSOVA can present as a clinical emergency because of hemodynamic alteration from an acute left-to-right shunt. RSOVA is not commonly considered in the differential diagnosis of acute chest pain with ST-segment elevation. Low aortic diastolic pressures due to acute run-off along with elevated ventricular end-diastolic pressures may cause transmural right ventricular ischemia, contributing to ST-elevation in aVR and diffuse ST-depression in other leads.[8],[9]

Clinical presentation with acute-onset chest pain, breathlessness, electrocardiographic changes of myocardial ischemia, and elevated troponins often divert the emergency room clinician to a diagnosis of acute coronary syndrome and alter the treatment plans. Myocardial ischemia from coronary insufficiency in acute RSOVA may present similar to acute coronary syndrome. Echocardiography is the main diagnostic test for RSOVA, while transesophageal imaging adds information such as size and shape of windsock.[10] As images are often suboptimal during bedside echocardiogram in sick dyspneic patients, underlying structural heart disease may not be sometimes identified.

Management of RSOVA with hemodynamic compromise resulting from the large shunt consists of stabilization of vital parameters in the emergency room, followed by imaging to delineate the aneurysm.[1] Immediate closure of the shunt is mandatory lifesaving procedure, which may be either surgical on cardiopulmonary bypass or by transcatheter intervention similar to the one performed in our patient. Associated defects such as ventricular septal defect, aortic regurgitation, or very large aneurysms measuring more than 16 mm may necessitate surgery.[3] Transcatheter closure may be preferred in patients with severe hemodynamic compromise with high anesthetic risks, acute renal failure, and extreme frailty.


  Conclusions Top


Acute RSOVA may lead to right ventricular ischemia and present with findings that mimic an acute coronary syndrome. Clinical and echocardiographic evaluation in an acutely dyspneic restless patient in emergency room may be challenging. Transesophageal echocardiogram aids in diagnosis as well as guides intervention when acute renal insufficiency precludes a contrast angiogram.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

K Sivakumar is an editorial board member of the Journal of The Indian Academy of Echocardiography & 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.

 
  References Top

1.
Weinreich M, Yu PJ, Trost B. Sinus of Valsalva aneurysms: Review of the literature and an update on management. Clin Cardiol 2015;38:185-9.  Back to cited text no. 1
    
2.
Charan Reddy KV, Vajifdar B, Kumar P, Vazirani A. Diagnosis and management of a rare case of ruptured sinus of Valsalva into the right atrium. J Cardiovasc Med Cardiol 2020;7:33-5.  Back to cited text no. 2
    
3.
Parashar NK, Bhasin D, Marotrao PS, Farooqui FA, Verma SK, Saxena A. Ruptured sinus of Valsalva aneurysm: Clinical case presentation and management. J Pract Cardiovasc Sci 2017;3:109-14.  Back to cited text no. 3
  [Full text]  
4.
Masuyama K, Nakamura H, Okamoto N, Tanaka A, Mori N, Nakamura D, et al. Unusual case of rupture of right sinus of Valsalva aneurysm into the left ventricle. Circ J 2017;81:577-8.  Back to cited text no. 4
    
5.
Lisowski P, Hirnie T, Adamczuk A, Lisowska A, Sobkowicz B, Nadlewski S, et al. Rupture of the sinus of Valsalva aneurysm complicated by infective endocarditisDiagnosis difficulties. Kardiol Pol 2006;64:77-9.  Back to cited text no. 5
    
6.
Sinha SC, Sujatha V, Mahapatro AK. Percutaneous transcatheter closure of ruptured sinus of Valsalva aneurysm: Immediate result and long-term follow-up. Int J Angiol 2015;24:99-104.  Back to cited text no. 6
    
7.
Hanna MF, Malguria N, Saboo SS, Jordan KG, Landay M, Ghoshhajra BB, et al. Cross-sectional imaging of sinus of Valsalva aneurysms: Lessons learned. Diagn Interv Radiol 2017;23:339-46.  Back to cited text no. 7
    
8.
Huang SW, Lo CH, Tsai CF, Su CH. Ruptured sinus of Valsalva aneurysm presenting as acute coronary syndrome with cardiogenic shock and aVR ST-elevation A case report. Acta Cardiol Sin 2020;36:76-80.  Back to cited text no. 8
    
9.
Zhong-Qun Z, Chong-Quan W, Nikus KC, Sclarovsky S, Chao-Rong H. A new electrocardiogram finding for massive pulmonary embolism: ST elevation in lead aVR with ST depression in leads I and V4 to V6. Am J Emerg Med 2013;31:456.e5-8.  Back to cited text no. 9
    
10.
Lakoumentas JA, Bonou MS, Brili S, Theocharis CS, Benroubis AD, Perpinia AS, et al. Ruptured aneurysm of the right sinus of Valsalva into the right ventricle. Hellenic J Cardiol 2002;43:242-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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