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 Table of Contents  
INTERESTING CASE REPORT
Year : 2018  |  Volume : 2  |  Issue : 3  |  Page : 170-173

Serendipitous continuous murmur in an octogenarian turned out to be an asymptomatic ruptured sinus of Valsalva


1 Department of Cardiology, ASRAMS, Eluru, Andhra Pradesh, India
2 Department of General Medicine, ASRAMS, Eluru, Andhra Pradesh, India

Date of Web Publication10-Dec-2018

Correspondence Address:
Dr. Iragavarapu Tammiraju
Department of Cardiology, ASRAMS, Eluru - 534 005, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_15_18

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  Abstract 

Sinus of Valsalva aneurysm is a rare cardiac anomaly which is more often congenital than acquired. Rupture of the aneurysm can present as plain as an asymptomatic murmur to as dramatic as a myocardial infarction or cardiogenic shock or even death. Ruptured sinus of Valsalva (RSOV) is very rare in the elderly, and an asymptomatic rupture in an octogenarian is rarer still. An octogenarian, accompanying his wife for her diabetic follow-up, was found to have a continuous murmur by chance examination. Echocardiography unmasked a RSOV; surprisingly, the elderly man was completely asymptomatic. The patient was referred to a cardiothoracic center for further management. A RSOV is a precarious disposition needing prompt recognition and urgent intervention even though asymptomatic. This case report highlights the importance of recognizing such entities even among the elderly despite its rarity. This case also highlights the cardiovascular auscultation as one of the most important, simple, and inexpensive clinical investigations.

Keywords: Asymptomatic, octogenarian, ruptured sinus of Valsalva


How to cite this article:
Tammiraju I, Akhil S, Kumar A A. Serendipitous continuous murmur in an octogenarian turned out to be an asymptomatic ruptured sinus of Valsalva. J Indian Acad Echocardiogr Cardiovasc Imaging 2018;2:170-3

How to cite this URL:
Tammiraju I, Akhil S, Kumar A A. Serendipitous continuous murmur in an octogenarian turned out to be an asymptomatic ruptured sinus of Valsalva. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2018 [cited 2019 Jan 16];2:170-3. Available from: http://www.jiaecho.org/text.asp?2018/2/3/170/247023




  Introduction Top


A localized weakness of the wall of a sinus of Valsalva, a relatively rare lesion, leads to aneurysmal bulging and even rupture. The localized aneurysms are usually congenital or follow infective endocarditis which is either isolated or associated with ventricular septal defect (VSD), aortic regurgitation (AR), etc., Rupture of the right sinus into the right ventricle is the most common occurrence. It mostly presents as congestive heart failure. Although surgery is the mainstay of treatment, device closure is increasingly performed these days. We report a case of an asymptomatic 80-year-old male who was found to have a ruptured sinus of Valsalva (RSOV).


  Clinical Presentation Top


An 80-year-old man with no significant past medical history accompanied his wife for her diabetic follow-up. A harsh, Grade 4/6 continuous murmur was heard along the mid-to-lower left sternal border on a chance examination of this elderly man. Pulmonary component of the second heart sound was prominent. He was asymptomatic with a heart rate of 84 beats/min. Blood pressure was 120/60 mmHg with wide pulse pressure, and oxygen saturation was 100% on room air. Other systems' examination was unremarkable.

On further evaluation, echocardiography revealed an aneurysm of the right sinus of Valsalva which had ruptured into the right ventricle. The right atrium was mildly dilated. Mild tricuspid and mitral regurgitation and moderate pulmonary hypertension were noted. Left ventricular systolic function was fairly normal. The patient was referred to the cardiothoracic department for repair of RSOV, but he was lost to follow-up.


  Discussion Top


Etiopathogenesis

James Hope brought to light this rare congenital anomaly, “a case of aneurysmal pouch of the aorta bursting into the right ventricle,” in 1839.[1] Sinuses of Valsalva develop as a consequence of incomplete fusion of the distal bulbar septum and truncal ridges, leading to a weakness between the aortic media and the annulus fibrosus of the aortic valve. There is subsequent aneurysmal enlargement at this weak point caused by the high-pressure head at the aortic root.[2],[3]

Majority of the aneurysms are congenital but can arise secondary to syphilis, endocarditis, Marfan syndrome, cystic medial necrosis, and trauma.[2],[4] The right coronary sinus accounted for 65%–70% of the aneurysms, noncoronary sinus aneurysms were 30%, and left coronary sinus aneurysms were <5%.[5] Sinuses of Valsalva aneurysms have one of three basic pathologic patterns. Unruptured aneurysms may cause distortion and obstruction in the right ventricular outflow tract and compress the interventricular septum, resulting in complete heart block with subsequent dizziness and syncope. Coronary artery compression may occur, producing myocardial ischemia and chest pain. Occasionally, a patient with unruptured sinus of Valsalva aneurysm presents with symptoms related to chronic AR.[3],[6] RSOV connects the high-pressure reservoir of the systemic circulation with the low-pressure system resulting in a systolic-diastolic, left-to-right continuous shunt which leads to the volume overload situation that is proportional to the shunt volume. Amount of shunt volume is influenced by the diameter of RSOV and by the level of pulmonary vascular resistance.[7]

Clinical picture

The aneurysms usually rupture between adolescence and early adulthood.[3] Moustafa et al. reported the mean age to be 45 years in a retrospective analysis of 86 patients who underwent surgical repair for the aneurysm.[6] Ruptured aneurysms have been reported more commonly in males, with a male: female ratio ranging from 1.7:1–4:1.[6],[7] Any coronary sinus can rupture into any chamber. Most commonly the right sinus ruptures into the right ventricle and sometimes into the right atrium as well. The noncoronary almost always ruptures into the right atrium and the left one into the left heart.[8] Rupture into the interventricular septum has also been reported.[9]

An aneurysm that actually ruptures is often heralded by the sudden onset of dyspnea and severe chest pain. Following this initial symptomatic period, the patient may become asymptomatic even without treatment as the body adjusts hemodynamically to the left-to-right shunting. However, as the shunting and volume overload overcome the compensatory mechanisms, symptoms of congestive heart failure result.[7] AR and VSD are the more commonly associated congenital anomalies among others such as patent ductus arteriosus, tetralogy of Fallot, coarctation of the aorta, and atrial septal defect.[5] Acute myocardial infarction can occur as a complication, where the left sinus aneurysm would impinge on the left coronary artery.[10] Infective endocarditis can occur as a complication as well.[4]

Classification

Sakakibara and Konno introduced a classification system for RSOV in 1962.[8] Type I is the most common. A modified Sakakibara classification was later introduced by Xin-jin et al. to simplify the original system [Table 1].[11]
Table 1: Classification of Ruptured Sinus of Valsalva

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Diagnosis

Classical history as described above with a continuous murmur on examination hints to the diagnosis of RSOV. As this patient was asymptomatic, the differentials of continuous murmur were analyzed. A physiological classification of continuous murmurs was described by Myers et al. [Table 2].[12]
Table 2: Physiological classification of continous murmurs

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A continuous murmur of patent ductus arteriosus, coronary arteriovenous fistula, and anomalous left coronary artery has also been reported in the elderly.[13],[14],[15] Two-dimensional (2D) echocardiography with Doppler and color flow imaging can help establish the diagnosis of the RSOV. Although a transthoracic echocardiography (TTE) is fairly sensitive in picking up the lesion, a transesophageal echocardiography (TEE) is better at delineating the aneurysm and identifying associated cardiac anomalies. Windsock deformity has been classically described on TEE which at times can also be appreciated on a TTE.[16]

In this case, echocardiography to our surprise revealed a RSOV into the right ventricle (Type 1). The modified 2D echo images of apical four-chamber and parasternal short-axis views showed typical windsock appearance [Figure 1] and [Figure 2]. Color Doppler imaging of modified short-axis view showed turbulent flow across the RSOV tract [Figure 3]. There are no associated lesions such as VSD, significant AR, or right ventricular outflow tract obstruction. Although an asymptomatic RSOV is common, it is very rare in extremes of age. It is highly uncommon for an elderly man to not only have a rupture but also be asymptomatic as well in his 9th decade. Dwivedi et al. reported a similar case of a 6-year-old child with left sinus of Valsalva aneurysm opening into the right atrium.[17] Review of literature showed no reports on asymptomatic RSOV in the 9th decade.
Figure 1: Modified apical four-chamber view showing ruptured sinus of Valsalva tract opening into the right ventricle with typical windsock appearance (white arrow)

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Figure 2: Modified short-axis view showing typical windsock appearance of ruptured sinus of Valsalva draining into the right ventricle (white arrow)

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Figure 3: Color Doppler view showing turbulent flow in the ruptured sinus of Valsalva tract (white arrows)

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Electrocardiography-gated computed tomography (CT) and magnetic resonance (MR) imaging are the newer modalities for diagnosing RSOV. There have been case reports of aneurysms being missed on an echocardiography and later identified on a CT/MR imaging.[18],[19]

Treatment

Heart failure should be managed according to the patients' hemodynamic status. There are no established guidelines for the treatment of RSOV but repair of the aneurysm is the mainstay which can be achieved through the chamber-only approach, dual-chamber approach, and transaortic approach.[20]

The more recent percutaneous transcatheter closure (TCC) can be performed after assessment of the maximum diameter of the aortic end of the RSOV, the minimum diameter and the length of the windsock, and the distance of the aortic end of RSOV from the coronary ostium. The presence of AR and VSD should be ruled out. Rashkind umbrella device was the first to be used in TCC of RSOV; Amplatzer duct occluder and Amplatzer septal occluder have also been utilized.[21] Cocoon duct occluder has also been made use of in eight patients by Sinha et al., with 100% procedural success.[22] Kerkar et al. reported complications such as failure to deploy, residual shunting, and procedure-related AR in a study of 20 patients who underwent TCC for RSOV.[23] In a study by Yan et al., survival postrepair at 10 years was 94% and at 15 years was 90%, whereas survival was reported to be 4 years in ruptured aneurysms if left unrepaired.[24]


  Conclusion Top


RSOV despite its rarity should be kept in the back of mind while working out the differential diagnosis for an asymptomatic continuous murmur even among the elderly as missing it can have dire consequences.

Cardiovascular auscultation remains one of the most important, simple, and inexpensive clinical investigations, which in those with continuous murmurs can lead to a possible diagnosis and establish a targeted strategy for evaluation and treatment.

2D echocardiography is an invaluable tool to identify RSOV though the use of CT/MR can further add to the anatomical delineation which would greatly aid the surgical team.

Timely recognition and intervention can substantially improve the survival rate and long-term outcome of RSOV.

Declaration of patient consent

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

Acknowledgments

We would like to thank Mr. M. Madhu, echocardiographer, ASRAM Medical College, Eluru, Andhra Pradesh, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hope J. A Treatise on the Diseases of the Heart and Great Vessels. 3rd ed. London, UK: John Churchill; 1839.  Back to cited text no. 1
    
2.
Shah RP, Ding ZP, Ng AS, Quek SS. A ten-year review of ruptured sinus of valsalva: Clinico-pathological and echo-Doppler features. Singapore Med J 2001;42:473-6.  Back to cited text no. 2
    
3.
Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK, et al. Transcatheter closure of a ruptured sinus of valsalva aneurysm. Circ J 2006;70:1043-7.  Back to cited text no. 3
    
4.
Kutiyal AS, Daga MK. Ruptured sinus of valsalva with infective endocarditis complicated with post-infectious acute glomerulonephritis: A rare case presentation. J Clin Diagn Res 2016;10:OD14-5.  Back to cited text no. 4
    
5.
Norwicki ER, Aberdeen E, Friedman S, Rashkind WJ. Congenital left aortic sinus-left ventricle fistula and review of aortocardiac fistulas. Ann Thorac Surg 1977;23:378-88.  Back to cited text no. 5
    
6.
Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, et al. Sinus of valsalva aneurysms–47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159-64.  Back to cited text no. 6
    
7.
Perloff JK. Congenital aneurysms of the sinuses of valsalva. In: Perloff JK, editor. The Clinical Recognition of Congenital Heart Disease. 6th ed. Philadelphia: Saunders W.B.; 2012. p. 417-28.  Back to cited text no. 7
    
8.
Sakakibara S, Konno S. Congenital aneurysm of the sinus of valsalva. Anatomy and classification. Am Heart J 1962;63:405-24.  Back to cited text no. 8
    
9.
Choudhary SK, Bhan A, Reddy SC, Sharma R, Murari V, Airan B, et al. Aneurysm of sinus of valsalva dissecting into interventricular septum. Ann Thorac Surg 1998;65:735-40.  Back to cited text no. 9
    
10.
Regueiro Abel M, Penas Lado M, López Ciudad V, Castro Beiras A. Sinus of valsalva aneurysm as a cause of acute myocardial infarction. Rev Esp Cardiol 2002;55:77-9.  Back to cited text no. 10
    
11.
Xin-Jin L, Xuan L, Bo P, Hong-Wei G, Wei W, Shou-Jun L, et al. Modified Sakakibara classification system for ruptured sinus of valsalva aneurysm. J Thorac Cardiovasc Surg 2013;146:874-8.  Back to cited text no. 11
    
12.
O'Rourke RA, Fuster V, Wayne RA, Hursts R. Hurt's the Heart. 12th ed., Ch. 12. New York: McGraw-Hill; 2010. p. 205-11.  Back to cited text no. 12
    
13.
Orimoto Y, Ishibashi H, Sugimoto I, Yamada T, Maruyama Y, Hagihara M, et al. A case of patent ductus arteriosus in an elderly patient treated by thoracic endovascular aortic repair. Ann Vasc Dis 2016;9:326-9.  Back to cited text no. 13
    
14.
Milici C, Bovelli D, Borghetti V, Khoury G, Bazzucchi M, Principi M, et al. A giant coronary artery aneurysm with coronary arteriovenous fistula in asymptomatic elderly patient. Case Rep Vasc Med 2013;2013:847972.  Back to cited text no. 14
    
15.
Korach A, Menon P, Dhadly M, Raby KE, Shemin RJ, Shapira OM, et al. Anomalous left main coronary artery from the main pulmonary artery in an elderly patient. Ann Thorac Surg 2006;82:2285-7.  Back to cited text no. 15
    
16.
Dev V, Goswami KC, Shrivastava S, Bahl VK, Saxena A. Echocardiographic diagnosis of aneurysm of the sinus of valsalva. Am Heart J 1993;126:930-6.  Back to cited text no. 16
    
17.
Dwivedi SK, Saran RK, Sethi R. Ruptured left sinus of valsalva aneurysm to right atrium. Indian Heart J 2005;57:73-5.  Back to cited text no. 17
    
18.
Das KM, El-Menyar AA, Arafa SE, Suwaidi JA. Intracardiac shunting of ruptured sinus of valsalva aneurysm in a patient presented with acute myocardial infarction: Role of 64-slice MDCT. Int J Cardiovasc Imaging 2006;22:797-802.  Back to cited text no. 18
    
19.
Bricker AO, Avutu B, Mohammed TL, Williamson EE, Syed IS, Julsrud PR, et al. Valsalva sinus aneurysms: Findings at CT and MR imaging. Radiographics 2010;30:99-110.  Back to cited text no. 19
    
20.
Gupta M, Shoeb M, Mishra PK, Bathla A, Prasad J. Management of the ruptured sinus of valsalva aneurysms. Int Surg J 2017;4:1873-7.  Back to cited text no. 20
    
21.
Arora R, Trehan V, Rangasetty UM, Mukhopadhyay S, Thakur AK, Kalra GS, et al. Transcatheter closure of ruptured sinus of valsalva aneurysm. J Interv Cardiol 2004;17:53-8.  Back to cited text no. 21
    
22.
Sinha SK, Khanna NN, Razi M, Krishna V, Jha MJ, Mishra V, et al. Safety and feasibility of transcatheter interruption of ruptured sinus of valsalva aneurysm using the cocoon duct occluder: Immediate results and mid-term follow-up. Cardiol Res 2017;8:154-60.  Back to cited text no. 22
    
23.
Kerkar PG, Lanjewar CP, Mishra N, Nyayadhish P, Mammen I. Transcatheter closure of ruptured sinus of valsalva aneurysm using the amplatzer duct occluder: Immediate results and mid-term follow-up. Eur Heart J 2010;31:2881-7.  Back to cited text no. 23
    
24.
Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of Valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann 2008;16:361-5.  Back to cited text no. 24
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]



 

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