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Table of Contents
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Year : 2021  |  Volume : 24  |  Issue : 1  |  Page : 72-74
Windsock deformity of submitral left ventricular aneurysm communicating into left atrium – role of transesophageal echocardiography


1 Department of Cardiac Anaesthesia, Kerala Institute of Medical Sciences, Trivandrum, Kerala, India
2 Department of Cardiothoracic and Vascular Surgery, Kerala Institute of Medical Sciences, Trivandrum, Kerala, India

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Date of Submission20-May-2019
Date of Decision23-May-2019
Date of Acceptance26-Sep-2019
Date of Web Publication22-Jan-2021
 

   Abstract 


Submitral left ventricular aneurysm (SMLA) is a rare condition. We report here a 38-year-old male patient, presented with mitral regurgitation and features of congestive cardiac failure (CCF) with New York Heart Association (NYHA) function class III, diagnosed to have SMLA. We discuss here the etiology, types, clinical presentation, and management of SMLA and also the role of transesophageal echocardiography in diagnosis.

Keywords: Submitral left ventricular aneurysm, transesophageal echocardiography, three-dimensional echocardiography

How to cite this article:
Singh SS, Cherian VT, Palangadan S. Windsock deformity of submitral left ventricular aneurysm communicating into left atrium – role of transesophageal echocardiography. Ann Card Anaesth 2021;24:72-4

How to cite this URL:
Singh SS, Cherian VT, Palangadan S. Windsock deformity of submitral left ventricular aneurysm communicating into left atrium – role of transesophageal echocardiography. Ann Card Anaesth [serial online] 2021 [cited 2021 Feb 24];24:72-4. Available from: https://www.annals.in/text.asp?2021/24/1/72/307772





   Introduction Top


Submitral left ventricular aneurysm (SMLA) is characterized by outpouching of the left ventricular wall in the posterior portion of the mitral annulus. Echocardiography plays an important role in the diagnosis of SMLA. Real-time three-dimensional (3D) echocardiography, nuclear magnetic resonance, and multidetector cardiac tomography (CT) also have been used recently in the diagnosis of SMLA.


   Case Report Top


A 38-year-old male, known case of rheumatic heart disease with mitral regurgitation presented with progressive dyspnea on exertion for the past 7 months and orthopnea for 1 week. He was on penicillin prophylaxis from his 18 years of age. His preoperative blood investigations revealed deranged liver function tests and serum creatinine. An electrocardiogram showed normal sinus rhythm with heart rate 110/min. Chest-X-ray revealed cardiomegaly. Transthoracic echocardiography (TTE) revealed submitral aneurysm below lateral annulus with dilated left atrium (LA), moderate mitral regurgitation (MR), and moderate tricuspid regurgitation (TR) with moderate pulmonary artery hypertension and good biventricular function. His coronary angiogram showed normal coronaries with no external compression of the coronary artery. The patient presented with symptoms suggestive of CCF with features of pulmonary edema. He was initially treated with diuretics and ionotropes, two days after which he recovered. He was posted for mitral valve repair/replacement. Patient was administered anesthesia as per institutional protocol, no major hemodynamic instability occurred during induction, and transesophageal echocardiography (TEE) probe (iE33 Philips with X7-2t) was inserted. TEE revealed SMLA below the posterior mitral leaflet of size 6.4 × 3.3 cm [Figure 1] and [Figure 2], bulging into LA creating a windsock deformity and SMLA was seen communicating with LA through a small slit in its roof along with moderate MR [[Figure 3] and Videos 1, 2] and moderate TR. Two distinct jets contributed to MR one through the valve leaflets and other through SMLA into LA. TEE findings were confirmed intraoperatively. Intraoperatively a 4 × 3 cm swelling was seen externally at atrioventricular groove just below the left atrial appendage. Under moderate hypothermic cardiopulmonary bypass (CPB) with cold blood cardioplegic arrest, interatrial septum (IAS) was opened along patent foramen ovale and walls of the SMLA inside LA were excised. The neck of the aneurysm was seen opening just below the PML annulus and the aneurysm was seen extending as a pouch downward and outward from left ventricle, which was seen externally. The neck of the aneurysm from left ventricle (LV) closed with interrupted 4-0 pledgeted sutures. The excess edges of the aneurysm inside LA were plicated. Mitral valve ring annuloplasty was done with 28 mm Medtronic ring. During ring annuloplasty, to reinforce the aneurysm closure, the annuloplasty ring sutures were passed through both the PML annulus and the closed aneurysm neck. Three interrupted pledgetted sutures were used to plicate tricuspid valve annulus from posterior to anterior leaflet. Patient was gradually weaned from CPB with Inj Milrinone 0.35 μg/kg/min and Inj Adrenaline 0.05 μg/kg/min. Post CPB, TEE showed an echo-free space on lateral aspect of PML annulus, but not communicating with LV [Video 3]. There was mild MR, mild TR, and no flow across IAS was seen. Postoperative period was uneventful and followup of the patient for 6 months showed favorable prognosis.
Figure 1: ME four chamber and 3D zoom view showing defect below posterior annulus and slit in SMA communicating to LA. SMA- submitral aneurysm

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Figure 2: ME commissural and two chamber view showing submitral aneurysm, yellow color arrows points toward the defect below the posterior annulus

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Figure 3: Color Doppler image showing SMA communicating to LA and eccentric MR jet

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   Discussion Top


SMLA was first described in 1812 by Corvisart[1] and first case series was reported by Abrahams et al. in 1962 from Nigeria.[2] Even though, it was more commonly documented in African blacks, over the last three decades it was also reported in Indian population.[3]

SMLA is most commonly seen below the PML annuls. Du Toit et al. classified SMLA into three types based on the extension of the aneurysm as Type I: single localized neck, Type II: multiple necks (separate distinct openings), and Type III: involvement of the entire mitral annulus.[4]

The etiology can be due to congenital defect in the posterior mitral valve ring[5] or due to infective and inflammatory causes like Takayasu's arteritis[6] and tubercular pericarditis.[7] The clinical presentation of SMLA may vary from an incidental finding in an asymptomatic patient to more common forms of presentations like significant mitral regurgitation, congestive heart failure, thromboembolism, ventricular arrhythmias, and myocardial ischemia due to compression of the coronary artery and sudden death.[8],[9]

TTE plays an important role in the diagnosis of SMLA, a subpericardial echo-free space is noted below the posterior mitral leaflet and communicating with LV.[10] However, TEE plays a key role in identifying the rupture of the aneurysm into LA,[11] which should be suspected in TTE whenever paravalvular jet of MR is seen. Real-time 3D TEE also help in delineating the aneurysm and better visualization of its extension and also its relationship with surrounding structures.[12] Management of SMLA includes initial stabilization with diuretics and afterload reducing drugs. Definitive treatment is surgical management, which includes closure of the neck of aneurysm and mitral valve repair using ring annuloplasty or mitral valve replacement in case of mitral leaflets are distorted.


   Conclusion Top


Even though SMLA is a rare condition, it should be considered as differential diagnosis in young population presenting with CCF with severe MR in decompensated state. TEE both 2D and 3D plays an important role in the diagnosis and understanding the relation of the aneurysm with other cardiac structures.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Janeira LF, Talit U, Parker R, Hughes CE, Tuna IC. Surgical management of ventricular tachycardia in subannular left ventricular aneurysm. Ann Thorac Surg 1995;60:438-40.  Back to cited text no. 1
    
2.
Abrahams DG, Barton CJ, Cockshott WP, Edington GM, Weaver EJ. Annular subvalvular left ventricular aneurysms. Q J Med 1962;31:345-60.  Back to cited text no. 2
    
3.
Chockalingam A, Gnanavelu G, Alagesan R, Subramaniam T. Congenital submitral aneurysm and sinus of Valsalva aneurysm. Echocardiography 2004;21:325-8.  Back to cited text no. 3
    
4.
Du Toit HJ, Von Oppell UO, Hewitson J, Lawrenson J, Davies J. Left ventricular sub-valvar mitral aneurysms. Interact Cardiovasc Thorac Surg 2003;2:547-51.  Back to cited text no. 4
    
5.
Chesler E, Mitha AS, Edwards JE. Congenital aneurysms adjacent to the anuli of the aortic and/or mitral valves. Chest 1982;82:334-7.  Back to cited text no. 5
    
6.
Rose AG, Folb J, Sinclair Smith CC, Schneider JW. Idiopathic annular submitral aneurysm associated with Takayasu's aortitis. Arch Pathol Lab Med 1995;119:831-5.  Back to cited text no. 6
    
7.
Lintermans JP. Calcified subvalvular left ventricular aneurysm: An unusual case in a 4-year-old child. Pediatr Radiol 1976;4:193-6.  Back to cited text no. 7
    
8.
Chi NH, Yu HY, Chang CI, Lin FY, Wang SS. Clinical surgical experience of congenital submitral left ventricular aneurysm. Thorac Cardiovasc Surg 2004;52:115-6.  Back to cited text no. 8
    
9.
Skoularigis J, Sareli P. Submitral left ventricular aneurysm compressing the left main coronary artery. Cathet Cardiovasc Diagn 1997;40:173-5.  Back to cited text no. 9
    
10.
Simpson L, Duncan JM, Stainback RF. Perforated submitral left ventricular aneurysm resulting in severe mitral annular regurgitation. Tex Heart Inst J 2006;33:492-4.  Back to cited text no. 10
    
11.
Morais H, Branco LM, Cunha R, Martins T. Rupture of a submitral ventricular aneurysm into the left atrium diagnosed by transesophageal echocardiography. Rev Port Cardiol 2007;26:367-72.  Back to cited text no. 11
    
12.
Peters F, Essop R. Congenital submitral aneurysm with rupture into the left atrium: Assessment by 2D and 3D transesophageal echocardiography. Echocardiography 2011;28:E121-4.  Back to cited text no. 12
    

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Correspondence Address:
Subash Sundar Singh
Department of cardiac anaesthesia, Kerala Institute of Medical Sciences, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.ACA_81_19

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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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