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   Introduction
   Case Report
   Discussion
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Table of Contents
BRIEF COMMUNICATION  
Year : 2014  |  Volume : 17  |  Issue : 2  |  Page : 133-136
Left atrial myxoma, ruptured chordae tendinae causing mitral regurgitation and coronary artery disease


1 Department of Anaesthesia and Intensive Care, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Cardiothoracic and Vascular Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

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Date of Web Publication1-Apr-2014
 

   Abstract 

Mitral regurgitation is uncommon with left atrial myxoma. The echocardiographic assessment of presence of mitral regurgitation and its severity are impaired by the presence of left atrial myxoma. We describe an uncommon association of left atrial myxoma with coronary artery disease and mitral regurgitation. MR was reported as mild on pre-operative transthoracic echocardiography but found to be severe due to ruptured chordae tendinae during intra-operative transesophageal echocardiography, which lead to change in the surgical plan to mitral valve replacement in addition to excision of myxoma.

Keywords: Coronary artery disease; Left atrial myxoma; Mitral regurgitation

How to cite this article:
Kumar B, Raj R, Jayant A, Kuthe S. Left atrial myxoma, ruptured chordae tendinae causing mitral regurgitation and coronary artery disease. Ann Card Anaesth 2014;17:133-6

How to cite this URL:
Kumar B, Raj R, Jayant A, Kuthe S. Left atrial myxoma, ruptured chordae tendinae causing mitral regurgitation and coronary artery disease. Ann Card Anaesth [serial online] 2014 [cited 2019 Sep 18];17:133-6. Available from: http://www.annals.in/text.asp?2014/17/2/133/129850



   Introduction Top


Surgical removal of left atrial (LA) myxoma is often accorded high priority due to the possibility of systemic tumor embolization. LA myxoma can also be associated with mitral regurgitation (MR), [1],[2],[3],[4],[5] which is often attributed to failure of leaflet coaptation due to the tumor itself. Less commonly, MR can be due to direct damage of leaflets or subvalvular apparatus by the tumor. [4],[5] A large LA myxoma may manifest with angina in the absence of coronary artery disease (CAD) due to coronary steal phenomenon; [6] however, CAD may be co-existent due to associated atherosclerosis or due to embolization of tumor fragments in to the coronary artery. We describe an uncommon association of LA myxoma, CAD and chronic MR; the MR was diagnosed as "mild" on pre-operative echocardiography carried out previously. During intra-operative transthoracic echocardiography (TTE), regurgitation was found to be severe due to ruptured chordae tendinae that lead to a change in the surgical plan.


   Case Report Top


A 62-year-old male patient presented with progressive dyspnea on exertion and pedal edema since 4 years. A pre-operative TTE showed a 25 × 25 mm polypoid LA mass, with heterogeneous echotexture (areas of echolucency in an otherwise echo-dense structure and speckles of calcification) arising from the inter-atrial septum. Associated findings included mild MR (regurgitant jet area 5.7 cm 2 , LA area 30 cm 2 ), dilated left ventricle (LV) with moderate systolic dysfunction. A coronary angiogram showed diseased left anterior descending (LAD) artery with distal cut-off and retrograde filling by grade 2 collaterals [Figure 1]. The right coronary artery (RCA) appeared normal, but it showed very sluggish flow. The patient was scheduled for excision of LA myxoma. On the day of surgery, 2D apical 4-chamber view using TTE showed smoke in the right atrium (RA) and right ventricle (RV) with poor RV contractility, color Doppler interrogation across the mitral valve showed a wall hugging eccentric MR jet suggestive of severe MR [Figure 2]. TTE examination in modified parasternal long axis view showed eccentric jet due to flail anterior mitral leaflet (AML) with ruptured chordae tendinae [Figure 3]. The TTE findings were confirmed on transesophageal echocardiography (TEE). Intra-operative surgical findings also corroborated the echocardiography findings [Figure 4]. Under standard hypothermic cardiopulmonary bypass (CPB) and cold blood hyperkalemic myocardial protection, the patient underwent mitral valve replacement and excision of LA myxoma.
Figure 1: Coronary angiogram showing distal cut off of left anterior descending artery with retrograde filling. LA - Left atrium

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Figure 2: Transthoracic apical four chamber view showing smoke in right atrium and right ventricle, a myxoma and an eccentric wall hugging jet into left atrium during systole

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Figure 3: Transthoracic parasternal long axis view showing flail tip of anterior mitral leaflet and left atrial myxoma

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Figure 4: Surgeons view showing ruptured chordae tendinae of anterior mitral leaflet

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   Discussion Top


LA myxomas predominantly arise from embryonic remnants of myxoid tissue in the area of fossa ovalis. They can also originate, in descending order of frequency, from the posterior atrial wall, the anterior atrial wall and the atrial appendage. A less common site of origin is the mitral valve leaflet. [7] The myxomas, are generally papillary with friable irregular surface or less commonly ovoid and smooth. [8] Most LA myxoma present with one or more of the triad of embolism, intracardiac obstruction and constitutional symptoms. Occasionally, there are no symptoms, particularly with small tumors. A solid, ovoid myxoma mimics other cardiac tumors like hemangiomas or sarcomas whereas papillary tumors commonly present with embolic phenomenon. [9] Systemic embolism occurs in about one-third of myxomas. In the majority of cases, the cerebral arteries, including the retinal arteries, are affected. Embolizations into visceral, renal and coronary arteries can also occur. [10] The ovoid myxomas often present with signs and symptoms of mitral valve inflow obstruction. The extent of valvular obstruction may vary with body position. If the tumor is large, deformable and has a long stalk, temporary complete obstruction of the mitral valve orifice may occur, resulting in syncope or sudden death. LA myxoma is relatively uncommonly associated with MR; however, when present, it is commonly attributed to failure of leaflet coaptation due to physical presence of the tumor. Other possible causes of MR include coexisting rheumatic or myxomatous mitral valve disease, annular dilatation and rarely direct damage of leaflets or subvalvular apparatus by the tumor.

On echocardiography, LA myxoma is seen as a sessile or, mobile mass attached to the endocardial surface arising from fossa ovalis. Its non-homogeneous texture, areas of echolucency due to hemorrhages or necrosis in the tumor and speckle of calcification help differentiate from other cardiac tumors. [11] LA thrombus generally occurs in patients with organic heart disease, atrial fibrillation or LV dysfunction. [12] LA thrombus is usually attached to the LA appendage or, the posterior LA wall by a broad base, usually immobile and are not associated with systemic constitutional symptoms. [13] TEE has 100% sensitivity for the diagnosis of LA myxoma. 2D echocardiographic and Doppler assessment of mitral valve may be impaired by the presence of LA myxoma and/or shadowing artifact produced by it. LA myxoma can mask the presence of MR and downgrade its severity. [13],[14] 3D TEE may be useful by showing echolucencies better than 2D TEE. In addition, real time 3D enface view of LA myxoma is better appreciated by the operating surgeon, which facilitates understanding of attachment of myxoma to different part of LA, any structural damage of mitral valve [11] and help surgical planning. In the present patient, flail tip of AML was not appreciated on 2D TTE apical 4-chamber view. On color-flow Doppler imaging, eccentric MR jet could be appreciated and on further careful examination in modified parasternal long axis view, the flail tip of AML was noticed [Figure 2]. These findings were confirmed intra-operatively during TEE examination and by the operating team and the flail A 2 segment of AML was due to ruptured chordae tendinae [Figure 4].

The presence of coronary artery lesions on angiogram in these patients can be due to associated CAD or due to embolization of tumor fragments into the coronary artery. The prevalence of CAD with myxoma varies from nil to as high as 20.3-36.6%, depending on age and associated risk factors for atherosclerosis. [15] The incidence of coronary artery embolization of myxoma is about 0.06%. It commonly presents with acute myocardial infarction (MI) due to sudden occlusion of a relatively large vessel. Inferior wall MI is most common and reported in 63.6% of cases, anterior in 22.7% and posterior in 9.1%. [16] In general, resection of the tumor is sufficient to ensure recovery of the patient; however, if there is total or subtotal occlusion of the coronary artery, coronary angioplasty or coronary artery bypass surgery may be necessary. [17] Thrombolytic therapy is not recommended for patients with cardiac myxomas due to the risk of embolism. [18] Although, the present patient had polypoid myxoma but the presence of chronic symptoms, involvement of distal LAD and its branches and absence of the features of embolization to other sites suggested the possibility of atherosclerotic CAD. The smoke in RA and RV due to poor RV contractility was apparently due to sluggish blood flow in RCA. Intra-operatively, RV volume overload was avoided during pre CPB period; the poor RV contractility was managed with judicious use of volume and elective adrenaline infusion before termination of CPB.

Rupture of papillary muscle due to MI may also present as prolapsed or flail mitral valve leaflet. The posterior papillary muscle is more commonly involved but it can affect either leaflet as the papillary muscles send chordae tendinae to both the leaflets of mitral valve. Besides acute and severe manifestation, direct visualization of the rupture of papillary muscle is necessary for its definitive diagnosis. [19] Index case had chronic symptoms with no feature of acute event and importantly demonstrated rupture of chordae tendinae rather than papillary muscle which was possibly due to "the wrecking ball" effect of myxoma on the mitral apparatus.

This case underscores the importance of careful echocardiographic examination in different windows with application of color Doppler in patients of LA myxoma to rule out any damage to the mitral valve apparatus and pre-operative coronary angiogram in patients with high-risk of embolization.

 
   References Top

1.Kamada T, Shiikawa A, Ohkado A, Murata A. A giant left atrial myxoma with severe mitral valve regurgitation: Report of a case. Kyobu Geka 2003;56:152-4.  Back to cited text no. 1
    
2.Yamanaka K, Miki S, Kusuhara K, Ueda Y, Okita Y, Tahata T, et al. Left atrial myxoma associated with ruptured chordae tendineae. Kyobu Geka 1991;44:337-9.  Back to cited text no. 2
    
3.Ceviz M, Erkut B, Gürlertop Y, Unlü Y. Giant left atrial myxoma in a patient with mitral insufficiency: Case report. Heart Surg Forum 2004;7:E269-70.  Back to cited text no. 3
    
4.Sharratt GP, Grover ML, Monro JL. Calcified left atrial myxoma with floppy mitral valve. Br Heart J 1979;42:608-10.  Back to cited text no. 4
    
5.Snir E, Caspi A, Vidne BA. Rupture of chordae tendineae associated myxoma of the left atrium. Scand J Thorac Cardiovasc Surg 1985;19:189-91.  Back to cited text no. 5
    
6.Alexiou K, Wilbring M, Matschke K. Angina pectoris as first manifestation of a huge biatrial myxoma. Acta Cardiol 2009;64:667-8.  Back to cited text no. 6
    
7.Wani ML, Ahangar AG, Singh S. Anterior mitral leaflet myxoma: A rare occurrence. Ann Card Anaesth 2013;16:304-5.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Singh SP, Chauhan S, Chauhan R. Solid ovoid left atrial myxoma. Ann Card Anaesth 2013;16:66-7.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.Bandyopadhyay S, Das RK, Bhelotkar A, Acharia T. A rare case of a left atrial hemangioma mimicking a myxoma. Ann Card Anaesth 2013;16:144-6.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
10.Rath S, Har-Zahav Y, Battler A, Agranat O, Neufeld HN. Coronary arterial embolus from left atrial myxoma. Am J Cardiol 1984;54:1392-3.  Back to cited text no. 10
    
11.Gadhinglajkar S, Sreedhar R. Intraoperative evaluation of left atrial myxoma using real-time 3D transesophageal echocardiography. Ann Card Anaesth 2010;13:180-1.  Back to cited text no. 11
[PUBMED]  Medknow Journal  
12.Tempe DK, Dutta D, Saigal D, Banerjee A. Mobile left atrial mass. Ann Card Anaesth 2013;16:221-2.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
13.Germing A, Lindstaedt M, Mügge A, Laczkovics A, Fritz M. Severity of mitral regurgitation may be underestimated in the presence of a left atrial myxoma. J Heart Valve Dis 2006;15:830-2.  Back to cited text no. 13
    
14.Teng YH, Wang CW, Mao CC. Underdiagnosis of the severity of mitral regurgitation in left atrial myxoma. J Heart Valve Dis 2010;19:806-7.  Back to cited text no. 14
    
15.Erdil N, Ates S, Cetin L, Demirkilic U, Sener E, Tatar H. Frequency of left atrial myxoma with concomitant coronary artery disease. Surg Today 2003;33:328-31.  Back to cited text no. 15
    
16.Panos A, Kalangos A, Sztajzel J. Left atrial myxoma presenting with myocardial infarction. Case report and review of the literature. Int J Cardiol 1997;62:73-5.  Back to cited text no. 16
    
17.Demir M, Akpinar O, Acarturk E. Atrial myxoma: An unusual cause of myocardial infarction. Tex Heart Inst J 2005;32:445-7.  Back to cited text no. 17
    
18.Abascal VM, Kasznica J, Aldea G, Davidoff R. Left atrial myxoma and acute myocardial infarction. A dangerous duo in the thrombolytic agent era. Chest 1996;109:1106-8.  Back to cited text no. 18
    
19.Fradley MG, Picard MH. Rupture of the posteromedial papillary muscle leading to partial flail of the anterior mitral leaflet.Circulation 2011;123:1044-5.  Back to cited text no. 19
    

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Correspondence Address:
Bhupesh Kumar
Department of Anaesthesia and Intensive Care, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.129850

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    Figures

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



 

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