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 Table of Contents  
INTERESTING CASE REPORT
Year : 2020  |  Volume : 4  |  Issue : 1  |  Page : 42-44

Left Atrial Myxoma: A Great Masquerader with Multiple Clinical Faces – A Case Series


Institute of Cardiology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Date of Submission31-May-2019
Date of Decision31-Jul-2019
Date of Acceptance03-Nov-2019
Date of Web Publication11-Apr-2020

Correspondence Address:
Dr. Mohamed Iliyas
Institute of Cardiology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_28_19

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  Abstract 

While syphilis and pulmonary embolism are well known with the eponym of “The Great Masquerader,” atrial myxoma should also be given the eponym due to its variable presentations and frequent misdiagnosis. Myxoma is the most common primary tumor of the adult heart, which is silent and is discovered incidentally. We would like to present three consecutive patients who attended our department with different symptomatologies over a time frame of 2 months. Later, all the three patients were found to be having the same lesion in the left atrium.

Keywords: Atrial fibrillation, left atrial myxoma, mitral stenosis, myocardial infarction


How to cite this article:
Iliyas M, Swaminathan N, Ganesan G, Sangareddi V. Left Atrial Myxoma: A Great Masquerader with Multiple Clinical Faces – A Case Series. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:42-4

How to cite this URL:
Iliyas M, Swaminathan N, Ganesan G, Sangareddi V. Left Atrial Myxoma: A Great Masquerader with Multiple Clinical Faces – A Case Series. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2020 Sep 21];4:42-4. Available from: http://www.jiaecho.org/text.asp?2020/4/1/42/282200




  Introduction Top


Myxoma is a benign tumor originating from connective tissue. Cardiac myxomas are thought to originate from the subendocardium or multipotent mesenchymal stem cells of the fossa ovalis.[1] Being a tumor of young females, four times commoner than in men, its origin from all four chambers and cardiac valves has been documented. Left atrium, right atrium, right ventricle, and left ventricle are involved in the decreasing order of frequency. The usual presenting features fall into one of the triads namely constitutional, obstructive, or embolic symptoms. With the wide availability of the transthoracic echocardiogram, a diagnosis of an intracardiac myxoma is very rarely missed. Surgical resection of the intracardiac tumor along with the excision of part of interatrial septum followed by pericardial or Dacron patch closure is the ideal treatment of choice which has led to a great reduction in the recurrence rate. The survival rate following the surgery is more than 90%.


  Clinical Presentation Top


Case 1

A 65-year-old female presented to the emergency room (ER) with acute-onset anginal chest pain, palpitation, and sweating for 7 hours (h). Her electrocardiogram (ECG) showed ST elevation in the anterior chest leads and was thrombolysed with streptokinase. Later, she was referred to our hospital for coronary angiogram. Echocardiography done in our hospital showed a 3.1 cm × 2.1 cm mobile mass arising from the lower part of interatrial septum and prolapsing into the left ventricle during atrial systole and returning to the left atrium during atrial diastole [Figure 1]. The mass had an irregular surface with numerous finger-like projections. Since atrial myxomas are usually covered with thrombus on its surface, a suspicion of coronary artery embolism was the first diagnosis we looked for, though its reported incidence is around 0.06%. Coronary angiogram revealed diffuse atheromatous changes involving the proximal and mid-left anterior descending (LAD) artery with a maximum of 90% eccentric stenosis noted in the mid-LAD segment [Figure 2]. Prominent left atrial (LA) branch arising from the left circumflex artery was noted. A tumor blush was seen in coronary angiography. Since coronary emboli from the tumor could not be ruled out which can recur, the tumor was completely excised along with the interatrial septum by the right atrial approach and pericardial patch closure was done. Coronary artery bypass grafting with a saphenous vein grafts to distal LAD was also carried out. The excised myxoma was covered with thrombus on its surface. The cut sections of the biopsy showed round-to-oval cells with abundant eosinophilic cytoplasm with inconspicuous nucleoli and irregular nucleus with the cells arranged in groups and nests. Many proliferating blood vessels and fibrotic bands were seen. All these features suggested that the biopsied atrial tumor was a myxoma.
Figure 1: Case 1 – Left atrial myxoma prolapsing into the left ventricle indicated by arrow

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Figure 2: Case 1 - Coronary angiography showing diffusely diseased left anterior descending artery

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Case 2

A 48-year-old female attended our outpatient department to get relief from breathlessness. She was asymptomatic 2 weeks back. She felt breathless while she climbed the stairs 2 weeks back. She became breathless at rest for 2 days. There was no history of multiple joint pains in her childhood. On examination, there was tapping apical impulse with a loud first heart sound accompanied by a low pitched rough rumbling mid-diastolic murmur that was best heard in the left lateral position with a presystolic accentuation. The ECG was normal. We suspected a rheumatic heart disease with a stenotic mitral valve. Echocardiogram focused on the mitral valves revealed no evidence of rheumatic involvement of the leaflets. However, there was a 6.6 cm × 4.08 cm sized mass in the left atrium that was attached to the entire anterior mitral leaflet and the whole of interatrial septum causing restricted mitral valve opening [Figure 3]. The calculated mitral orifice area was 0.98 cm2. It was accompanied by a mild eccentric mitral regurgitation. Subsequently, she was referred for resection of the tumor.
Figure 3: Case 2 – Large left atrial myxoma adherent to the anterior mitral leaflet causing restriction of movement

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Case 3

A 52-year-old female attended the ER with complaints of palpitations and giddiness for the past 9 days. It was episodic without any precipitating and relieving factors. She never experienced syncope. However, she had occasionally felt that her heart stops for a few seconds. She was not on any regular medications and had nil relevant past medical history. Examination revealed irregularly irregular radial pulse with a pulse deficit of 18/min and a variable first heart sound. The rest of the examination was unremarkable. The diagnosis of atrial fibrillation was made from her ECG. We had suspected a nonvalvular atrial fibrillation, but her echocardiogram showed a 4.5 cm × 3.9 cm LA mass arising from the interatrial septum and touching the anterior mitral leaflet [Figure 4]. There was mild mitral regurgitation without any mitral stenosis. The mitral valves were normal. There was no LA appendage clot. Following the diagnosis, she underwent complete resection of the tumor.
Figure 4: Case 3 – Left atrial myxoma without restricting anterior mitral leaflet mobility

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  Discussion Top


LA myxoma had presented as an acute coronary syndrome (Case 1), non-rheumatic mitral stenosis (Case 2), and atrial fibrillation (Case 3) in our case series. Myxoma should be suspected in patients with signs of severe mitral stenosis and a comparatively smaller-sized left atrium.[2] Myxoma can be sessile or pedunculated; if sessile, they are usually villous. The surface serves as a source for thrombus. Our first case had numerous villous-like projections, and hence, thrombogenic surface was suspected. Since the patient had presented with myocardial infarction, the possibility of coronary artery embolism was kept in mind while performing coronary angiogram. Diffuse coronary artery disease dismissed the possibility of coronary artery embolism, and the presence of LA myxoma was coincidental.

An Indian study involving 171 patients found dyspnea to be the most common symptom with auscultation abnormalities in 89% of their cases.[3] Right-sided myxomas and female patients had systemic symptoms. This study showed that atrial myxoma tends to occur at an earlier age group among Indians compared to the Western population. A French study involving 112 patients showed neurologic symptoms and embolic complications to be common among males, while females developed more systemic symptoms.[4] Compared to older patients, younger patients had neurologic symptoms and auscultatory abnormalities. In one case series of 17 cases, all the patients had mitral valve obstructive symptoms like in our second case.[5]


  Conclusion Top


LA enlargement was the most common ECG abnormality in both the above-mentioned case series. Atrial fibrillation was rare (5%). Our third patient presented with atrial fibrillation without any evidence of mitral stenosis or LA enlargement. Thorough knowledge of the various presenting features of atrial myxoma and a high index of suspicion is necessary to diagnose atrial myxoma at the earliest.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F, et al. Histopathologic and clinical characterization of cardiac myxoma: Review of 53 cases from a single institution. Am Heart J 2000;140:134-8.  Back to cited text no. 1
    
2.
Ghahramani AR, Arnold JR, Hildner FJ, Sommer LS, Samet P. Left atrial myxoma: Hemodynamic and phonocardiographic features. Am J Med 1972;52:525-32.  Back to cited text no. 2
    
3.
Aggarwal SK, Barik R, Sarma TC, Iyer VR, Sai V, Mishra J, et al. Clinical presentation and investigation findings in cardiac myxomas: New insights from the developing world. Am Heart J 2007;154:1102-7.  Back to cited text no. 3
    
4.
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001;80:159-72.  Back to cited text no. 4
    
5.
Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695-701.  Back to cited text no. 5
    


    Figures

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



 

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