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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 139-141

Left atrial tumor, complete heart block, and triple-vessel coronary artery disease: A very rare case report


Department of Cardiology, Advanced Cardiac Centre, PGIMER, Chandigarh, India

Date of Web Publication31-Jul-2019

Correspondence Address:
Dr. Suraj Khanal
Department of Cardiology, 3rd Floor, Block-C, Advanced Cardiac Centre, PGIMER, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JCPC.JCPC_21_19

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  Abstract 


Primary cardiac tumor (PCT) is a very rare clinical presentation, with myxoma representing the most common type. Majority of the myxomas are found in the left atrium along with a female dominance. This report describes a very rare case of a 67-year-old woman presenting with PCT, complete heart block, and triple-vessel coronary artery disease. The patient was planned to undergo a coronary artery bypass grafting (CABG) along with concomitant tumor excision and epicardial pacemaker insertion. Since the patient and her family did not consent for the CABG, transvenous permanent pacemaker insertion was performed with an advice to follow-up for CABG and tumor excision.

Keywords: Complete heart block, coronary artery disease, primary cardiac tumor, three-vessel disease


How to cite this article:
Khanal S, Pruthvi C R, Kanabar K, Bootla D, Prasad K. Left atrial tumor, complete heart block, and triple-vessel coronary artery disease: A very rare case report. J Clin Prev Cardiol 2019;8:139-41

How to cite this URL:
Khanal S, Pruthvi C R, Kanabar K, Bootla D, Prasad K. Left atrial tumor, complete heart block, and triple-vessel coronary artery disease: A very rare case report. J Clin Prev Cardiol [serial online] 2019 [cited 2019 Oct 21];8:139-41. Available from: http://www.jcpconline.org/text.asp?2019/8/3/139/263830




  Introduction Top


With an incidence of 0.3%–0.7%, primary cardiac tumor (PCT) is a very rare clinical presentation as per the published surgical and autopsy reports.[1],[2] Majority of these tumors are benign in nature, with myxoma representing the most common type. In a 14-year population study on 42 consecutive cases, the incidence rate of PCTs was found to be 1.38 cases/100,000 population/year, with 38 benign (90.5%) and 4 (9.5%) malignant cases.[3] More than 80% of the myxomas are found in the left atrium (LA) along with a female dominance and a peak onset age of 40–60 years.[4] This report describes a very rare case of a 67-year-old woman presenting with PCT, complete heart block (CHB), and triple-vessel coronary artery disease (CAD). We reviewed evidence for this unusual presentation in the literature but could not find even a single reported case till date.


  Case Report Top


A 67-year-old female patient with a history of hypertension presented with complaints of recurrent episodes of presyncope for 3 days. The patient had a history of occasional episodes of dizziness (New York Heart Association Class II) and dyspnea on exertion for 6 and 4 months, respectively. On examination, she was found to have bradycardia (pulse rate 32/min), cannon A waves on jugular venous pressure, and a systolic murmur in the mitral area. Electrocardiogram showed CHB with atrioventricular (AV) dissociation along with a narrow QRS escape (ventricular rhythm of 32/min) and alternating bundle-branch block [Figure 1]. The cardiac enzymes including quantitative cardiac troponin-T and creatine kinase-muscle/brain were within the normal range. Temporary pacemaker insertion was done for the management of CHB. A two-dimensional (2-D) transthoracic echocardiogram revealed a thick interatrial septum (IAS) of 22 mm along with a 23 mm × 23 mm mobile, pedunculated mass in the LA, attached to the IAS and prolapsing across the mitral valve. There was no significant obstruction to mitral valve inflow with a mean and peak gradient of 2 mmHg and 9 mmHg, respectively [Figure 2]. Mild eccentric mitral regurgitation was present, whereas the left ventricular systolic function was normal. The mass was extending toward the AV ring and the consistency was suggestive of PCT (suspected LA myxoma), although no histological confirmation could be ascertained. A coronary angiogram showed triple-vessel disease, with 70% stenosis of the left anterior descending artery, 90% stenosis of the left circumflex artery, and 80% stenosis of the right coronary artery, respectively [Figure 3]. In view of the rare clinical presentation, coronary artery bypass grafting (CABG) was planned along with concomitant tumor excision and epicardial pacemaker insertion. Since the patient and her family had refused to undergo CABG, transvenous permanent pacemaker insertion was selected as an alternate treatment in view of recurrent episodes of presyncope. The patient was discharged subsequently with an advice to follow-up for CABG and tumor excision.
Figure 1: Electrocardiogram showing complete heart block with atrioventricular dissociation along with a narrow QRS escape

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Figure 2: A two-dimensional transthoracic echocardiogram

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Figure 3: Coronary angiogram

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  Discussion Top


PCT is a rare clinical presentation that can develop in any of the cardiac cavities with 90% of the tumors located at LA mainly adhering to the IAS near the fossa ovalis. The patients with myxoma are frequently asymptomatic, and the diagnosis is often an incidental finding on a 2-D echocardiogram.[5] While myxoma is the most common PCT, the other benign primary tumors include papillary fibroelastoma, rhabdomyoma, fibroma, hemangioma, and lipoma. LA mass in the present case could have various possible differential diagnosis; however, based on the prevalence, location, age, and gender of the patient, the clinical diagnosis of myxoma was considered. Other differential diagnoses that can be considered for an intracavitary cardiac mass-like myxoma include thrombus, lipoma, and nonmyxomatous neoplasm.[4]

First-degree AV block occurs commonly in patients with myxoma. However, to the best of our knowledge, there are only two reported cases of AV dissociation in patients with LA tumor.[6],[7] In the present case, the patient presented to us with recurrent episodes of presyncope. Since the patient did not give consent for CABG with tumor excision and permanent epicardial pacing due to the perceived risks of the procedure, a transvenous permanent pacemaker insertion was carried out. In a case published previously, atrium-His bundle (AH) interval and His bundle-ventricular (HV) interval were measured during a phase of 1:1 AV conduction. While the AH interval was significantly prolonged (>350 ms), the HV interval was within the normal range (45 ms),[6] a finding suggestive of a direct compressive or a local infiltrative effect of the tumor on the AV node. Compressive effect on the AV node has been the most commonly accepted mechanism that links myxoma and AV block, but a local infiltration of the tumor cannot be ruled out. One peculiarity in the presented case was the occurrence of AV block with a left atrial location of the tumor. Since the AV node is a right-sided structure, direct compression can be more easily explained in the context of a mass located in the right atrium.[7],[8],[9] A myxoma located on the left side of the IAS can compress the His bundle or the slow AV nodal pathway that may have left-sided extension near the posteroseptal mitral annulus. In the present case, the echocardiogram has shown the extension of the mass toward the region of the compact AV node, thus explaining the occurrence of AV dissociation.

The coronary angiogram in the present case revealed a triple-vessel CAD. The prevalence of CAD in patients with myxoma ranges between 20.3% and 36.6%.[10] The coexistence of CAD reflects the higher age of presentation of myxoma and validates the strategy of a routine coronary angiogram before the surgery. This co-occurrence of CAD, CHB, and PCT is unique and has never been reported in the literature to the best of our knowledge. Although the ideal management in the present case would have been a surgical excision of the LA mass with CABG and concomitant permanent epicardial pacemaker insertion, the same could not be performed due to the refusal by the patient. In a study by Habertheuer et al., patients with a complete resection had a median survival of 45.7 ± 16.4 months as compared to only 9.3 ± 4.2 months where a complete resection was not technically feasible.[11]

In the present study, the patient did not consent for the surgery and was discharged following transvenous pacemaker insertion for CHB, which we believe was the predominant cause of her acute presentation. The major limitation of this study is the absence of histological confirmation of the diagnosis of PCT and refusal of CABG by the patient. In addition, cardiac magnetic resonance imaging would have yielded a precise diagnosis and the extent of the disease.

This case reports the unusual presentation of LA tumor, CHB, and triple-vessel CAD along with a possible clinical management strategy.


  Conclusion Top


The co-occurrence of LA tumor, CHB, and triple-vessel CAD can occur as an unusual clinical presentation in elderly patients and should be managed with appropriate surgical resection strategy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bruce CJ. Cardiac tumours: Diagnosis and management. Heart 2011;97:151-60.  Back to cited text no. 1
    
2.
Leja MJ, Shah DJ, Reardon MJ. Primary cardiac tumors. Tex Heart Inst J 2011;38:261-2.  Back to cited text no. 2
    
3.
Cresti A, Chiavarelli M, Glauber M, Tanganelli P, Scalese M, Cesareo F, et al. Incidence rate of primary cardiac tumors: A 14-year population study. J Cardiovasc Med (Hagerstown) 2016;17:37-43.  Back to cited text no. 3
    
4.
Burke A, Jeudy J Jr. Virmani R. Cardiac tumours: An update: Cardiac tumours. Heart 2008;94:117-23.  Back to cited text no. 4
    
5.
Auger D, Pressacco J, Marcotte F, Tremblay A, Dore A, Ducharme A. Cardiac masses: An integrative approach using echocardiography and other imaging modalities. Heart 2011;97:1101-9.  Back to cited text no. 5
    
6.
De Maria E, Bonetti L, Olaru A, Cappelli S. Left atrial myxoma associated with advanced atrioventricular block in a very elderly woman. Int J Cardiovasc Res 2014;3:1.  Back to cited text no. 6
    
7.
Tantchou Tchoumi JC, Giamberti A, Cirri S, Butera G. Bi-auricular myxoma associated with atrioventricular dissociation in an 18-year-old boy: A case report. Cardiol Young 2012;22:341-3.  Back to cited text no. 7
    
8.
Martínez-Mateo V, Arias MA, Juárez-Tosina R, Rodríguez-Padial L. Permanent third-degree atrioventricular block as clinical presentation of an intracardiac bronchogenic cyst. Europace 2008;10:638-40.  Back to cited text no. 8
    
9.
Ngow HA, Wan Khairina WM. Isolated right atrium tuberculoma causing complete heart block. Postgrad Med J 2010;86:624-6.  Back to cited text no. 9
    
10.
Li AH, Liau CS, Wu CC, Chien KL, Ho YL, Huang CH, et al. Role of coronary angiography in myxoma patients: A 14-year experience in one medical center. Cardiology 1999;92:232-5.  Back to cited text no. 10
    
11.
Habertheuer A, Laufer G, Wiedemann D, Andreas M, Ehrlich M, Rath C, et al. Primary cardiac tumors on the verge of oblivion: A European experience over 15 years. J Cardiothorac Surg 2015;10:56.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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