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| CASE REPORT |
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| Year : 2019 | Volume
: 3
| Issue : 3 | Page : 122-124 |
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Right atrial myxoma presenting as pyrexia of unknown origin
Narendra Kumar1, Madhan Kumar Ranganathan2, Cyril James3, Shaimaa Mustafa4, Arun Kumar Jha5, Ismail Aksoy6, Manish Barman7
1 Department of Cardiology, Manchester University NHS Foundation Trust, Manchester, UK
2 Department of Clinical Appeals, BronxCare Health System, New York City, New York, USA
3 Department of Cardiology, Wexford General Hospital, Wexford, Ireland
4 Department of Cardiovascular Medicine, Faculty of Medicine, Benha University, Banha, Egypt
5 Department of Cardiology, Indira Gandhi Institute of Cardiology, Patna, Bihar, India
6 Department of Cardiology, Admiraal De Ruyter Hospital, Goes, The Netherlands
7 Department of Cardiology, Hamad Medical Corporation; Department of Medicine, Weill Cornell Medicine University, Education City, Ar-Rayyan, Doha, Qatar
| Date of Submission | 03-Aug-2019 |
| Date of Acceptance | 24-Oct-2019 |
| Date of Web Publication | 29-Nov-2019 |
Correspondence Address:
Dr. Narendra Kumar
Department of Cardiology, Manchester University NHS Foundation Trust, Oxford Road, Manchester, M13 9WL
UK
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/hm.hm_16_19
Most cardiac tumors are benign and localized to the left atrium. Right atrial myxomas are rare and mostly discovered during symptomatic workup or as an incidental finding. Usually, right atrial myxomas are comparatively asymptomatic compared to their left atrial counterparts and do not embolize to systemic circulation unless associated with other anatomical defects. We report a rare case presenting with a history of fever and diagnosed by transthoracic echocardiography. The myxoma was successfully removed surgically, and the patient has significantly improved clinically since then.
Keywords: Fever, myxoma, pyrexia of unknown origin, right atrium, right myxoma
How to cite this article:
Kumar N, Ranganathan MK, James C, Mustafa S, Jha AK, Aksoy I, Barman M. Right atrial myxoma presenting as pyrexia of unknown origin. Heart Mind 2019;3:122-4 |
| Introduction | |  |
Cardiac primary tumors have an incidence of 0.0017%–0.19% according to surgery findings, with a majority of them being benign. Atrial myxomas are the most common tumors seen among patients. Right atrial myxomas are rare and account for only 15% of myxomas. The Mayo Clinic series and a surgical case series by Yu et al. have shed important light about their epidemiology and management.[1],[2] Often, right atrial myxoma patients are asymptomatic, but they may present with weight loss, fever, anemia, Raynaud's phenomenon, and elevated erythrocyte sedimentation rate. Electrocardiogram may reveal abnormalities in up to 75% of patients with evidence of left atrial or right atrial enlargement and occasionally, atrial arrhythmias including atrial fibrillation, atrial flutter, and atrial tachycardia. Due to postural variation of the myxoma, not only it damages the chordal apparatus and neighboring leaflets, but also clinical signs and symptoms may vary.
Thrombus from the surface of tumor or its fragments may emboli to distant organs, resulting in chest pain, syncope, hemoptysis, dyspnea, pulmonary hypertension, or even death. Echocardiography is the standard modality for diagnosis and can be augmented with various imaging modalities such as cardiac magnetic resonance imaging and computed tomography (CT). Usually, surgical removal is highly curative with rare recurrences.
This clinical case is interesting considering the clinical presentation and unusual location in the right atrium.
| Case Report | | |
A 50-year-old female diabetic patient presented to the outpatient department with complaints of fever and abdominal pain for 3 months. She had a history of progressive dyspnea on exertion reaching Class 3 New York Heart Association and developed hematuria since the last week. Electrocardiogram revealed sinus rhythm with no significant changes. Chest radiogram showed clear lung field. Only C-reactive protein was marginally elevated; otherwise, other parameters such as complete blood count, procalcitonin, and rheumatologic laboratory tests were within normal limits and family history was negative. Fever was not associated with chills, without any diurnal variation, and was not associated with any respiratory or gastrointestinal involvement. Transthoracic two-dimensional (2D) echocardiography showed a huge mass of 10 cm × 6 cm occupying the whole right atrium moving to and fro across the tricuspid valve with small patent foramen ovale (PFO). To rule out secondary or metastatic tumors, high-resolution CT scan of the abdomen was done, which showed renal infarction and distant metastasis to spleen. Further, pulmonary CT angiogram revealed thrombi in pulmonary artery branches. She was taken urgently for surgery, and a mass measuring 10 cm × 6 cm was resected with closure of PFO using an autologous pericardial patch. Detailed histopathology examination confirmed the diagnosis of myxoma. In the 3-month follow-up, the patient has symptomatically significantly improved.
| Discussion | | |
Up to 3/4th of primary cardiac tumors are benign, with nearly half of them being myxomas. The incidence of the localization of myxomas in the right atrium is 15%–28%. Similarly, our patient also had a benign myxoma localized to the right atrium. The clinical spectrum of presentation of myxomas may vary widely. However, most of such patients present with “myxoma triad which is a triad of symptoms including embolic phenomena, intracardiac flow obstruction, and constitutional symptoms.” Nonspecific symptoms such as fever, myalgia, arthralgia, and weight loss may be seen in 20%–60% of symptomatic cardiac patients. Some patients may have familial history in young years with nevi; atrial myxoma; myxoid neurofibroma; and ephelides syndrome or the lentigines, atrial myxoma, and blue nevi syndrome.[3] Intracardiac flow obstruction symptoms occur in almost every second patient with a cardiac myxoma. The cardiac tumor may not only impede the blood flow across the atrioventricular valve but may also cause mechanical obstruction leading to hemodynamic changes and also damage of the heart valves.
Constitutional symptoms such as fever, malaise, anorexia, and weight loss mimicking connective tissue disorder (due to the release of interleukin-6) are more common in women than men. In addition, more symptoms are observed in cases of right myxoma compared to left-sided ones. The obstruction symptoms are mostly progressive and notorious for causing intermittent syncope, which are in turn related to postural variations and may be associated with sudden cardiac death.[4],[5] The usual locations for right myxomas are interatrial septum, vena cava, and coronary sinus. Usually, right atrial myxomas are asymptomatic. However, symptoms may be caused by reduction in cardiac output secondary to change in the right atrial outflow obstruction. It has been observed that symptoms are unusually more if the cardiac tumor is in the right side. The mechanical obstruction signs and symptoms were not present in this case possibly due to presentation in the early stage of symptoms. Physical examination revealed significantly raised jugular vein. Although there was no peripheral edema, the spleen and liver were enlarged. A systolic murmur of grade ¼ was auscultated at the left sternal border.
Embolization is usually seen in up to 35% of left atrial myxomas. Therefore, such patients may present with the complaints of arrhythmias, chest pain, dyspnea, transient ischemic attacks, loss of vision, etc., Among patients with right atrial myxoma, embolization is seen in up to 10% of patients only which can be of two types: pulmonary artery embolization which can be life threatening and systemic embolization if the patient has associated atrial septal defect or patent ductus arteriosus. Transthoracic 2D echocardiography is the mainstay in the diagnosis of cardiac tumors, which was indeed helpful for this case too. Oliveira et al. have conclusively shown that transesophageal echocardiography is the gold standard for the diagnosis and evaluation of cardiac myxomas.[6] In this case report, a huge right atrial mass of 10 cm × 6 cm was discovered [Figure 1] in a 50-year-old female patient presenting as fever. With the aid of 2D echocardiography, the diagnosis was confirmed and secondary metastasis leading to renal infarction was shown by high-resolution CT scan.[7],[8],[9] The right atrial myxoma was removed by our cardiac surgery team during open-heart surgery procedure as seen in [Figure 2]. Since then, the patient has drastically improved in signs and symptoms with more than 1 year of follow-up. | Figure 1: Transesophageal echocardiogram showing myxoma in the right atrium
Click here to view |
Cardiac myxomas are mostly benign which may have atypical presentation and can easily be diagnosed with transthoracic echocardiography and are completely cured by surgical excision.
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 | | |
| 1. | Wold LE, Lie JT. Cardiac myxomas: A clinicopathologic profile. Am J Pathol 1980;101:219-40.  |
| 2. | Yu K, Liu Y, Wang H, Hu S, Long C. Epidemiological and pathological characteristics of cardiac tumors: A clinical study of 242 cases. Interact Cardiovasc Thorac Surg 2007;6:636-9. |
| 3. | Atherton DJ, Pitcher DW, Wells RS, MacDonald DM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: The NAME syndrome. Br J Dermatol 1980;103:421-9. |
| 4. | Phan K, Wang N, Pison L, Kumar N, Hitos K, Thomas SP, et al. Rivaroxaban versus warfarin or dabigatran in patients undergoing catheter ablation for atrial fibrillation: A meta-analysis. Int J Cardiol 2015;185:209-13. |
| 5. | Gelsomino S, Lozekoot PW, Lorusso R, de Jong MJ, Parise O, Matteucci F, et al. The optimal weaning strategy for intraaortic balloon counterpulsation: Volume-based versus rate-based approach in an animal model. Ann Thorac Surg 2016;101:1485-93. |
| 6. | Oliveira R, Branco L, Galrinho A, Abreu A, Abreu J, Fiarresga A, et al. Cardiac myxoma: A 13-year experience in echocardiographic diagnosis. Rev Port Cardiol 2010;29:1087-100. |
| 7. | Kumar N, Bonizzi P, Mafi Rad M, Lankveld T. Left atrial dyssynchrony time measured by tissue Doppler imaging to predict atrial fibrillation recurrences after pulmonary vein isolation: Is this a mirage or the panacea? Anatol J Cardiol 2015;15:123-4. |
| 8. | Kumar N, Dinh T, Magdi Abbas M, Phan K, Manusama R, Philippens S, et al. Failure to reach the optimal temperature during cryoablation due to refrigerant cylinder problem. Res Cardiovasc Med 2015;4:e25592. [Full text] |
| 9. | Thyagarajan B, Kumar MP, Patel S, Agrawal A. Extracardiac manifestations of atrial myxomas. J Saudi Heart Assoc 2017;29:37-43. |
[Figure 1], [Figure 2]
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