Login | Users Online: 2371  
Home Print this page Email this page Small font sizeDefault font sizeIncrease font size   
Home | About us | Editorial board | Search | Ahead of print | Current Issue | Archives | Submit article | Instructions | Subscribe | Advertise | Contact us

Table of Contents
Year : 2016  |  Volume : 17  |  Issue : 1  |  Page : 19-22  

Sporadic multicentric right atrial and right ventricular myxoma presenting as acute pulmonary thromboembolism

1 Department of Cardiology, CARE Hospital, Bhubaneswar, Odisha, India
2 Department of Cardio-thoracic and Vascular Surgery, CARE Hospital, Bhubaneswar, Odisha, India
3 Department of Pathology, CARE Hospital, Bhubaneswar, Odisha, India

Date of Web Publication18-May-2016

Correspondence Address:
Satyajit Singh
Department of Cardiology, CARE Hospital, Plot No - 329/1929, Near Municipal Kalyan Mandap, Chandrasekharpur, Bhubaneswar - 751 016, Odisha
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1995-705X.182642

Rights and Permissions

Multicentric cardiac myxoma is a rare syndrome; usually it is familial. We report a rare case of sporadic right atrium (RA) and right ventricle (RV) myxoma in a 26-year-old female presenting to our hospital for the evaluation of sudden onset of dyspnea and left precordial pain attributed to the embolization of degenerating tumor fragments to the pulmonary artery (PA). The exact incidence of sporadic multicentric RA and RV myxoma presenting as acute pulmonary embolism is unknown as multicentric RA and RV myxoma are very rare. Myxomas presenting as pulmonary embolism is <10%. Majority of cardiac myxomas present as exertional dyspnea, chest pain, positional syncope, fever, weight loss and other constitutional symptoms. Any young patient presenting with acute onset dyspnea with multiple cardiac masses may have tumor embolization to the PA diagnosis with transthoracic echocardiography and high-resolution computed tomography of thorax, fast-tracks patient transfer for urgent cardiac surgery to prevent further embolization.

Keywords: Echocardiography, multicentric cardiac myxoma, pulmonary embolism

How to cite this article:
Singh S, Tripathy MP, Mohanty BB, Biswas S. Sporadic multicentric right atrial and right ventricular myxoma presenting as acute pulmonary thromboembolism. Heart Views 2016;17:19-22

How to cite this URL:
Singh S, Tripathy MP, Mohanty BB, Biswas S. Sporadic multicentric right atrial and right ventricular myxoma presenting as acute pulmonary thromboembolism. Heart Views [serial online] 2016 [cited 2023 Dec 7];17:19-22. Available from: https://www.heartviews.org/text.asp?2016/17/1/19/182642

   Introduction Top

Cardiac myxoma represents approximately 50% of all benign cardiac tumors in adults, with left atrial myxomas constituting 75% of all myxomas.[1] Usually, myxoma is a solitary mass. Multicentric cardiac myxomas are very rare and usually, biatrial. The incidence is <2.5% of all cardiac myxomas.[2] Familial multicentric cardiac myxomas may present as a case of left atrial (LA) and left ventricular (LV) mass simultaneously.[3]

We report a 26-year-old female patient who presented to our emergency department with acute onset of dyspnea and was diagnosed as multicentric right atrium (RA) and right ventricle (RV) myxoma with embolization of tumor fragments to the pulmonary artery (PA) during evaluation with transthoracic echocardiogram and high-resolution computed tomography (HRCT) of the thorax.

   Case Report Top

A 26-year-old female presented to the emergency department with sudden onset of dyspnea and left precordial pain of 6 hr duration. She had no history of exertional dyspnea, fever, weight loss, prolonged immobilization, syncope, focal neurological deficits, substance abuse, endocrine disorders and dermatological ailments in the past. She had no family history of cardiac disorders, including cardiac tumors. The patient's physical examination revealed blood pressure of 94/60 mmHg, heart rate of 120 beats/min, respiratory rate of 40/min, elevated jugular venous pressure with prominent 'v' wave. On cardiovascular system examination, S1 was normal, S2 was narrowly split with loud P2; a pan-systolic murmur of grade III/VI intensity was present along left sternal border. Tumor plop was audible. Chest was clear on auscultation. Abdominal examination was normal. Electrocardiogram revealed sinus tachycardia, right axis deviation and QR in V1 lead. Pulse oximetry revealed a saturation of 85% at room air increasing to 90% with 100% oxygen at 6 L/min. Laboratory examination revealed hemoglobin - 12.6 g/dl, total leukocytes count - 12.600/µL and platelet count of 1,00,000/µL. Liver function tests and renal function tests were normal. Chest roentgenogram was unremarkable.

Transthoracic echocardiogram showed a large pedunculated mobile mass in RA with heterogenous echotexture attached to RA free wall and another mass attached to interatrial septum near fossa ovalis [Figure 1]a. Another mass was seen in RV measuring 11 mm × 11 mm just beneath the septal leaflet of tricuspid valve [Figure 1]b. Larger RA mass was prolapsing into RV producing severe eccentric TR jet with a gradient of 60 mmHg [Figure 1]c, supplementary movie 1]. RV outflow tract and PA was dilated [Figure 1]d. Inferior vena cava was free of any mass or thrombus (supplementary movie 2). HRCT thorax revealed embolization to the lateral aspect of right PA and along the medial aspect of left PA causing subtotal obstruction with pulmonary infarct in left lung [Figure 2]a and [Figure 2]b. Ultrasonography of abdomen and bilateral lower limb Doppler was unremarkable. Contrast-enhanced computed tomography scan of the abdomen was normal.
Figure 1: (a) Modified apical 4 chamber view TTE showing a 2.4 cm × 2.4 cm pedunculated myxoma in the right atrium (RA) attached to RA free wall extending to interatrial septum, (b) Modified apical 4 chamber view TTE showing 1.1 cm × 1.1 cm pedunculated myxoma attached to the septal leaflet of the tricuspid valve, (c) CW Doppler across the tricuspid valve showing severe high-.pressure TR with 60 mmHg gradient, (d) TTE basal short axis view showing dilated RVOT, pulmonary artery and heterogenous pedunculated right atrium mass with calcium specks attached to RA free wall

Click here to view
Figure 2: (a) High-resolution computed tomography of the thorax showing embolic fragment in the lateral aspect of right pulmonary artery (PA) and medial aspect of left PA, (b) High-.resolution computed tomography thorax showing pulmonary infarct in left lung field

Click here to view

Once the diagnosis of multicentric RA and RV myxoma with tumor fragment embolization to PA was made, emergency surgery was performed by standard median sternotomy and cardio-pulmonary bypass using bicaval and ascending aortic cannulation to prevent further embolic events. Systemic hypothermia was used, and cardioplegia was administered for myocardial protection. The RA was then opened and a round lobulated mass measuring 3 cm × 2.5 cm × 2.5 cm was found attached to the RA free wall by a pedicle. Another mass attached to interatrial septum near fossa ovalis was excised. Multiple degenerating tumor strands were detected in RA, which were not visualized by transthoracic echocardiography. Another solitary mass was excised from the RV originating beneath the septal leaflet of tricuspid valve [Figure 3]a and [Figure 3]b. After the direct closure of RA, patient was weaned off the extra-corporeal circulation.
Figure 3: (a) Intra-operative findings showing 3 right atrial myxomas one of which the largest one attached to right atrial free wall, (b) Gross morphology of excised myxoma: Pedunculated lobulated mass held by forcep and degenerating myxomatous mass with hemorrhage seen on extreme left side of picture, (c) Photomicrograph (H and E, ×100) loose myxomatoid stroma with stellate and spindle cells (from right atrium mass), (d) Photomicrograph (H and E, ×45) myxoid stroma with scanty stellate cells and spindle cells amidst degenerating cells and hemorrhage (from right ventricle mass)

Click here to view

Gross pathological examination revealed multiple flesh-colored soft to firm lobulated mass. Gross foci of degeneration were detected. Microscopy revealed hypocellular tumor tissue with myxoid background, isolated spindle and stellate cells with no abnormal mitosis or pleomorphism [Figure 3]c and [Figure 3]d. Gross pathology and histopathological examination were consistent with the diagnosis of cardiac myxoma.

After the surgical excision of the tumor, severity of TR decreased from severe to mild. Her breathlessness decreased. Patient was administered anticoagulation therapy postsurgery. Echocardiographic follow-up at 6 months revealed no recurrence.

   Discussion Top

We experienced a case of 26-year-old female with acute pulmonary embolism attributed to embolization of degenerating tumor fragments from multicentric RA and RV myxoma, sporadic in nature, as there was no family history of cardiac tumors in first-degree and second-degree relatives. Echocardiographic screening in relatives was normal. Usually, myxomas present with obstructive, constitutional or embolic symptoms.

Our patient had no constitutional symptoms of fever, weight loss, malaise or joint pains. She had no obstructive symptoms of exertional dyspnea or positional syncope in the past. Embolism occurs in 30–40% of patients with myxoma.[4],[5] Since most myxomas are located in the LA, systemic embolism is particularly frequent. Pulmonary thrombo-embolism is reported to occur in approximately 3.2% of myxoma patients.[6] Pulmonary embolism is the most dreaded and devastating complication of right-sided myxoma. In cases of right atrial myxomas, clinically evident embolic events are uncommon. Nevertheless, there have been reports of embolization of thrombi or tumor fragments into the pulmonary vessels in cases of right atrial or right ventricular myxoma.[7],[8]

Carney's syndrome consists of myxomas in other locations (breast or skin), spotty pigmentation (lentigines, pigmented nevi or both) and endocrine overactivity (pituitary adenoma, primary pigmented nodular adrenocortical disease, bilateral testicular tumors and schwannomas.[9],[10]

Approximately 7% of cardiac mxyomas are familial or part of Carney's complex. Familial myxomas tend to occur in younger individuals, are often multiple in locations and more likely to have postoperative recurrences. In the case presented here, despite being a multicentric tumor with younger age at presentation, there was no family history unlike other multicentric cardiac myxomas and thus favoring a sporadic occurrence.

Primary benign cardiac tumors are a potentially curable form of heart disease. Early surgical excision of the cardiac myxoma is advocated to prevent embolization, sudden cardiac death and for relief of obstructive symptoms. Cardiac myxomas recur in approximately 12–22% of familial cases and in <3% of sporadic cases.[11] Recurrence is attributed to incomplete, inadequate resection of the tumor, multicentricity, younger age at diagnosis and familial myxoma syndrome. Annual follow-up examination with echocardiography is recommended in multicentric myxoma postoperatively due to recurrence risk.

   Conclusion Top

In patients presenting with sudden onset of dyspnea especially young patients, tumor embolization from right sided tumors should be kept in the differential diagnosis, and timely transthoracic echocardiogram in the emergency room can help in fast-tracking the patient for urgent cardiac surgery to prevent the catastrophic complication of pulmonary embolism and sudden cardiac death.

   References Top

Bjessmo S, Ivert T. Cardiac myxoma: 40 years' experience in 63 patients. Ann Thorac Surg 1997;63:697-700.  Back to cited text no. 1
Doig JC, Ray S, Srikrishna SV, Hilton CJ, McComb JM. Transthoracic and transoesophageal echocardiography in the diagnosis and management of a biatrial myxoma. Eur Heart J 1993;14:1719-20.  Back to cited text no. 2
Akbarzadeh Z, Esmailzadeh M, Yousefi A, Safaei A, Raisi K, Sharifi F. Multicentric familial cardiac myxoma. Eur J Echocardiogr 2005;6:148-50.  Back to cited text no. 3
Blondeau P. Primary cardiac tumors – French studies of 533 cases. Thorac Cardiovasc Surg 1990;38 Suppl 2:192-5.  Back to cited text no. 4
Aldridge HE, Greenwood WF. Myxoma of the left atrium. Br Heart J 1960;22:189-200.  Back to cited text no. 5
Gabe ED, Rodríguez Correa C, Vigliano C, San Martino J, Wisner JN, González P, et al. Cardiac myxoma. Clinical-pathological correlation. Rev Esp Cardiol 2002;55:505-13.  Back to cited text no. 6
Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and echocardiographic features of right atrial masses. Am Heart J 1984;107:745-58.  Back to cited text no. 7
González A, Altieri PI, Màrquez EU, Cox RA, Castillo M. Massive pulmonary embolism associated with a right ventricular myxoma. Am J Med 1980;69:795-8.  Back to cited text no. 8
Bennet WS, Skelton TN, Lehan PH. The complex of myxomas, pigmentation and endocrine overactivity. Medicine 1990;65:399.  Back to cited text no. 9
Watson JC, Stratakis CA, Bryant-Greenwood PK, Koch CA, Kirschner LS, Nguyen T, et al. Neurosurgical implications of Carney complex. J Neurosurg 2000;92:413-8.  Back to cited text no. 10
McCarthy PM, Piehler JM, Schaff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr, et al. The significance of multiple, recurrent, and “complex” cardiac myxomas. J Thorac Cardiovasc Surg 1986;91:389-96.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 New Onset Cardiac Murmur and Exertional Dyspnea in an Apparently Healthy Child: A Rare Localization of Obstructive Myxoma in the Right Ventricle Outflow Tract without Pulmonary Embolization—A Case Report and Literature Review
Carolina D’Anna, Alberto Villani, Antonio Ammirati, Paola Francalanci, Laura Ragni, Giulia Cecconi, Aurelio Secinaro, Marcello Chinali, Antonella Santilli, Paolo Guccione, Lorenzo Galletti, Gianluca Brancaccio
International Journal of Environmental Research and Public Health. 2022; 19(19): 12888
[Pubmed] | [DOI]
2 A right atrial myxoma presenting with misleading features of acalculous cholecystitis
Ioannis G Lempesis,Anna Naxaki,Eirini Koukoufiki,Ioanna Karagkouni,Amalia Tzanatou,Calliroe Tourtidou,Peter C Avgerinos
Oxford Medical Case Reports. 2020; 2020(2)
[Pubmed] | [DOI]
3 The filling defect of pulmonary artery, an imaging finding what we should know
Min Liu, Xin Cao Tao, Zhenguo Zhai, Zhanhong Ma, Li Zhu, Jie Luo
Pulmonary Circulation. 2020; 10(1): 2045894020
[Pubmed] | [DOI]
4 Asymptomatic spontaneous complete dislodgement of a right atrial myxoma
Haseeb Nawaz,Khalid Changal,Imad Hariri,Owais Idris,Karan Bhatti
British Journal of Hospital Medicine. 2019; 80(5): ii
[Pubmed] | [DOI]
5 Pulmonary embolism as the initial manifestation of right atrial myxoma
Guofeng Ma,Dan Wang,Yongtao He,Ruifeng Zhang,Yong Zhou,Kejing Ying
Medicine. 2019; 98(51): e18386
[Pubmed] | [DOI]
6 Neurological manifestations of atrial myxoma: A retrospective analysis
Xue-Yan Wen,Yong-Min Chen,Li-Li Yu,Shu-Rong Wang,Hong-Bo Zheng,Zhi-Bin Chen,Lin Ma,Xiao-Ping Liao,Qi-Fu Li
Oncology Letters. 2018;
[Pubmed] | [DOI]
7 Pulmonary Embolism Following Incomplete Surgical Resection of a Right Ventricular Myxoma: A Case Report and Review of the Literature
Yazan Assaf,Maher Nasser,Hani Jneid,David Ott
Cardiology and Therapy. 2018;
[Pubmed] | [DOI]
8 Recurrent somatic mutations of PRKAR1A in isolated cardiac myxoma
Jian He,Mingju Sun,Enyou Li,Yingyong Hou,Matthew J. Shepard,Di Chen,Karel Pacak,Changsong Wang,Lei Guo,Zhengping Zhuang,Yang Liu
Oncotarget. 2017; 8(61): 103968
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded131    
    Comments [Add]    
    Cited by others 8    

Recommend this journal