Primary heart neoplasms are rare occurring with an estimated incidence of 0.0017-0.19%. Myxoma is the most prevalent primary heart tumor. The right atrium is an unusual localization, occurring only in 15-20% of myxoma cases. We report a rare case of a massive right atrial myxoma causing tricuspid valve obstruction and presenting as syncope and exertional dyspnea. This case illustrates the influence of myxoma's size, position and mobility as well as patient's body posture and respiration to the development of signs and symptoms. Three-dimensional echocardiography proved useful in surgery planning, allowing a better definition of the tumor outline and attachment

Carla Bartosch

Jorge Almeida

Jorge Casanova

João Almeida

K Reynen

K Yu

LE Wold

M Yuce

Olga Azevedo

Paulo Pinho

Rosa Medeiros

RS Bilku

S Alsafwah

SK Surabhi

T Kanda

Tânia Nolasco

English

PubMed

Springer - Publisher Connector

CARDIOVASCULAR 
ULTRASOUND
Azevedo et al. Cardiovascular Ultrasound 2010, 8:23
http://www.cardiovascularultrasound.com/content/8/1/23
Open AccessC A S E  R E P O R TCase reportMassive right atrial myxoma presenting as syncope 
and exertional dyspnea: case report
Olga Azevedo*1, Jorge Almeida2, Tânia Nolasco2, Rosa Medeiros1, Jorge Casanova2, Carla Bartosch3, João Almeida1 
and Paulo Pinho2
Abstract
Primary heart neoplasms are rare occurring with an estimated incidence of 0.0017-0.19%. Myxoma is the most 
prevalent primary heart tumor. The right atrium is an unusual localization, occurring only in 15-20% of myxoma cases. 
We report a rare case of a massive right atrial myxoma causing tricuspid valve obstruction and presenting as syncope 
and exertional dyspnea. This case illustrates the influence of myxoma's size, position and mobility as well as patient's 
body posture and respiration to the development of signs and symptoms. Three-dimensional echocardiography 
proved useful in surgery planning, allowing a better definition of the tumor outline and attachment.
Background
Primary tumors of the heart are rare, with an estimated
incidence ranging 0.0017-0.19%, accounting for < 5% of
all cardiac tumors. Approximately three quarters of them
are benign and nearly half of the benign tumors are myx-
omas. About 75% of myxomas arise in the left atrium.
Right atrial myxomas are rare as myxomas are estimated
to occur in the right atrium in only 15-20% of the cases
[1]. The low incidence rate of right atrial myxoma has
been reported for decades in several autopsy case series.
The Mayo clinic series (1954-1979) included 23673
patients and found 41 patients with primary heart tumors
(0.17%). A myxoma was found in 28 of those patients and
its location was the left atrium in 17 cases and the right
atrium in 4 cases (14%) [2]. More recently, Yu et al., in a
surgical case series, reviewed 33108 patients submitted to
cardiac surgery and found 234 cases with a confirmed
diagnosis of primary heart tumor (0.71%). A myxoma was
found in 184 patients. However, a right atrial localization
was observed in only 12 of those cases (6.5%) [3].
Right atrial myxomas may remain asymptomatic [4] or
eventually cause constitutional signs and symptoms,
including fever, weight loss, arthralgias, Raynaud's phe-
nomenon, anemia, hypergammaglobulinaemia and ele-
vated erythrocyte sedimentation rate (ESR) [1], due to the
production of interleukin-6 [5]. Pulmonary embolism of
tumor fragments or thrombus from the tumor surface
may also occur, resulting in dyspnea, chest pain, haemop-
tysis, syncope, pulmonary hypertension, right sided heart
failure or death [6]. Less frequently, obstruction of the tri-
cuspid valve occurs, resulting in exertional dyspnea, syn-
cope or sudden death [7,8]. Signs and symptoms depend
on size, position and mobility of the tumor, varying with
patient's respiration and body posture [1].
Echocardiography became the standard diagnosis tech-
nique and surgical removal is the recommended therapy,
as it is usually curative [1].
Herein, we present a rare clinical case of a massive right
atrial myxoma with an uncommon clinical presentation.
The value of 3D-echocardiography to the management of
the tumor is discussed.
Case Presentation
Clinical Case
A 74 year-old female, with previous history of hyperten-
sion, experienced syncope while performing a vigorous
exercise (sawing wood). The development of exertional
dyspnea in the next three months motivated her to seek
medical attention. Cardiac auscultation was unremark-
able. Laboratory data showed an elevated ESR (40 mm).
Electrocardiogram was normal. Transthoracic echocar-
diogram (TTE) revealed an echogenic right atrial mass, of
huge dimensions (4 × 6 cm), connected to the lower por-
tion of the interatrial septum by a small pedicle and pro-
lapsing through the tricuspid valve into the right ventricle
* Correspondence: olgazevedo@yahoo.com.br
1 Cardiology Department, Centro Hospitalar do Alto Ave - Unidade de 
Guimarães, Guimarães, Portugal
Full list of author information is available at the end of the article© 2010 Azevedo et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
Azevedo et al. Cardiovascular Ultrasound 2010, 8:23
http://www.cardiovascularultrasound.com/content/8/1/23
Page 2 of 3during diastole (Figure 1 and 2, Additional files 1 and 2).
Doppler revealed a mean pressure gradient between right
atrium and ventricle of 4 mmHg. Transthoracic 3 D echo
allowed a better definition of the mass outline and con-
firmed the attachment to the interatrial septum below the
fossa ovalis (Figure 3, Additional file 3). The mass was
surgically removed and pathologic analysis confirmed the
echocardiographic suspicion of a myxoma (Figure 4). In
the follow-up performed two months after surgery, the
patient reported a significant symptomatic improvement.
Discussion
This clinical case is quite unusual, considering the mas-
sive dimensions of the myxoma, the uncommon localiza-
tion in the right atrium and the rare clinical presentation
as syncope [8].
In this clinical case, a previously asymptomatic massive
right atrial myxoma prolapsing into the right ventricle
during diastole probably caused temporary complete
obstruction of the tricuspid valve and consequently syn-
cope during a strenuous exercise. Lighter exercises could
also have caused lesser degrees of valve obstruction and
consequently exertional dyspnea, which is in agreement
with the symptomatic improvement reported after the
tumor removal. Conversely, in the decubitus position, the
cardiac auscultation was unremarkable and the echocar-
diogram did not find a higher pressure gradient between
the right cardiac chambers, which reinforces the role of
patient's posture and respiration to the development of
clinical signs.
Although TTE provided a good visualization of the
mass, 3 D echo proved helpful in surgery planning, allow-
ing a better definition of the tumor outline, position and
attachment in the interatrial septum.
Figure 1 M-mode of the right atrial mass. M-mode obtained from a 
subcostal view showing a huge mass in the right atrium prolapsing 
into the right ventricle during diastole.
Figure 2 Right atrial mass on 2D-echocardiography. A subcostal 
view showing a huge echogenic mass in the right atrium, prolapsing 
through the tricuspid valve.
Figure 3 Right atrial mass on 3D-echocardiography. 3 D full vol-
ume image reconstruction better defined the mass contour and con-
firmed its attachment (arrow) in the interatrial septum below the fossa 
ovalis.
Figure 4 Histopathological analysis of the atrial mass. (A) Explant-
ed mass, measuring 4 × 6 cm, and its attachment remnant; (B) Histo-
logical section showing the typical myxoid stroma and embedded 
polygonal cells, blood vessels and areas of hemorrhage (Hematoxylin 
and Eosin, × 100).
Azevedo et al. Cardiovascular Ultrasound 2010, 8:23
http://www.cardiovascularultrasound.com/content/8/1/23
Page 3 of 3Conclusion
In summary, right atrial myxomas are rare and syncope is
a possible although uncommon clinical presentation.
This case emphasizes the importance of myxoma's size,
position and mobility as well as patient's posture and res-
piration to the development of signs and symptoms.
Right heart tumors should be considered in differential
diagnosis of unexplained syncope. TTE is the fundamen-
tal diagnostic technique but 3 D echo may also prove
helpful in surgery planning, allowing a better definition of
the tumor outline, spatial relations and attachment.
Additional material
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
OA, Jorge A and RM drafted the manuscript. TN performed the echocardio-
gram. Jorge A and JC interpreted the echocardiography data. JC performed
the cardiac surgery. CB carried out the pathological analysis. João A and PP
reviewed the manuscript. All authors read and approved the final manuscript.
Author Details
1Cardiology Department, Centro Hospitalar do Alto Ave - Unidade de 
Guimarães, Guimarães, Portugal, 2Cardio-Thoracic Surgery Center, Hospital São 
João, Porto, Portugal and 3Pathology Department, Hospital São João, Porto, 
Portugal
References
1. Reynen K: Cardiac myxomas.  N Engl J Med 1995, 333:1610-7.
2. Wold LE, Lie JT: Cardiac myxomas. A clinicopathological profile.  Am J 
Pathol 1980, 101:219-33.
3. 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.
4. Yuce M, Dagdelen S, Ergelen M, Eren N, Caglar N: A huge obstructive 
myxoma located in the right heart without causing any symptom.  Int J 
Cardiol 2007, 114:405-6.
5. Kanda T, Umeyama S, Sasaki S, Nakasato Y, Morishita Y, Imai S, Suzuki T, 
Murata K: Interleukin-6 and cardiac myxoma.  Am J Cardiol 1994, 
74:965-7.
6. Alsafwah S, Lababidi Z: Recurrent pulmonary embolism originating 
from right atrial myxoma.  J Am Soc Echocardiogr 2001, 14:305-7.
7. Bilku RS, Loubani M, Been M, Patel RL: Massive right atrial myxoma 
causing exertional dyspnoea.  Eur J Echocardiogr 2008, 9:130-2.
8. Surabhi SK, Fasseas P, VanDecker WA, Hanau CA, Wolf NM: Right atrial 
myxoma in a patient presenting with syncope.  Tex Heart Inst J 2001, 
28:228-9.
doi: 10.1186/1476-7120-8-23
Cite this article as: Azevedo et al., Massive right atrial myxoma presenting as 
syncope and exertional dyspnea: case report Cardiovascular Ultrasound 2010, 
8:23
Additional file 1 2D-echocardiography: subcostal view. Huge right 
atrial mass attached to the lower portion of the interatrial septum by a 
small pedicle, moving back and forth through the tricuspid valve.
Additional file 2 2D-echocardiography: apical 4 chambers view. Color 
flow Doppler revealed a mild tricuspid regurgitation. The diastolic flow 
shows laminar flow through the tricuspid valve, which is in agreement with 
the pressure gradient between the right cardiac chambers.
Additional file 3 3D-echocardiography. Live 3 D Echo showing the mass 
attachment below the fossa ovalis and its movement to and through the tri-
cuspid valve.
Received: 3 June 2010 Accepted: 18 June 2010 
Published: 18 June 2010
This article is available from: http://www.cardiovascularultrasound.com/content/8/1/23© 2010 Azevedo et a ; licensee BioMed Central Ltd. is an Op n Access articl  distributed un er the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Card ovascular Ultrasound 2010, 8:23


Massive right atrial myxoma presenting as syncope and exertional dyspnea: case report

Abstract Primary heart neoplasms are rare occurring with an estimated incidence of 0.0017-0.19%. Myxoma is the most prevalent primary heart tumor. The right atrium is an unusual localization, occurring only in 15-20% of myxoma cases. We report a rare case of a massive right atrial myxoma causing tricuspid valve obstruction and presenting as syncope and exertional dyspnea. This case illustrates the influence of myxoma's size, position and mobility as well as patient's body posture and respiration to the development of signs and symptoms. Three-dimensional echocardiography proved useful in surgery planning, allowing a better definition of the tumor outline and attachment.</p

Bartosch Carla

Casanova Jorge

Medeiros Rosa

Nolasco Tânia

Almeida Jorge

Azevedo Olga

Almeida João

Pinho Paulo

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Caglar N: A huge obstructive myxoma located in the right heart without causing any symptom.

Cardiac myxomas.

Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact Cardiovasc Thorac Surg

Interleukin-6 and cardiac myxoma.

Lababidi Z: Recurrent pulmonary embolism originating from right atrial myxoma.

Lie JT: Cardiac myxomas. A clinicopathological profile.

Right atrial myxoma in a patient presenting with syncope. Tex Heart Inst J

RL: Massive right atrial myxoma causing exertional dyspnoea.

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Massive right atrial myxoma presenting as syncope and exertional dyspnea: case report

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