International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumada Thani 1429 H) [631998]

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
141Review article
Atrial Myxoma: Trends in Management
Reyaz A. Lone, Ahanger A G, Shyam Singh, Wani Mehmood, Shabir Shah, Lone GN, Dar AM, Bhat MA,
Sharma ML, Wani Lateef
Cardiovascular Thoracic Surgery Department, Sheri Kashmir, Institute of Medical Sciences Srinagar
(India)
Abstract
Myxomas are the most c ommon type of cardiac tumours in all age groups accounting for one –
third to one -half of cases at postmortum and for about three quarter of tumours treated surgically .
Most atrial myxomas, whether left or right, arise from the atrial septum. About 10% have other
sites of origin, particularly posterior wall, anterior wall and the appendages (in order of
frequency) . Myxomas are frequently located in left atrium and produce symptoms when they
fragment and cause systemic emboli or when they interfere with cardi ac valvular function and
cause pulmonary congestion. Careful surgical management of these lesions should be curative
with minimal early and late morbidity and mortality. Recurrence of atrial myxomas can occur
most likely in about 3% of patients. However , extensive resection of the myxoma attached to
atrial septum or atrial wall can reduce the likelihood of recurren ce to a greater extent. L ong term
clinical and echocardiographic follow -up is mandatory.
Correspondence:
Dr. Reyaz A. Lone
Cardiovascular & Thoracic Surgery Department
Sheri Kashmir, Institute of Medical Sciences Srinagar (India)
[anonimizat]
Introduction
Despite De -Senac’s assertion in 1783 that “t he heart is an organ too noble to be attacked by a
primary tumour” the heart has no specific immunity from neoplasia and cardiac tumours have
become increasingly recognized. Though cardiac tumours can be primary or metastatic, later
group is more common. Among primary cardiac tumours, three quarters are benign and

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
142remainders are malignant.
Myxomas are the most common type of cardiac tumours in all age groups accounting for one –
third to one -half of cases at postmortum and for about three quarter of tumours treated surgically .
(1)Most atrial myxomas, whether left or right, arise f rom the atrial septum, usually from the
region of the limbus of fossa ovalis. About 10% have other sites of origin, particularly posterior
wall, anterior wall and the appendages (in order of frequency) . (2)
Review of Literature
First recognition of a he art tumour is attributed to Columbus in 1559 (3), followed by
Malpighi, who in 1666 wrote a dissertation entitled “De polypo cordis” . (4)
Bahnson and Newman (1953) reported(5) the earliest surgical approaches to myxomas by
removing a myxoma from the righ t atrium via right anterior thoracotomy using a short period of
caval obstruction at normothermia. Craford (1955) [6]successfully excised a myxoma from left
atrium using cardiopulmonary bypass whereas Coates et al (1958) [7]reported a successful
excisio n of a right atrial myxoma.
Firror WB (1966) [8]in a follow -up study of 3 patients, who had undergone removal of
left atrial myxoma five to ten year previously, concluded that it would appear that simple
excision of left atrial myxomas without removal of the atrial wall from which it has arisen is an
adequate operation and is not associated with any evidence of recurrence.
James V. Richardson et al (1979) [9] conducted a study on 11 patients who underwent
surgical excision of atrial myxoma during a 15 ye ars’ period, with no hospital deaths. The
operation consisted of excision of tumour with a generous portion of atiral septum or wall. Patch
reconstruction of atrial septum was required in most patients. There were 2 late deaths.
Late recurrences have been reported in other series but no recurrences were diagnosed in any of
the patients in the series re -examined by echocardiography 7 to 156 months after operation.
Late functional results were excellent. It was concluded that excision of atrial myxoma can be
accomplished safely with good results. Recurrence of atrial myxoma can be as many as 14%
patients, but most likely in 5%. It appeared that extensive resection of the myxoma attachment to

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
143atrial septum, or atrial wall can reduce likelihood of recu urence. Re -recurrence can be noted
several years postoperatively.
Larrieu AJ et al (1982)[10] in their experience of 25 cases of primary cardiac tumours
reported that malignant primary cardiac neoplasms are mostly sarcomas and usually follow a
rapid le thal course. However, they may simulate benign disease, usually a benign myxoma, and
therefore, close preoperative and postoperative scrutiny of all patients with cardiac tumours is
recommended. Surgical resection, when possible, is the treatment of choi ce for all primary
cardiac tumours, since with benign tumours, it is curative and with malignant tumours, it may
prolong life for up to a year.
David C. Cleveland et al (1983) [11] observed that intracavitary tumours of right atrium are
rare, generally diagnosed as myxoma. Once identified by echocardiography, right atrial lesions
should be evaluated further by good quality inferior vanacavagram. The pattern of recurrence
after surgical resection of atrial myxoma would suggest a minimum of five years of follow up by
echocardiography. Myxoma can be malignant; thus complete surgical excision encompassing
full thickness of normal surrounding tissue should be the goal in every case.
Henson EC (1985)[12], in his clinical experience and late results of 33 p atients of atrial
myxomas, 24 patients have been studied by two -dimensional echocardiography upto 20 years
after operation (mean 4 years) with no recurrence. Excellent results were obtained by simple
excision of the tumours with or without the margin of n ormal atrial septum. Long -term clinical
and echocardiographic follow -up is recommended since late recurrence, although rare, has been
reported.
Uberto Bortolotti et al (1990) [13], 54 patients underwent excision of an intracardiac
myxoma, which was locat ed in the left atrium in 46 (85%), right atrium 6 (11%) and right
ventricle in 2 (4%). Extended follow -up of patients with intracardiac myxoma revealed that
excision of such tumours is curative and long term outcome is excellent. Regardless of their
location, cardiac myxomas should always be approached, at least initially, through the intra -atrial
septum. This route provides adequate exposure of the mass in most intances, allowed radical
excision, and is associated with a low incidence of late postoperat ive arrythmias.

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
144Selke FW(1990)[14]in his series of 22 patients who underwent 24 operations for cardiac
myxomas, 2 patients had complex myxoma syndrome. Mitral valve replacement was required in
2 patients. The long -term prognosis of these relatively la rge group patients with cardiac
myxomas has been good. Patients without complex myxoma syndrome had no recurrence. Long
term disability and a chronic arrythmias have been infrequent and functional status and
employability of these patients had been very good.
Meyns B et al (1993)[15], in a series of 32 consecutive patients undergoing resection of a
cardiac myxoma over 20 years period (1971 -1991) reported a mean age of patients as 57 ±11
years. All the myxomas were located in the left atrium and were res ected. Follow up showed
excellent results with no recurrence and concluded that recurrence is mainly confined to patients
with familial presentation and/or myxoma complex.
Castells E (1993),(16)in his series of 27 operated cases, with a follow up of 22 years (mean
6.5 years) reported satisfactory long -term results without recurrence. Hospital mortality was
7.3% and long -term mortality 7%. The postoperative life expectancy of these patients seems
similar to that of normal population, except in cases of recurrence and mitral valve replacement.
Recurrence is very low except in case of young patients and recurrent, familial, multiple or
complex myxomas. The multigrowth potential of the tumour seems more important than
inadequate surgical resection.
Kabba ni SS et al (1994)(17)used biatrial approach for atrial myxoma in 23 patients.
Diagnosis was established preoperatively in all patients by echocardiography with or without
angiocardiography. Twenty one patients had left atrial myxoma and 2 had right at rial myxoma.
All tumours were excised with wide margin of full thickness septum. Four patients had
concomitant mitral valve reconstruction. He suggested that the advantage of dual incisions is:
complete tumour removal, mitral valve visualization and the operative ease, especially with large
tumours.
Kamili and Ahangar (1997) (18) reported a giant right atrial myxoma weighing 100gms, about
8x8cms in size and recommended prompt diagnosis, location of tumour by 2D echocardiography
and subjecting patients to surgery as soon as possible as there is ever lasting danger of embolism.

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
145Staffan Bjessmo et al (1997)(19)in their experience of 63 patients over 40 years,
concluded that surgical resection of a single myxoma is safe and effective treatment, with a low
risk of recurrence. After uncomplicated resection, the frequency with which postoperative
echocardiography is performed, should be limited, except for patients with multiple, atypical, or
familial myxomas, a few transthoracic echocardiographic examination s performed at 5 years’
intervals should be adequate if there has been no recurrence in the first few years.
Laurent Pinede (2001)(20)in his series of 112 consecutive cases of clinical presentation of
left atrial cardiac myxoma, illustrated that this ben ign tumour may cause a wide range of clinical
symptoms not only such as cardiac disease, but also infective, immunologic, or neurloogic
diseases. Diagnosis is rarely made only on clinical grounds, because there are no specific
physical signs or symptoms. Myxomas usually occur sporadically, but familial or recurrent cases
have been reported. Two -dimensional echocardiography including the transesophageal approach
is the technique of choice for diagnosis and follow -up of this tumour. An embolic event in yo ung
adults, in the absence of signs of symptoms of endocarditis or arrhythmia must lead to a primary
consideration of myxoma as embolic source.
Elvira Acebo et al (2003)(21) in their series of clinicopathologic study and DNA analysis
of 37 cardiac myxomas over 28 years showed that villous surface of myxomas predispose to
embolism, and large left atrial tumours are related to atrial fibrillation. Echocardiography is
reliable method with which to predict tumour size and morphology. Myxoma cells usually
express IL -6 and some tumours have abnormal cellular DNA content. Surgical excision of the
tumour is safe and effective treatment.
Pathophysiology
Most myxomas (80 -90%) are in the left atrium. Right atrial myxomas tend to be more
solid and sessil e than left atrial myxomas, with a wider attachment to the atrial wall or septum .(22)
They can be multicentric (within a single chamber) or biatrial. The most common arrangement
(75%) of biatrial tumours involves attachment of two stalks to the opposite side of the same area
of the septum . (23) Most cases are sporadic, approximately 10% are familial and are transmitted
in an autosomal dominant mode. Multiple tumours occur in approximately 50% of familial cases

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
146and are more frequently located in the vent ricle. Myxomas can be polypoid, round or oval. They
are gelatinous with a smooth or lobulated surface and usually are whitish, yellowish, or brown in
colour. The mobility of tumour depends upon the extent of attachment to the inter -atrial septum
and the length of the stalk.
Although atrial myxomas are typically benign, local recurrence due to inadequate
resection or malignant change has been reported. Occasionally, atrial myxomas recur at a distant
site because of intravascular tumour embolization. Th e risk of recurrence is higher in the familial
myxoma syndrome.
Symptoms are produced by mechanical interference with cardiac function or
embolization. Being intra -vascular and friable, myxomas account for most cases of tumour
embolism. Embolism occurs in 30 -40% of patients. The site of embolism is dependent upon the
location (left or right atrium) and the presence of an intracardiac shunt.
Jong -Won Ha and associates (24)reported a more frequent occurrence of systemic
embolism in polypoid tumours as c ompared to round (50% to 60%). Also, polypoid tumours
more frequently prolapse into the ventricle. Prolapse of tumour through the mitral or tricuspid
valve may result in the destruction of the annulus or valve leaflets also. Left atrial myxomas
produce s ymptoms when they reach a weight of about 70gms, right atrial myxomas grow to
approximately twice this size before becoming symptomatic. Tumours vary in size, ranging from
1-15cms in diameter.
Gender
Approximately 75% of sporadic myxomas occur i n females. Female sex predominance is less
pronounced in familial atrial myxomas.
Clinical features
Symptoms range from non -specific to constitutional to sudden cardiac death. In about 20%
cases, myxomas are asymptomatic and are discovered as a n incidental finding. Signs and
symptoms of mitral stenosis, endocarditis, mitral regurgitation and collagen vascular disease can
simulate those of atrial myxomas. A high index of suspicion aids in diagnosis.

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
147Symptoms of left -sided heart failure
– Dyspnea on exertion that may progress to orthopnea, paroxysmal nocturnal dyspnea
and pulmonary edema is observed.
– Symptoms are caused by obstruction at mitral valve orifice. Valve damage may result in
mitral regurgitation.
Sympt oms of right -sided heart failur
– Patients experience fatigue and peripheral edema.
– Abdominal distension due to ascites is rare; however, it is more common in slowly
growing right sided tumours.
-These symptoms are also observed in later stage of progressive heart failure associated
with left atrial myxomas.
Severe dizziness/syncope
– This is experienced by approximately 20% of patients.
– The most frequent cause in patients with left atrial myxomas is obstruction of mitral
valve
– Symptoms may change as the patient changes position.
Symptoms related to embolization
– Systemic or pulmonary embolization may occur from left or right sided tumours.
– Left sided symptoms are produced from the infarction or hemorrhage of viscerae.
– Embolization to central nervous system may result in transient ischemic attack, stroke,
or seizure.
– Involvement of retinal arteries may result in vision loss
– Systemic embolization that causes occlusion of any artery, including coronary, aortic,
renal, visceral or peripheral, may result in the infarction or ischemia of the
correspondin g organ.
– On the right side, embolization results in pulmonary embolism and infarction.
– Multiple, recurrent small emboli may result in pulmonary hypertension and cor –
pulmonale.
– Presence of an intra -cardiac shunt (atrial septal defect or patent foramen oval e) may
result in paradoxical embolism.
Constitutional symptoms
– These include fever, weight loss, arthralgias, and Raynaud’s phenomenon are observed
in 50% of patients. These symptoms may be related to overproduction of interleukin -6.
– Hemoptysis due to pul monary edema or infarction is observed in up to 15% of patients.
– Chest pain is infrequent, if it occurs, it may be due to coronary embolization.
Physical symptoms
– Jugular venous pressure may be elevated, and a prominent A -wave may be present.

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
148- A loud S1 caused by delay in mitral valve closure due to prolapse of the tumour into
mitral valve orifice (Mimicking mitral stenosis).
– P2may be delayed. Its intensity may be normal or increased, depending on presence of
pulmonary hypertension.
– In many patients, early diastolic sound, called a tumour plop is heard. This sound is
produced by the impact of the tumour against the endocardial wall or when its excursion
is halted.
– An S3or S4 may be audible.
– A diastolic atrial rumble may be heard if the tumour is obstructi ng mitral valve.
– If there is valve damage from tumour, mitral regurgitation may cause systolic murmur at
apex.
– Right atrial tumour may cause a diastolic rumble or holosystolic murmur due to tricuspid
regurgitation.
General physical examination
May reveal fever, cyanosis, digital clubbing, rash or Petechiae.
Associated spotty pigmentation, myxomas in breast, skin, thyroid gland or neural tissue may be
present (Carney syndrome).
Laboratory Studies
Laboratory Studies are non -specific and non -diagnostic. If p resent, abnormalities may include
the following: –
– Elevated erythrocyte sedimentation rate (ESR) and elevated C -reactive protein and
serum globulin levels.
– Leucocytosis.
– Anaemia may be normochromic or hypochromic. Hemolytic anaemia may occur
because of the mechanical destruction of erythrocytes by the tumour.
– Serum interleukin -6 level may be raised and can be used as a marker of recurrence.
Imaging studies
Chest radiography may show
– Cardiomegaly
– Abnormal cardiac silhouette, mimicking mitral stenosis.
– Unusu al intracardiac tumour calcification.
– Pulmonary edema
– Biventricular hypertrophy with or without LA enlargement.
Echocardiography

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
149Although transesophageal echocardiography is more sensitive, 2 -dimensional echocardiography
is usually adequate for diagnos is.
All four chambers should be visualized because of multicentricity of tumour.
Transesophageal echocardiography
– It has better specificity and 100% sensitivity compared to transthoracic
echocardiography.
– Has good resolution of both atria and atrial septum and better anatomic details.
– Reveals smaller (1 -3mm in diameter) vegetations or tumour and detects shunting.
– Imaging (MRI)
– It provides useful information about size, shape and surface characteristics.
– Information about tissue composition can be used to di fferentiate a tumour and a
thrombus.
Other tests
ECG may show left atrial enlargement, atrial fibrillation, atrial flutter or conduction disturbances .
Cardiac catheterization
Used in selected patients in case non -invasive evaluation is inadequate.
Used to exclude co -existing coronary artery disease in patients of over 40 years of age.
Histological studies : are characterized by the presence of lipidic cells embedded in myxoid
stoma.
Treatment
No known medical treatment exists for atrial myxomia, drug therap y is used only for
complications such as Congestive Heart Failure or cardiac arrhythmias. Surgical resection of the
myxoma is the treatment of choice, surgery being safe with low morbidity and mortality.
Pericardial/PTFE patch can be used to close the su rgical defect caused by excision of tumour.
The risk of tumour fragmentation and embolization, vigorous palpation or manipulation is
avoided or performed only after cardioplegia. Damaged valve may require repair or replacement.
Recurrence is usually attr ibutable to incomplete excision of tumour, growth from second focus or
intracardiac implantation from primary tumour.

International Journal of Health Sciences Vol. 2 No. 2 July 2008 (Jumad’a Thani 1429 H)
150Conclusion
Myxomas are the most common tumour of the heart. They are frequently located in left
atrium and produce symptoms when they fragment and cause systemic emboli or when they
interfere with cardiac valvular function and cause pulmonary congestion. Careful surgical
management of these lesions should be curative with minimal early and late morbidity and
mortality. Optimal operativ e technique emphasized minimal manipulation of the heart before
institution of cardiopulmonary bypass and aortic cross -clamping and careful examination of
intracardiac chambers with meticulous removal of myxomatous debris. Recurrence of atrial
myxomas can occur most likely in about 3% of patients. However, extensive resection of the
myxoma attached to atrial septum or atrial wall can reduce the likelihood of recurrence to a
greater extent. Thus, long term clinical and echocardiographic follow -up is manda tory.
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