Intracardiac Myxoma: Effect of Tumour Morphology
On Clinical Presentation and Surgical Intervention
Title:
Authors: Ahmad K. Darwazah (MD, FRCS) 1, Wael Abdel Aziz (MD) 2 Mohammed El
Awady (MD) 3
1
Cardiothoracic surgery department, Menofia faculty of medicine, Menofia
University, Egypt.
2
Cardiothoracic surgery department, Mansoura faculty of medicine, Mansoura
University, Egypt.
3
Cardiothoracic surgery department, Banha faculty of medicine, Banha University,
Egypt.
Editor: Dr. Mohamed Ahmed Elawady, MD
E-mail: mohamedawady@yahoo.com
Telephone: +966599276628
Fax number: +96638152692
Keywords: Intra
cardiac ,Myxoma.
1
Intracardiac Myxoma: Effect of Tumour Morphology on Clinical
Presentation and Surgical Intervention
Ahmad K. Darwazah (MD, FRCS) 1, Wael Abdel Aziz (MD) 2 Mohammed El Awady
(MD) 3
Background: Presentation of cardiac myxomas is quite variable and is affected by the site,
size, and mobility of these tumours. This study was conducted to evaluate the relationship
between the morphology of resected tumours and their clinical presentation and whether they
have any influence on the decision of surgery.
Patients and Methods : Seventeen patients were operated upon for cardiac myxomas over a
period of seven years (2003-2010). The majority of patients were females (65%). The age at
presentation varied from 18-68 years (mean 42±13.8). The majority of tumours were located
in the left atrium (n = 11). Other sites were the right atrium (n = 5) and the left ventricle (n =
1). The morphology of the resected tumours was evaluated regarding their size; number,
shape and mobility.
Results: The majority of our patients represented with cardiac symptoms (59%), followed by
constitutional manifestations (53%) and embolic episodes (12%). Preoperative diagnosis was
established in 88% of cases by transthoracic echocardiography. The commonest site of
attachment of tumours was interatrial septum (53%), while the remaining tumour originated
from atrial wall, appendage and IVC. All tumours were solitary except the one originating
from the left ventricle. 71% of tumours were rounded in shape and the rest were
pedunculated. The size of tumors varied from 1.8-10 cm X 1-6 cm. All pedunculated tumors
were symptomatic. On the other hand 3 patients who had rounded tumours were
asymptomatic. Surgical excision was performed electively once the diagnosis was made,
except in 6 patients who underwent emergency operation. Associated procedures in the form
of tricuspid valve repair, mitral valve replacement, pulmonary embolectomy and CABG were
performed in association with tumour excision.
Conclusions:
Presentation of cardiac myxomas is greatly affected by their morphology. This
explains their variable symptomatology. In some cases, their diagnosis is missed because
they are asymptomatic or masked by other associated lesions. Tumours originating from
unusual sites and large tumours may necessitate modification of either bypass technique or
surgical approach.
2
Introduction
Primary cardiac tumours are uncommon and represent 5-10% of all neoplasms of the
heart and pericardium (1). 80% of these tumours are benign, and more than half of these are
myxomas(2).
The presentation of myxomas is typically variable (3). It is determined by their
location, size and mobility. Most cases are either presented with one or more of the triad of
intracardiac obstruction, embolism and constitutional symptoms. In small tumours, patients
are often asymptomatic (4). In this study, we review our experience with seventeen cases
operated upon for excision of myxomas during a period of 7 years. Patients were evaluated
regarding their tumour morphology to find out the effects on clinical presentation and
surgical intervention.
Patients and Methods
Between 2003 and 2010, 17 patients underwent surgical excision of intracardiac
myxomas. Patients profile is summarised in Table 1. The majority of cases were females. The
age at presentation varied from 18 to 68 years with a mean of 42 ± 13.8. There were 11 left
atrial myxomas (65%), 5 right atrial myxomas (29%) and one tumour originated from the left
ventricle (6%). The majority of patients presented with cardiac symptoms followed by
constitutional and embolic episodes. Three patients (18%) were asymptomatic.
Physical examination revealed the presence of mitral stenosis in 7 patients (41%) and
combined mitral stenosis and incompetence in 2 patients (12%). Four patients with right
atrial myxoma had tricuspid regurgitation. Evidence of right sided heart failure was seen in 3
patients (18%). In two patients, the tumour was discovered accidently during preoperative
evaluation for coronary artery disease and preparation for gastric banding operation. In one
patient, the tumour was discovered intraoperatively during mitral valve surgery. The
diagnosis of the tumour was missed in one patient at the initial presentation of pulmonary
embolism and was discovered two months later during follow up. Preoperative diagnosis was
established in 15 patients by TTE echocardiography. One patient was diagnosed by
Transoesophageal echocardiography and confirmed by MRI. Cardiac catheterisation was
performed in 8 patients because of age or associated anginal pain. Associated coronary artery
disease was seen in one patient, rheumatic mitral stenosis in another and variable degree of
tricuspid regurgitation was seen in 4 patients. Pulmonary artery pressure was elevated in two
patients with left atrial tumours.
Surgical technique:
Excision of tumours was performed through median sternotomy using cardiopulmonary
bypass with moderate hypothermia (32-34˚C), topical cooling and antegrade crystalloid
cardioplegia to protect the myocardium. Manipulation of the heart was avoided for fear of
tumour fragmentation and systemic embolization. The approach used to resect the tumours
was primarily based on the site of the myxoma. Left atrial approach was used in left atrial
tumours in the majority of cases.
However, in 4 patients a biatrial approach was used due to difficulty to remove the
tumours. Right atrial tumours were resected through right atriotomy, while left
ventriculotomy was used to remove the left ventricular tumour. The site of attachment of
3
tumour was cauterized. Careful exploration of cardiac chambers was performed to exclude
multiple tumours. All excised tumours underwent histopathological examination.
Results
The majority of cases underwent elective surgery once the diagnosis was made (Table
2). Surgery was undertaken on emergency bases in 6 patients (35%) due to the presence of
severe and serious symptoms in the form of syncopal attacks, pulmonary embolism and left
axillary artery occlusion. The resected tumours morphology is summarized in Table 3. All
resected tumours were single except the tumour originating from the left ventricle. The
largest and smallest tumour was found in the right atrium. The shape of tumours was either
rounded or polypoid (Table 4). Tumours arising from the left atrium were mainly rounded in
shape, their size varied from 2-9 x 1-6 cm. The majority originated from the atrial septum
(73%). In two patients, the tumour appeared polypoid crossing the mitral valve down to mid
cavity of left ventricle.
Tumours of the right atrium originated from the interatrial septum, atrial wall and right
atrial appendage. Their sizes varied from 1.8-10 x 1-5 cm. The majority were polypoid (60%)
and found crossing the tricuspid valve. Two rare cases were found attached at the junction of
IVC and right atrium.
Only one patient (6%) had multiple tumours arising from the left ventricle. The main
bulk was attached by a short stalk to interventricular septum near the apex. Another two
small lesions were found attached to the nearby trabeculae of the left ventricular cavity. The
mass was rounded in shape 3.7 x 2.0 cm in size. Its surface was covered by a thrombus.
In 4 patients, associated procedures (Table 2) were performed. Out of the 4 patients
who had tricuspid regurgitation, only one had tricuspid repair due to severe incompetence
using DeVaga’s annuloplasty. One patient with right atrial myxoma had associated
pulmonary embolism. Removal of clots from pulmonary artery was performed by open
pulmonary embolectomy. Two patients with left atrial tumours had associated procedures,
one patient had mitral valve replacement for rheumatic calcific mitral stenosis using
mechanical valve (Sorin 27). The other patient had coronary artery bypass surgery to graft
LAD, PDA and obtuse marginal arteries using both LIMA and saphenous vein grafts.
Complete resection of tumours was performed. The base of attachment was cauterised except
in 4 cases with left atrial tumours, by which a biatrial approach was done with excision of
part of inter-atrial septum. The remaining septal defect was repaired using pericardial patch.
(Table 5).
Table 1. Presentation of 17 Patients with intracardiac Myxoma.
Presentation
Number of patients/
Percentage
Cardiac Symptoms
Dyspnea
10(59%)
Palpitation
2 (12%)
Angina
3 (18%)
CHF
3 (18%)
Syncope
4 (24%)
Hemoptysis
1 (6%)
4
Embolic Symptoms
CNS
1 (6%)
Lt axillary artery
1 (6%)
Pulmonary artery
1 (6%)
Constitutional Symptoms
9 (53%)
Asymptomatic
3 (18%)
Signs
Diastolic murmur
7 (41%)
Systolic mumur
4 (24%)
Combined murmur
2 (12%)
No murmur
4 (24%)
Hepatomegally
3 (18%)
Peripheral oedema
3 (18%)
Associated lesions
CAD
1 (6%)
Rhc MS
1 (6%)
CHF: Congestive Heart Failure; CNS: Central Nervous System;
CAD = coronary artery disease; Rhc MS: rheumatic mitral stenosis
All patients survived surgery. Hospital mortality was 6%. One patient died of
respiratory failure; he had a difficult intubation during which the trachea was injured which
necessitated tracheostomy before surgical intervension. The patient was ventilated for three
weeks during which he developed severe mediastinitis. Re-exploration was performed in one
patient due to postoperative bleeding. Two patients (12%) had postoperative atrial fibrillation
to which Amiodarone (Cordarone) infusion was given. First degree heart block was seen in
one patient, which was recovered spontaneously. Postoperative renal impairment was seen in
one patient with right atrial myxoma. Recovery was obtained after 5 days with dopamine
infusion.
Histopathological examination of all resected tumours revealed a myxoid matrix with
scattered polygonal cells typical of myxoma.
Table 2 Surgical Data
Elective operation
Emergency operation
Approach
Right Atriotomy
Left Atriotomy
Biatrial
Left ventriculotomy
Associated procedures
Tricuspid valve repair
Number of patients
/percentage
11 (65%)
6 (35%)
5 (29%)
7 (41%)
4 (24%)
1 (6%)
1 (6%)
5
Mitral valve replacement
Pulmonary Embolectomy
CABG
CPB Time (min)
Cross Clamping time (min)
1 (6%)
1 (6%)
1 (6%)
60 ± 23
42 ± 20
Discussion
Myxoma is the most common primary cardiac tumour. It occurs in all age groups,
frequently between the third and sixth decades of life (4), commonly affecting women. These
tumours arise from mesenchymal cardiomyocyte progenitor cells (5). Some cases are
associated with herpes simplex virus type I. Their antigens produce both interleukin 6 and
vascular endothelial growth factors (6), both are responsible for constitutional symptoms and
angiogenesis which are prominent features of myxoma(7).
Commonly, these tumours arise in the atrium. 75% of cases originate in the left atrium,
while 15-20% in the right atrium (4). The ventricles are the least to be affected. Rare cases
may arise from cardiac valves and pulmonary artery, veins and vena cava (8).
As in previous studies, the majority of our patients had myxoma arising from the left
atrium, few cases originated from the right atrium and left ventricle.
Tumours arising from the left atrium were found attached to the interatrial septum in the
majority of cases, which is similar to previously published cases (9). Those arising from the
right atrium, their site of attachment were equally distributed between the interatrial septum,
atrial wall and appendage. Two rare cases of right atrial myxoma in our series were found
near inferior vena cava. One originating at the superio-anterior junction of IVC with the right
atrium and one originated from the supra hepatic IVC.
Table 3:Morphology of Resected Tumours
Number of patients/percentage
Site
Left Atrium
11 (65%)
Septum
8 (73%)
Wall
2 (18%)
Appendage
1 (9%)
Right Atrium
5 (29%)
Septum
1 (20%)
Wall
1 (20%)
Appendage
1 (20%)
IVC
2 (40%)
Left Ventricle
1 (6%)
Shape
Rounded
Polypoid
Number
Single
Multiple
12 (71%)
5 (29%)
16 (94%)
1 (6%)
6
Size
Length
Width
Consistency
Firm
Soft (gelatinous)
1.8-10cm (mean) 5.3±2.6
1-6cm (mean) 2.9±1.5
12 (71%)
5 (29%)
The other rare case in our series originated from the left ventricle. Usually, these
tumours constitute about 3-5% of myxomas(10). Commonly, they are solitary and arise near
the posterior papillary muscle (11). The tumour in our case originated from the interventricular
septum and was multiple.
The majority of myxomas are polypoid, often pedunculated in shape with an
asymmetrical soft and mobile surface. Rarely, they appear solid, round in shape with nonmobile surface (12). Contrary to the above findings, the majority of our tumours appeared solid
and rounded, only few cases were polypoid and soft. We found a direct relation between
symptomatology and the shape of the tumours. Both rounded and polypoid tumours presented
with variable symptoms and signs. It was noticed that polypoid (pedunculated) tumours
prolapsing either through mitral or tricuspid valves produce early and serious symptoms as
syncope which necessitated emergency operation. On the other hand, three cases of solid
rounded tumours were asymptomatic.
.
Table 4:Symptoms in relation to the type of tumour
Polypoid
Roudned
Number of cases
5
12
Gender (F/M)
3/2
8/4
Location (L/R)
2/3
10/2
Heart Failure
2
1
Constitutional symptoms
4
5
Embolic episodes
–
2
Syncope
3
1
Asymptomatic
–
3
F/M; Female/male, L/R; Left including atrium and ventricle/Right
Table 5: Different approaches for excision of left atrial myxoma in relation to size,
attachment and pedicle.
Case
Number
1
2
3
Shape
Rounded
Rounded
Pedunculated
Site of
attachment
Septum
Wall
Septum
Size
(cm)
2x3
4x2
8x2
7
Pedicle/attachment
Short, wide attachment
Short, narrow attachment
long, narrow attachment
Excision
Approach
Biatrial
Left atriotomy
Left atriotomy
4
5
6
7
8
9
10
11
Rounded
Roudned
Roudned
Roudned
Roudned
Peduncualted
Rounded
Rounded
Appendage
Septum
Septum
Septum
Septum
Post wall
Septum
Septum
3x2
3x1
3x2
3x3
5x4
9x2
4x3
6x5.5
short, narrow attachment
No pedicle, wide attachment
Short, narrow attachment
Short, narrow attachment
Short, wide attachment
Long pedicle,narrow attachment
Short, narrow attachment
No pedicle, wide
attachment
Left Atriotomy
Biatrial
Left atriotomy
Left atriotomy
Bi atrial
Left Atriotomy
Left atriotomy
Inverted T-shaped
biatrial Incision
The mobility of pedunculated tumours and it’s back and forth movement across either
mitral or tricuspid valves can interfere with valve closure or damage the valve structure(4).
This was clearly seen among our few pedunculated tumours which caused either tricuspid or
mitral regurgitation. The effect on mitral valve was mild in comparison to tricuspid valve.
This was directly related to the size of tumours. Those arising from the right atrium were
larger than left atrium. Subsequently, the effect on tricuspid valve was more profound. Non
of the incompetent mitral valves needed repair. On the other had one case of tricuspid
regurgitation needed repair.
Presentation of myxoma is typically variable (3). The majority of cases often present
with one or more of triad of intracardiac obstruction of A–V valves, embolism and
constitutional symptoms(4). Asymptomatic cases and those discovered intraopertively have
also been reported(3). The commonest presentation in our cases was cardiac symptoms
followed by constitutional manifestation and embolic episodes. From our study, we noticed
that the patients symptoms were related to the shape, size and site of attachment of tumours.
Those originating from the left atrium presented mainly by dyspnea due to obstruction
of the mitral valve. Most of the tumours were solid, rounded masses. On the other hand, those
originating from the right atrium presented with heart failure and syncope. They were large in
size and pedunculated prolapsing through and obstructing tricuspid valve.
Constitutional symptoms in the form of fever, loss of weight, rash, arthralgia, myalgia
and laboratory abnormalities as anemia, and elevation of erythrocyte sedimentation rate, creactive protein and  globulin levels are often seen in 65% of cases(13). These symptoms are
caused by immunological response to interleukin 6 and  globulin (14). Almost half of our
patients had one or more of these non-specific symptoms and laboratory abnormalities.
Previous studies found that these constitutional symptoms are observed irrespective of the
site and size of the tumour (10, 15, 16). This could be true regarding the size of the tumour, but
we noticed that all patients with right atrial myxoma had these symptoms in comparison to
only 36% of patients with left atrial masses.
Embolic manifestations associated with cardiac myxomas are common. The incidence
may reach 30-40%. Embolism is usually caused by either necrotic tumour fragments or
thrombi from the surface of the tumour (17,18). Emboli arising from myxomas have a special
predilection to the brain, but other organs as the liver, spleen, kidney, retina, coronary and
peripheral arteries are also involved(17,18). The incidence of embolism among our patients was
12%. The two patients were young in their thirties. The tumours were located in the right
atrium and left ventricle and they were rounded with rough surface.
8
Embolectomy of pulmonary artery revealed blood clots, there was no evidence of
tumour fragments. This is easily explained by the solid nature of the tumour and its rough
surface which potentiates the formation of thrombus. It is worth mentioning that patients
presented with embolization, had a delay in diagnosis of their tumours. So, it is important to
emphasize that young patients presented with either peripheral or central embolization should
be suspected to have myxoma and investigated thouroughly to exclude the presence of these
tumours.
The majority of cardiac myxomas can be diagnosed by TTE transthoracic
echocardiography. Small sized tumours can be identified by either CT or MRI (19) which can
also differentiate tissue composition. The importance of TTE as a diagnostic tool was clearly
seen in our present work. 88% of patients were diagnosed by TTE. In the remaining patients,
transoesophageal echocardiography was used to diagnose a small tumour arising from IVC
while in the remaining patient the diagnoses was missed and discovered during operation.
In our study the relationship between tumour morphology and its influence on decision
and conduct of operative strategy was observed among few patients. The surgical approach,
bypass technique and urgency of operation were influenced greatly by site, size and
presenting symptoms.
Surgical excision of myxoma is usually performed directly through the involved cardiac
chamber. In our study, we noticed that the approach of excision of left atrial myxoma was
related to the size, attachment base and pedicle of the mass. Those tumours with long and
short pedicle regardless of the size were removed by left atriotomy approach. On the other
hand, bi-atrial incision was used for tumours with wide attachment with no pedicle. An
inverted-T shaped transeptal incision was used in large tumours with wide attachment.
Emergency surgical excision was directly related to presenting symptoms. Life
threatening syncopal attacks and embolization were the main indication of emergency
operation among our group of patients. It was noticed that syncopal attacks were commonly
seen in pedunculated tumours, while embolization was only seen in rounded masses.
Surgical excision of myxomas is usually performed under CPB with moderate
hypothermia. Minimal manipulation, proper exposure and complete excision are always
needed. Modification of bypass technique was needed in our study in one patient with
myxoma originating from IVC. Both systemic temperature and flow were reduced to allow
removal of IVC cannula in order to expose and resect the tumour completely.
In conclusion, morphology of cardiac myxomas greatly affects their presentation. Both
pedunculated and large sized tumours often produce early symptoms and are associated with
valvular involvement. On the other hand, rounded tumours present late unless they obstruct a
nearby valvular apparatus.
Tumours originating from unusual sites and large sized masses may necessitate
modification of either bypass technique or surgical approach.
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9
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