A clinical study on Atrial Myxoma and It's management is in progress.

1. By : DR NABAJYOTI HAZARIKA
CTVS Deptt., Gauhati Medical College & Hospital,
Guwahati, Assam, India
ATRIAL
MYXOMAS

2.  Myxomas are one of the most benign
neoplasm which comprise 50%
 out of which 15% myxomas are
observed in children

3.  5% of myxoma patients show a familial pattern of
tumor development based on autosomal dominant
inheritance. These patients and 20% of those with
sporadic myxoma have an abnormal DNA
genotype chromosomal pattern.
 In contrast to the “typical” sporadic myxoma
profile, familial patients are more likely to be
younger, equally likely to be male or female, and
more often (22%) have multicentric tumors
originating from either the atrium or ventricle.

4.  Familial myxomas have the same histology, they have a
higher recurrence rate after surgical resection (21 to
67%). Approximately 20% of familial patients have
associated conditions such as adrenocortical nodule
hyperplasia, Sertoli cell tumors of the testes, pituitary
tumors, multiple myxoid breast fibroadenomas,
cutaneous myomas, and facial or labial pigmented
spots. These conditions often are described as complex
myxomas within the group of familial myxoma. A
familial syndrome with autosomal X-linked inheritance
characterized by primary pigmented nodular
adrenocortical disease with hypercortisolism, cutaneous
pigmentous lentigines, and cardiac myxoma is referred
to as Carney’s complex.

5.  A series of six atrial tumors, with characteristics we now recognize as
myxoma, was published in 1845 by King.
 The first echocardiographic diagnosis of an intracardiac tumor was made
in 1959.
 An intracardiac myxoma was diagnosed by angiography in 1952 by
Goldberg, but attempts at surgical removal were unsuccessful.
 Treatment of cardiac tumors was profoundly influenced by two events: the
introduction of cardiopulmonary bypass in 1953 by John Gibbon, which
allowed a safe and reproducible approach to the cardiac chambers, and the
introduction of cardiac echocardiography, which allowed safe and
noninvasive diagnosis of an intracardiac mass.

6.  A large right atrial myxoma was removed by Bhanson
in 1952 using caval inflow occlusion, but the patient
died 24 days later.
 Crafoord in Sweden first successfully removed a left
atrial myxoma in 1954 using cardiopulmonary bypass,
and Kay in Los Angeles first removed a left ventricular
myxoma in 1959.
 By 1964, 60 atrial myxomas had been removed
successfully, with improved results owing to the
increasing safety of cardiopulmonary bypass and use of
echocardiography for detection.

7. *familial syndrome with autosomal X-linked inheritance
characterized by primary pigmented nodular
adrenocortical disease with hypercortisolism, cutaneous
pigmentous lentigines, and cardiac myxoma is referred
to as Carney’s complex.

8.  Both biatrial and multicentric myxomas are more
common in familial disease. Biatrial tumors probably
arise from bidirectional growth of a tumor originating
within the atrial septum. Atrial myxomas generally
arise from the interatrial septum at the border of the
fossa ovalis but can originate anywhere within the
atrium, including the appendage.
 In addition, isolated reports confirm that myxomas can
arise from the cardiac valves, pulmonary artery and
vein, and vena cava.

9.  Right atrial myxomas are more likely to have
broad-based attachments than left atrial tumors;
they also are more likely to be calcified, and thus
visible on chest radiographs.
 Ventricular myxomas occur more often in women
and children and may be multicentric. Right
ventricular tumors typically arise from the free
wall, and left ventricular tumors tend to originate
in the proximity of the posterior papillary muscle.

10.  two-thirds of myxomas are round or oval tumors
with a smooth or lobulated surface. Most are
polypoid, compact, pedunculated, mobile, and not
likely to fragment spontaneously.
 Less common villous or papillary myxomas are
gelatinous and fragile and prone to fragmentation
and embolization, occurring about one-third of
the time.

11.  Myxomas are
white,
yellow, or
brown in color, and
frequently covered with thrombus.
Focal areas of hemorrhage,
cyst formation, or
necrosis

12.  The average size is about 5 cm in diameter, but
growth to 15 cm in diameter and larger has been
reported.
 Myxomatous tumors appear to grow rapidly, but
growth rates vary, and occasionally, tumor growth
arrests spontaneously. Weights range from 8 to
175 g, with a mean between 50 and 60 g.

13.  Histologically, myxomas are composed of
polygonal-shaped cells and capillary channels
within an acid mucopolysaccharide matrix. The
cells appear singularly or in small clusters
throughout the matrix, and mitoses are rare.
 Ten percent of the tumors have microscopic
deposits of calcium and metastatic bone deposits,
as well as sometimes glandular-like structure.

15.  Myxomas arise from the endocardium and are
considered derivative of the subendocardial
multipotential mesenchymal cell.
 Occasional presence of hematopoietic tissue and
bone in these tumors. Interestingly, myxomas
have developed after cardiac trauma, including
repair of atrial septal defects and trans-septal
puncture for percutaneous dilatation of the mitral
valve.

16.  The classic clinical presentation of a myxoma is
intracardiac obstruction with congestive heart
failure (67%);
 Signs of embolization (29%); systemic or
constitutional symptoms of fever (19%); weight
loss or fatigue (17%);
 Immunologic manifestations of myalgia,
weakness,
 Arthralgia (5%). Cardiac rhythm disturbances and
infection occur less frequently.

17.  Nearly all myxoma patients admit to a variety of constitutional symptoms.
 These symptoms may be accompanied by
weakness of one side of the body,
bylateral weakness,
stroke on evolution or stroke,
a leukocytosis,
elevated erythrocyte levels and sedimentation rate,
hemolytic anemia,
thrombocytopenia,
elevated C-reactive protein.
Immunoelectrophoresis may reveal abnormal immunoglobulin levels
with increased circulating IgG.
elevated levels of interleukin-6

18.  associated conditions are -
including lymphadenopathy,
tumor metastasis,
ventricular hypertrophy,
development of constitutional symptoms.
Other less frequent complaints include
Raynaud’s
phenomenon,
arthralgias, myalgias,
erythematous rash,
clubbing of the digits.

19.  Possible etiologies of such varied complaints and
symptoms include -
tumor embolization with secondary myalgias
arthralgias,
elevated immunoglobulin response.
Circulating antibody–tumor antigen
complexes with complement activation also
may play a role.

20.  Obstruction of blood flow in the heart is the most common cause
of acute presenting symptoms. The nature of these symptoms is
determined by which of the chambers is involved and the size of
the tumor.
 Myxomas in the left atrium tend to mimic mitral disease. These
produce positional dyspnea and other signs and symptoms of
heart failure associated with elevated left atrial and pulmonary
venous pressures.
 Clinically, mitral stenosis often is suspected and leads to
echocardiography and diagnosis of myxoma. Syncopal episodes
occur in some patients and are thought to result from temporary
occlusion of the mitral orifice.

21.  Right atrial myxomas can produce a clinical
picture of right-sided heart failure with signs and
symptoms of venous hypertension, including
hepatomegaly, ascites, and dependent edema and
can cause tricuspid valve stenosis by partially
obstructing the orifice.
 If a patent foramen ovale is present, right-to-left
atrial shunting may occur with central cyanosis,
and paradoxical embolization has been reported.

22.  Systemic embolization is the second most common mode of
myxomatous presentation, occurring in 30 to 40%
 Because the majority of myxomas are leftsided, approximately
50% of embolic episodes affect the central nervous system owing
to both intra and extracranial vascular obstruction.
 Neurologic deficits may present as –
seizures,
hemiparesis, and
brain necrosis.

23.  Retinal artery embolization with visual loss has
occurred in some patients.
 Embolic myxomatous material may cause –
blocking iliac,
femoral arteries, and
sometimes visceras also
 Right-sided myxomatous emboli mainly obstruct
pulmonary arteries and cause pulmonary
hypertension and even death from acute
obstruction.

24.  Infection arising in a myxoma is a rare
 If occurs urgent surgical intervention is required

25.  Clinical examination
 “tumor plop” and unpredictable sign and
symptoms during auscultation apart from other
general examinations which includes physical
examination etc.
 Chest Radiograph and Electrocardiogram

26.  Nonspecific abnormalities such as chamber
enlargement, cardiomegaly, bundle-branch
blocks, and axis deviation can be found. Fewer
than 20% of patients have atrial fibrillation.
Evaluation of nonspecific electrocardiographic
abnormalities occasionally leads to an incidental
diagnosis of myxoma most electrocardiograms
are not helpful in establishing a diagnosis.

27.  Cross-sectional echocardiography is the most
useful test employed for the diagnosis and
evaluation of myxoma. The sensitivity of two-
dimensional (2-D) echocardiography for myxoma
is 100%.
 this imaging technique largely has supplanted
angiocardiography.
 coronary angiography.
 Transesophageal echocardiography (TEE)
 CT SCAN and MRI

29.  Surgical resection is the only effective therapeutic option
for patients with cardiac myxoma and should not be delayed
because death from obstruction to flow within the heart or
embolization may occur in as many as 8% of patients
awaiting operation.
 A median sternotomy approach with ascending aortic and
bicaval cannulation usually is employed.
 Manipulation of the heart before initiation of
cardiopulmonary bypass is minimized in deference to the
known friability and embolic tendency of myxomas.
 CPB management.

36. 1. Smith C: Tumors of the heart. Arch Pathol Lab Med 1986; 110:371.
2. McAllister HA, Fenoglio JJ Jr: Tumors of the cardiovascular system,
in Atlas of Tumor Pathology, Series 2. Washington, DC, Armed
Forces Institute of Pathology, 1978.
3. Straus R, Merliss R: Primary tumors of the heart. Arch Pathol 1945;
39:74.
4. Reynen K: Cardiac myxomas. N Engl J Med 1995; 333:1610.
5. Wold LE, Lie JT: Cardiac myxomas: a clinicopathologic profile. Am
J Pathol 1980; 101:219.
6. Silverman NA: Primary cardiac tumors. Ann Surg 1980; 91:127.

52. Ever Green Landscape of Assam, India
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