This document provides an overview of atrial myxoma, including its history, pathology, clinical presentation, diagnosis, and surgical management. Some key points: - Atrial myxoma is the most common primary cardiac tumor, usually located in the left atrium and arising from the interatrial septum. - Clinical presentation varies depending on location and size of the tumor but can include signs of heart failure, murmurs, arrhythmias, and embolic events. - Echocardiography is the primary diagnostic tool to determine tumor location, size, and attachment site. - Surgical resection is the only effective treatment due to risk of embolization and hemodynamic complications.

1. ATRIAL
MYXOMA
SURESH KUMAR – MEDICINE 2ND
YEAR

2. HISTORY
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.

3. Treatment of cardiac tumors was profoundly influenced by
two events:
1. The introduction of cardiopulmonary bypass in 1953 by
John Gibbon, which allowed a safe and reproducible
approach to the cardiac chambers
2. The introduction of cardiac echocardiography, which
allowed safe and non invasive diagnosis of an intracardiac
mass.

4. 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

5. INTRODUCTION
Primary tumor of the heart
 Myxomas are one of the most benign neoplasm which
comprise 50%
out of which 15% myxomas are observed in children
 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.

6. 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
 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.

7.  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

8. Relative incidence of benign heart tumors

9. Relative incidence of primary malignant heart tumors

10. PATHOLOGY
 Myxomas are composed of stellate, frequently
multinucleated myxoma cells, admixed with cells showing,
endothelial, smooth muscle and or fibroblastic
differentiation
 The cells are embedded in an abundant acid
mucopolysacharide ground substance
 Hemmorhage, poorly organising thrombus & mononuclear
inflammation also are usually present

12. Clinical Presentation
 Cardiac and non-cardiac manifestations
 Location and size of tumor are the major determinants of
specific signs and symptoms
 Signs & symptoms similar to all form of heart disease:
 Chest pain
 Syncope
 Heart failure
 Murmurs
 Arrhythmias
 Conduction disturbance
 Pericardial effusion or tamponade

13. Myxoma
 Most common type of primary cardiac tumor (1/3
to ½ of all cases)
 Most commonly in 3rd
– 6th
decade; female > male
 Sporadic vs familial
 Majority sporadic; some are familial (autosomal
dominant transmission) or part of a syndrome
1. Carney complex – spotty skin pigmentation,
myxomas, endocrine overactivity, schwannomas
2. NAME syndrome – nevi, atrial myxoma, myxoid
neurofibroma, ephelides
3. LAMB syndrome – lentigines, atrial myxoma, blue
nevi

14. Myxoma
Sporadic Familial or Syndrome Myxoma
• Solitary
• More common
• Usually located in left atria
• Arise from inter-atrial septum in
vicinity of fossa ovalis
• May also occur in the ventricles
or multiple locations
• Younger individual
• Often multiple location
• Less common (10%)
• Autosomal dominant pattern of
transmission
• Associated with freckling, non-
cardiac tumors, endocrine
neoplasms
• Recurrent after surgery

15.  These benign masses are most often attached to the atrial wall,
but can arise on a valve or in a ventricle
They can produce a "ball valve" effect by intermittently
occluding the atrioventricular valve orifice. Embolization of
fragments of tumor may also occur
Myxomas are easily diagnosed by echocardiography

16. Myxoma – Symptoms and Signs
Symptoms Incidence (%)
Dyspnea on exertion
Paroxysmal dyspnea
Fever
Weight loss
Severe dizziness/syncope
Sudden death
Hemoptysis
>75
~25
~50
~25
~20
~15
~15

17. Myxoma – Symptoms and Signs
Signs Incidence (%)
Mitral diastolic murmur
Mitral systolic murmur
Pulmonary hypertension
Right heart failure
Pulmonary emboli
Anemia
Elevated ESR
Third heart sound (tumor plop)
Atrial fibrillation
Elevated globulins
Clubbing
Raynaud’s phenomenon
~75
~50
~70
~70
~25
>33
>33
>33
~15
~10
~5
<5

18. CLINICAL PRESENTATION
 Systemic or cardiovascular findings
 Cardiovascular findings:
1. Atrial
 resemble mitral valve disease  most
common clinical presentation
 Stenosis – tumor prolapse into the
mitral orifice during diastole
 Regurgitation – injury to the valve by
tumor-induced trauma
2. Ventricular – outflow obstruction  syncope

19. DIAGNOSIS
1. Two-dimensional transthoracic or trans-esophageal
echocardiography
• Determine site of tumor attachment and tumor
size
• Screening of 1st
degree relatives for familial or
syndrome myxoma
2. CT scan and MRI
• Tumor size, shape, composition, and surface
characteristics
3. Cardiac catheterization
• Risk of tumor emboli for suspected CAD

20. Myxoma
TEE showing a large mass (M), in the left atrium with
attachment to interatrial septum and prolapsing through the
mitral valve into the left ventricular cavity in diastole. (M =
myxoma).

23. SURGICAL
MANAGEMENT
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

24. THANK YOU