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Cardiac Tumors

Moises M. Bartolome III, MD, FPCP


Department of Medicine
Our Lady of Fatima University
Primary tumors of the heart RARE (.0017 -
.28%)

Often BENIGN (75%)

Potential for life-threatening complication

Curable by surgery
Relative incidence of benign heart tumors

% of Group
TUMOR Adults Children Infants
Myxoma 46 15 0
Lipoma 21 0 0
Papillary fibroelastoma 16 0 0
Rhabdomyoma 2 46 65
Fibroma 3 15 12
Hemangioma 5 5 4
Teratoma 1 13 18
Mesothelioma of AV node 3 4 2
Granular cell tumor 1 0 0
Neurofibroma 1 1 0
Lymphangioma 1 0 0
Hamartoma 0 1 0
Relative incidence of primary malignant heart tumors

% of Group
TUMOR TYPE Adults Children Infants
Angiosarcoma 33 0 0
Rhabdomyosarcoma 21 33 66
Mesothelioma 16 0 0
Fibrosarcoma 11 11 33
Malignant lymphoma 6 0 0
Extraskeletal osteosarcoma 4 0 0
Thymoma 3 0 0
Neurogeic sarcoma 3 11 0
Leiomyosarcoma 1 0 0
Liposarcoma 1 0 0
Synovial sarcoma 1 0 0
Malignant teratoma 0 44 0
Clinical Presentation

Cardiac and non-cardiac manifestations

Location and size of tumor 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
Myxoma

Most common type of primary cardiac tumor (1/3


to 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
Myxoma

Sporadic Familial or Syndrome


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

The left atrium has been opened to


reveal the most common primary
cardiac neoplasm--an atrial
myxoma. 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.
Myxoma Symptoms and Signs

Symptoms Incidence (%)


Dyspnea on exertion >75
Paroxysmal dyspnea ~25
Fever ~50
Weight loss ~25
Severe dizziness/syncope ~20
Sudden death ~15
Hemoptysis ~15
Myxoma Symptoms and Signs

Signs Incidence (%)


Mitral diastolic murmur ~75
Mitral systolic murmur ~50
Pulmonary hypertension ~70
Right heart failure ~70
Pulmonary emboli ~25
Anemia >33
Elevated ESR >33
Third heart sound (tumor plop) >33
Atrial fibrillation ~15
Elevated globulins ~10
Clubbing ~5
Raynauds phenomenon <5
Myxoma

Clinical Presentation

Systemic or cardiovascular findings


Cardiovascular findings:
1. Atrial
s/sx 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
Myxoma

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
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).
Rhabdomyomas

Most common in infants and children (75%


< 1 y/o)

Most common in ventricles s/sx due to


mechanical obstruction mimic valvular
stenosis, CHF, restrictive or hypertrophic
cardiomyopathy, & pericardial constriction

Multiple in 90% of cases

May be associated with tuberous sclerosis,


adenoma sebaceum, benign kidney tumors
Rhabdomyomas
Cardiac Lipomas

2nd most common benign tumor

Usually incidental post mortem findings

Usually solitary; grow as large as 15 cm

Clinical:
Symptoms due to mechanical
interference with cardiac function
Arrhythmias
Conduction disturbances
Abnormality of cardiac silhouette on CXR
Cardiac Lipomas

If subepicardial
Compression of the heart
Pericardial effusion

If subendocardial
With intracavitary extension, may
produce symptoms characteristic of their
location

Most common chambers affected: LV, RA,


IAS
Cardiac Lipomas

Coronal turbo fast low-angle


shot (FLASH) MRI scan of a
patient with a right atrial
lipoma shows a high-signal-
intensity mass (L) in the
lateral wall of the right
atrium. High signal intensity
on T1 imaging is strongly
suggestive of fatty tissue
and identifies this mass as a
lipoma.
Fibromas

2nd most common in pediatric age group

Benign connective tissue tumor

Associated with calcification

Occur in the ventricle and IVS

s/sx secondary to mechanical interference


with cardiac flow, ventricular contraction
abnormalities, conduction disturbance
Fibromas
Papillary Fibroelastoma

Common findings on cardiac valves or the


adjacent endothelium at post-mortem

Seldom result in clinical manifestations

Growth may cause mechanical interference


with valve function

Found in elderly population (mean age 60


y/o)
Papillary Fibroelastoma
Hemangiomas and Mesotheliomas

Generally small tumors

Most often intra-myocardial in location

May cause atrioventricular (AV) conduction


disturbances and sudden death due to
predilection for region of AV node
Hemangiomas and Mesotheliomas

Surgical exploration showed


lobulating tumor originating in
right atrial wall. White arrows
indicate tumor and white
arrow head indicates right
atrial wall (A). Gross specimen
of the intracardiac tumor was
shiny and lobulating oval
shaped with hemorrhagic
component in it (B)
Sarcomas

Most common malignant tumor; 2nd most


common primary tumor of heart

Common in male (3:1)

Characterized by rapidly downhill course leading


to patients death weeks to months from time of
presentation due to:
1. Hemodynamic compromise
2. Local invasion
3. Distant metastases
Sarcomas

Histologic types:
Angiosarcomas most common
Rhabdomyosarcoma
Fibrosarcoma
Osteosarcoma

Characterized by rapid growth

At presentation, often spread extensively for


surgical excision

Commonly involve RA & pericardium right-


sided failure, pericardial disease, vena cava
obstruction
Sarcomas

May occur in left side mistaken for


myxoma

Treatment & Prognosis:

Surgery not effective to establish


diagnosis
Occurrence of distant metastases
Sarcomas

Gross specimen with opened left ventricle and multiple


nodules of a sarcoma (*) mimicking myocardial hypertrophy
Cardiac Metastases
( Tumors Metastatic to the Heart)
40x more common than primary tumors

Occurs in 1-20% of all tumor types

Malignant melanoma highest predilection


for cardiac metastasis (50-65%)

Most common from breast and lung CA

Almost always occur in the setting of


widespread primary disease

May be the initial presentation of tumor


elsewhere
Cardiac Metastases

Reach the heart via bloodstream,


lymphatics or direct invasion

Usually present as small, firm nodules

Location:
1. Pericardium most common
2. Myocardium
3. Rarely, endocardium and cardiac valves
Cardiac Metastases

Clinical presentation:

Depends on location and size of tumor

Signs & symptoms occur only in 10%; non-


specific

Usually occurs in the setting of recognized


neoplasm
Cardiac Metastases

Clinical presentation:

1. Dyspnea most common


2. Signs of pericarditis
a) Chest pain aggravated by coughing,
inspiration or recumbency
b) Pericardial friction rub on auscultation
c) Characteristic ECG changes
3. Cardiac tamponade
a) Increased JVP
b) Pulsus paradoxus
c) Echo evidence of RA and RV collapse
Cardiac Metastases

Seen over the surface of the


epicardium are pale white-tan
nodules of metastatic tumor.
Metastases may lead to a
hemorrhagic pericarditis.
Cardiovascular Manifestations
of Systemic Diseases
Diabetes Mellitus

1. Increased incidence of CAD most


common cause of death in adults with DM

Two types of vascular disease


a) Macrovascular
Atherosclerosis & arteriosclerosis - CAD
Cerebral circulation TIA, stroke
Lower limb circulation claudication,
ulceration, gangrene
b) Microvascular
Retinopathy, nephropathy, neuropathy,
small artery occlusions of the heart
Diabetes Mellitus

1. Increased incidence of CAD

MI more frequent but also tend to be


larger in size and more likely to result in
complications such as heart failure, shock,
and death

Abnormal or absent pain response to


myocardial ischemia due to generalized
autonomic nervous system dysfunction
up to 90% silent ischemias
Diabetes Mellitus

1. Increased incidence of CAD

Presentation of ischemia may be:


Exertional or episodic dyspnea
Flash pulmonary edema
Arrhythmias
Heart block
Syncope
Diabetes Mellitus

2. Restrictive cardiomyopathy

Myocardial dysfunction in the absence of


large-vessel CAD

Abnormal relaxation of myocardium


elevated left ventricular filling pressure

Diastolic heart failure


Diabetes Mellitus

3. Autonomic Neuropathy

Secondary to autonomic denervation

Manifested as fixed tachycardia with


subsequent parasympathetic damage
decreased heart rate

Complete autonomic denervation HR


no longer responsive to physiologic
stimuli
Malnutrition & Vitamin Deficiency

1. Kwashiorkor or Marasmus or Both

Heart becomes thin, pale, and flabby with


myofibrillar atrophy and interstitial
edema

Low systolic pressure and cardiac output

Narrow pulse pressure

Generalized edema due to:


a) Reduced serum oncotic pressure
b) Myocardial dysfunction
Malnutrition & Vitamin Deficiency

1. Kwashiorkor or Marasmus or Both

Effects of starvation on the heart:


a) Decreased contractile force
decreased cardiac output
b) Diminished diastolic compliance

Clinical:
Bradycardia
Hypotension
ECG: NSSTTWC, ectopic rhythm
MVP
Decreased exercise capacity
Heart failure, worsened or precipitated by
feeding
Malnutrition & Vitamin Deficiency

1. Kwashiorkor or Marasmus or Both

Decreased intake also seen in:


a) Chronic disease AIDS, PTB
b) Semi-starvation anorexia nervosa
c) Severe CHF GI hypoperfusion and
venous congestion anorexia and
malabsorption

Treatment should be gradual rapid


expansion leads to stress to heart
heart failure
Malnutrition & Vitamin Deficiency

2. Thiamine deficiency (Beriberi)

May occur in the presence of adequate


intake of calories and protein if polished
rice is used

Deficiency common among alcoholics

Clinical:
a) Generalized malnutrition
b) Peripheral neuropathy
c) Glossitis
d) Anemia
Malnutrition & Vitamin Deficiency

2. Thiamine deficiency (Beriberi)

Characteristic cardiovascular syndrome:


a) Heart failure with increased cardiac
output high output due to
vasomotor depression leading to
reduced systemic vascular resistance
b) Tachycardia
c) Elevated filling pressures in the left
and right sides of the heart
Malnutrition & Vitamin Deficiency

2. Thiamine deficiency (Beriberi)

Diagnostic criteria:
a) Clinical features
Dependent edema
Low peripheral vascular resistance,
decreased minimum BP, increased pulse
pressure
Hyperkinetic circulatory state (mid-systolic
murmur and S3)
Enlarged heart
T-wave changes on ECG: inverted,
diphasic, depressed
Peripheral neuritis
Dietary deficiency for at least 3 months or
chronic alcoholism
Malnutrition & Vitamin Deficiency

2. Thiamine deficiency (Beriberi)

Diagnostic criteria:

b) Presence of thiamine deficiency


Decreased blood thiamine
concentration
Decreased ESR

Improvement after adequate thiamine


therapy
Obesity

Increased CV mortality and morbidity

Increased prevalence of
Hypertension
Glucose intolerance
Atherosclerotic coronary artery disease

Distinct CVS abnormalities


Obesity

Distinct CVS abnormalities:


Increased total and central blood volumes
Increased cardiac output and LV filling
pressure
Hypertension
Eccentric LVH
Pickwickian syndrome cor pulmonale,
apnea, hypoxemia
Heart failure (+) crackles, inc. JVP, S3, S4
Edema
Exercise intolerance
Obesity

Treatment:

Weight reduction most effective

Digitalis

Sodium restriction

Diuretics
Thyroid Disease

Physiologic effects of thyroid hormone:

1. Increased total body metabolism and


oxygen consumption
Increase workload on the heart
2. Direct inotropic, chronotropic, and
dromotropic effects
Tachycardia, increased cardiac output
3. Increase synthesis of myosin, Na+,K+ -
ATPase
4. Increase density of myocardial -
adrenergic receptors
Hyperthyroidism

Essentials of Diagnosis:
1. Low TSH levels
2. Increased T3, T4, iodine uptake

General Considerations:
1. Increased levels of thyroid hormone
hyperdynamic CVS
Increased cardiac output, contractility;
tachycardia
2. Decreased SVR
Hyperthyroidism

Symptoms and Signs:


1. Systemic s/sx
a) Weight loss
b) Increased appetite
c) Resting tremors of the hand
d) Nervousness, anxiety, insomnia, mood
swings, irritability
e) Heat intolerance & sweaty skin
f) Proximal muscle weakness & wasting
g) Increased bowel movement or
diarrhea
h) Diplopia
i) Periodic paralysis
Hyperthyroidism

Symptoms and Signs:


2. Cardiovascular s/sx
a) Palpitations
b) Dyspnea with or without LV failure
c) Atypical chest pain
d) Cardiac arrhythmias AF, PACs
e) Apathetic hyperthyroidism
Elderly patient
Present only with CV manifestations
of thyrotoxicosis such as AF
(resistant to therapy until hyper-
thyroidism is controlled)
Hyperthyroidism

Physical Examination:

1. Stare, lid retraction, exophthalmos


2. Skin soft and velvety
3. Goiter audible bruit
4. Precordium
Inspection hyperdynamic
Auscultation loud S1, systolic
ejection murmur
Palpation rapid & bounding pulse
5. Proximal muscle weakness
6. Hyperreflexic DTRs
Hyperthyroidism

Diagnostic Studies:

1. ECG sinus tachycardia, AF

2. Echocardiography hypercontractility,
increased LV mass & hypertrophy

3. Thyroid function test T3, T4, TSH, RAIU


Hyperthyroidism

Initial Diagnosis:

1. Atrial arrhythmias
2. Cardiac enlargement
3. Ventricular failure
4. s/sx of hyperthyroidism

Definite Diagnosis:
1. (+) signs and symptoms
2. Biochemical evidence of hyperthyroidism
3. Reversal of findings after treatment
Hyperthyroidism

Treatment:
Directed at improving s/sx, reducing the
demands to the heart

1. Anti-thyroid drugs
2. Thyroid ablation
3. Steroids hydrocortisone 50-100 mg q 6-
8 hours
4. Beta blockers if without CHF Propanolol
20-30 mg 4x/day
5. Digitalis
6. Anti-coagulation
Hypothyroidism

Essentials of Diagnosis:
Increased TSH
Low T3, T4, FTI

General Considerations:
Given to any form of TH deficiency
Myxedema TH deficiency with profound
hypothermia, hypoventilation,
hypotension, CNS signs (coma)
Associated with accelerated athero-
sclerosis
Angina uncommon due to decreased
metabolic demand
Hypothyroidism

Clinical Findings:

1. Systemic signs and symptoms

a) Weight gain, weakness, lethargy, fatigue,


depression
b) Constipation, cold intolerance, dry skin,
coarse hair
c) Menstrual disorders, impotence or
decreased libido
Hypothyroidism

Clinical Findings:

2. Cardiovascular signs and symptoms

a) Decreased CO, SV, HR

b) Loss of inotropism and chronotropism

c) Heart failure rare decreased CO match


metabolic demands
Hypothyroidism

Diagnostic Studies:

1. ECG
sinus bradycardia
prolonged PR & QT interval
low voltage complexes
flattened or inverted T waves
Atrial, ventricular or interventricular delay

2. Echocardiography effusion, ASH


Hypothyroidism

Diagnostic Studies:

3. Laboratory findings

Low T3, T4; high TSH levels


Increased cholesterol & triglyceride
Hyponatremia
Increased CK-MM but not CK-MB
anemia
Hypothyroidism

Initial Diagnosis:
1. Pericardial effusion or decreased
contractile performance
2. Clinical suspicion of hypothyroidism

Definite Diagnosis:
1. Clinical findings
2. Biochemical evidence of hypothyroidism
3. Reversal of abnormalities after treatment
with thyroid hormone
Hypothyroidism

Treatment:

Thyroid hormone replacement

If > 50 y/o judicious & slow


replacement
To prevent exacerbation of angina or
precipitation of AMI
of the usual replacement dose (25
mg/day)
Malignant Carcinoid

Tumors that elaborate vasoactive amines


(eg serotonin), kinins, indoles
responsible for diarrhea, flushing, labile BP

Gastrointestinal carcinoids
Almost exclusively in the right side
Occur only with hepatic metastases
substance responsible for the cardiac
lesions inactivated by passage through
liver and lungs
Malignant Carcinoid

Left-sided lesions occur when


1. there exists a right-to-left shunt, or
2. tumor is located in the lungs

Lesion: fibrous plaques on the endothelium


of cardiac chambers, valves, and great
vessels result in distortion of the cardiac
valves
Malignant Carcinoid

Clinical syndrome:

1. Tricuspid regurgitation, pulmonic


stenosis or both
2. High-output cardiac state may occur
due to decrease in systemic vascular
resistance
3. Coronary artery spasm due to a
circulating vasoactive substance
Malignant Carcinoid
Pheochromocytoma

High circulating levels of catecholamines


labile or sustained hypertension LVH

May cause direct myocardial injury


Focal myocardial necrosis and
inflammatory cell infiltration (50% of
patients) contribute to significant LV
failure and pulmonary edema

LV function and CHF may resolve after


removal of tumor
Pheochromocytoma

Systemic signs and symptoms:


1. Attacks of headache
2. Palpitations
3. Tachycardia
4. Sweating
5. Irritability

CVS signs and symptoms:


1. Inc. HR, contractility, conduction velocity
2. Orthostatic hypotension
3. Hypertension (85%) sustained or
paroxysmal
4. LVH
5. LV failure due to focal myocardial necrosis
6. Pulmonary edema
Acromegaly

Excessive growth hormone

1. CHF due to high cardiac output


2. Diastolic dysfunction due to ventricular
hypertrophy increased LV chamber
size or wall thickness
3. Global systolic dysfunction
4. Suppression of renin-aldosterone axis
increased total body sodium and
plasma volume hypertension
Rheumatoid Arthritis

Inflammation of any or all anatomical parts


of the heart

Pericarditis
Most common cause of clinically
apparent disease
Found by echocardiography in 10-50% of
patients, particularly those with sub-
cutaneous nodules
Usually benign course but may progress
to cardiac tamponade or constrictive
pericarditis
Rheumatoid Arthritis

Coronary arteritis
20% of cases; rarely results in angina or MI

Cardiac valves
Mitral or aortic regurgitation
Inflammation and granuloma formation

Myocarditis
Rarely result in cardiac dysfunction

Pericardial fluid
Exudate, dec. conc. complements, dec.
Glucose, elevated cholesterol
Rheumatoid Arthritis

Two-dimensional color and spectral Doppler echocardiographic studies


of patients with rheumatic heart disease show moderate to severe aortic
valve insufficiency with no stenosis (A, arrow) and bowing of the anterior
mitral leaflet with severe insufficiency and no stenosis (B, arrow).
Rheumatoid Arthritis

Treatment:

Treat underlying RA

Glucocorticoids

Pericardiectomy
Systemic Lupus Erythematosus

Pericarditis
2/3 of patients
Benign course
Rarely tamponade or constriction

Myocarditis
Seen in autopsy in up to 80%
Only 20% clinically detected
Parallels the activity of the disease
Seldom results to clinical heart failure,
unless associated with hypertension
Systemic Lupus Erythematosus

Valvular Heart Disease

Clinically most important and frequent SLE-


associated CV manifestation
SLE with elevated antibody to cardiolipin
high incidence of valvular disease
Younger patients with active disease, 5 yrs.
Libman-Sacks lesion
Characteristic endocardial lesion
Wart-like lesions most often located at
angle of the valves or ventricular surface
of MV
Hemodynamically important valcular lesions
rare
Systemic Lupus Erythematosus

Coronary Artery Disease

Secondary to arteritis of large coronary


arteries, embolism
Also due to atherosclerosis related to
hypertension or glucocorticoid therapy

Thrombotic Disease

Deep venous thrombosis


Pulmonary, peripheral or cerebral thrombosis
Associated with anti-phospholipid antibodies
produce endothelial dysfunction
Systemic Lupus Erythematosus
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