Cardiac TumorsCardiac TumorsMoises M. Bartolome III, MD, FPCP

Department of MedicineOur 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

Relative incidence of primary malignant heart tumors

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 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 syndrome1. Carney complex – spotty skin pigmentation,

myxomas, endocrine overactivity, schwannomas2. NAME syndrome – nevi, atrial myxoma, myxoid

neurofibroma, ephelides3. LAMB syndrome – lentigines, atrial myxoma, blue

nevi

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

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 exertionParoxysmal dyspneaFeverWeight lossSevere dizziness/syncopeSudden deathHemoptysis

>75~25~50~25~20~15~15

Myxoma – Symptoms and Signs

Signs Incidence (%)

Mitral diastolic murmurMitral systolic murmurPulmonary hypertensionRight heart failurePulmonary emboliAnemiaElevated ESRThird heart sound (tumor plop)Atrial fibrillationElevated globulinsClubbingRaynaud’s phenomenon

~75~50~70~70~25>33>33>33~15~10~5<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 trauma2. 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 myxoma2. 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 patient’s death weeks to months from time of presentation due to:1. Hemodynamic compromise2. Local invasion3. 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 common2. Myocardium3. 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 common2. Signs of pericarditis

a) Chest pain aggravated by coughing, inspiration or recumbency

b) Pericardial friction rub on auscultationc) Characteristic ECG changes

3. Cardiac tamponadea) Increased JVPb) Pulsus paradoxusc) 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 Cardiovascular Manifestations of Systemic Diseasesof Systemic Diseases

Diabetes Mellitus

1. Increased incidence of CAD most common cause of death in adults with DM

• Two types of vascular diseasea) Macrovascular

Atherosclerosis & arteriosclerosis - CAD Cerebral circulation TIA, stroke Lower limb circulation claudication,

ulceration, gangreneb) 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 pressureb) Myocardial dysfunction

Malnutrition & Vitamin Deficiency

1. Kwashiorkor or Marasmus or Both

• Effects of starvation on the heart:a) Decreased contractile force

decreased cardiac outputb) 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, PTBb) Semi-starvation – anorexia nervosac) 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 malnutritionb) Peripheral neuropathyc) Glossitisd) 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) Tachycardiac) 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 ß-adrenergic receptors

Hyperthyroidism

Essentials of Diagnosis:1. Low TSH levels2. Increased T3, T4, iodine uptake

General Considerations:1. Increased levels of thyroid hormone

hyperdynamic CVS• Increased cardiac output, contractility;

tachycardia2. Decreased SVR

Hyperthyroidism

Symptoms and Signs:1. Systemic s/sx

a) Weight lossb) Increased appetitec) Resting tremors of the handd) Nervousness, anxiety, insomnia, mood

swings, irritabilitye) Heat intolerance & sweaty skinf) Proximal muscle weakness & wastingg) Increased bowel movement or diarrheah) Diplopiai) Periodic paralysis

Hyperthyroidism

Symptoms and Signs:2. Cardiovascular s/sx

a) Palpitationsb) Dyspnea – with or without LV failurec) Atypical chest paind) Cardiac arrhythmias – AF, PACse) 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, exophthalmos2. Skin – soft and velvety3. Goiter – audible bruit4. Precordium

• Inspection – hyperdynamic• Auscultation – loud S1, systolic ejection

murmur• Palpation – rapid & bounding pulse

5. Proximal muscle weakness6. 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 arrhythmias2. Cardiac enlargement3. Ventricular failure4. s/sx of hyperthyroidism

Definite Diagnosis:1. (+) signs and symptoms2. Biochemical evidence of hyperthyroidism3. Reversal of findings after treatment

Hyperthyroidism

Treatment:• Directed at improving s/sx, reducing the

demands to the heart

1. Anti-thyroid drugs2. Thyroid ablation3. Steroids – hydrocortisone 50-100 mg q 6-8

hours4. Beta blockers if without CHF – Propanolol

20-30 mg 4x/day5. Digitalis6. 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 performance2. Clinical suspicion of hypothyroidism

Definite Diagnosis:1. Clinical findings2. Biochemical evidence of hypothyroidism3. 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 ¼ 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, or2. 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 headache2. Palpitations3. Tachycardia4. Sweating5. Irritability

CVS signs and symptoms:1. Inc. HR, contractility, conduction velocity2. Orthostatic hypotension3. Hypertension (85%) – sustained or paroxysmal4. LVH5. LV failure – due to focal myocardial necrosis6. Pulmonary edema

Acromegaly

• Excessive growth hormone

1. CHF due to high cardiac output2. Diastolic dysfunction due to ventricular

hypertrophy – increased LV chamber size or wall thickness

3. Global systolic dysfunction4. 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

Thank you