Cardiac tumors
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Cardiac tumors Cardiac tumors Presentation Transcript

  • 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
  • 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 3 rd – 6 th decade; female > male
    • Sporadic vs familial
    • Majority sporadic; some are familial (autosomal dominant transmission) or part of a syndrome
      • Carney complex – spotty skin pigmentation, myxomas, endocrine overactivity, schwannomas
      • NAME syndrome – nevi, atrial myxoma, myxoid neurofibroma, ephelides
      • 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 exertion Paroxysmal dyspnea Fever Weight loss Severe dizziness/syncope Sudden death Hemoptysis >75 ~25 ~50 ~25 ~20 ~15 ~15
  • 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
  • Myxoma
    • Clinical Presentation
      • Systemic or cardiovascular findings
      • Cardiovascular findings:
        • 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
        • Ventricular – outflow obstruction  syncope
  • Myxoma
    • Diagnosis:
      • Two-dimensional transthoracic or trans-esophageal echocardiography
        • Determine site of tumor attachment and tumor size
        • Screening of 1 st degree relatives for familial or syndrome myxoma
      • CT scan and MRI
        • Tumor size, shape, composition, and surface characteristics
      • 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
    • 2 nd 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
    • 2 nd 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; 2 nd 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:
      • Hemodynamic compromise
      • Local invasion
      • 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:
      • Pericardium – most common
      • Myocardium
      • 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:
    • Dyspnea – most common
    • Signs of pericarditis
      • Chest pain aggravated by coughing, inspiration or recumbency
      • Pericardial friction rub on auscultation
      • Characteristic ECG changes
    • Cardiac tamponade
      • Increased JVP
      • Pulsus paradoxus
      • 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
    • Increased incidence of CAD  most common cause of death in adults with DM
      • Two types of vascular disease
        • Macrovascular
          • Atherosclerosis & arteriosclerosis - CAD
          • Cerebral circulation  TIA, stroke
          • Lower limb circulation  claudication, ulceration, gangrene
        • Microvascular
          • Retinopathy, nephropathy, neuropathy, small artery occlusions of the heart
  • Diabetes Mellitus
    • 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
    • Increased incidence of CAD
      • Presentation of ischemia may be:
        • Exertional or episodic dyspnea
        • Flash pulmonary edema
        • Arrhythmias
        • Heart block
        • Syncope
  • Diabetes Mellitus
    • 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
    • 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
    • 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:
        • Reduced serum oncotic pressure
        • Myocardial dysfunction
  • Malnutrition & Vitamin Deficiency
    • Kwashiorkor or Marasmus or Both
      • Effects of starvation on the heart:
        • Decreased contractile force  decreased cardiac output
        • Diminished diastolic compliance
      • Clinical:
        • Bradycardia
        • Hypotension
        • ECG: NSSTTWC, ectopic rhythm
        • MVP
        • Decreased exercise capacity
        • Heart failure, worsened or precipitated by feeding
  • Malnutrition & Vitamin Deficiency
    • Kwashiorkor or Marasmus or Both
      • Decreased intake also seen in:
        • Chronic disease – AIDS, PTB
        • Semi-starvation – anorexia nervosa
        • 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
    • 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:
        • Generalized malnutrition
        • Peripheral neuropathy
        • Glossitis
        • Anemia
  • Malnutrition & Vitamin Deficiency
    • Thiamine deficiency (Beriberi)
      • Characteristic cardiovascular syndrome:
        • Heart failure with increased cardiac output  high output due to vasomotor depression leading to reduced systemic vascular resistance
        • Tachycardia
        • Elevated filling pressures in the left and right sides of the heart
  • Malnutrition & Vitamin Deficiency
    • Thiamine deficiency (Beriberi)
      • Diagnostic criteria:
        • 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
    • Thiamine deficiency (Beriberi)
      • Diagnostic criteria:
        • 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:
      • Increased total body metabolism and oxygen consumption
        • Increase workload on the heart
      • Direct inotropic, chronotropic, and dromotropic effects
        • Tachycardia, increased cardiac output
      • Increase synthesis of myosin, Na + ,K + - ATPase
      • Increase density of myocardial ß-adrenergic receptors
  • Hyperthyroidism
    • Essentials of Diagnosis:
      • Low TSH levels
      • Increased T3, T4, iodine uptake
    • General Considerations:
      • Increased levels of thyroid hormone  hyperdynamic CVS
        • Increased cardiac output, contractility; tachycardia
      • Decreased SVR
  • Hyperthyroidism
    • Symptoms and Signs:
      • Systemic s/sx
        • Weight loss
        • Increased appetite
        • Resting tremors of the hand
        • Nervousness, anxiety, insomnia, mood swings, irritability
        • Heat intolerance & sweaty skin
        • Proximal muscle weakness & wasting
        • Increased bowel movement or diarrhea
        • Diplopia
        • Periodic paralysis
  • Hyperthyroidism
    • Symptoms and Signs:
      • Cardiovascular s/sx
        • Palpitations
        • Dyspnea – with or without LV failure
        • Atypical chest pain
        • Cardiac arrhythmias – AF, PACs
        • 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:
      • Stare, lid retraction, exophthalmos
      • Skin – soft and velvety
      • Goiter – audible bruit
      • Precordium
        • Inspection – hyperdynamic
        • Auscultation – loud S1, systolic ejection murmur
        • Palpation – rapid & bounding pulse
      • Proximal muscle weakness
      • Hyperreflexic DTRs
  • Hyperthyroidism
    • Diagnostic Studies:
      • ECG – sinus tachycardia, AF
      • Echocardiography – hypercontractility, increased LV mass & hypertrophy
      • Thyroid function test – T3, T4, TSH, RAIU
  • Hyperthyroidism
    • Initial Diagnosis:
      • Atrial arrhythmias
      • Cardiac enlargement
      • Ventricular failure
      • s/sx of hyperthyroidism
    • Definite Diagnosis:
      • (+) signs and symptoms
      • Biochemical evidence of hyperthyroidism
      • Reversal of findings after treatment
  • Hyperthyroidism
    • Treatment:
      • Directed at improving s/sx, reducing the demands to the heart
      • Anti-thyroid drugs
      • Thyroid ablation
      • Steroids – hydrocortisone 50-100 mg q 6-8 hours
      • Beta blockers if without CHF – Propanolol 20-30 mg 4x/day
      • Digitalis
      • 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:
    • Systemic signs and symptoms
      • Weight gain, weakness, lethargy, fatigue, depression
      • Constipation, cold intolerance, dry skin, coarse hair
      • Menstrual disorders, impotence or decreased libido
  • Hypothyroidism
    • Clinical Findings:
    • Cardiovascular signs and symptoms
      • Decreased CO, SV, HR
      • Loss of inotropism and chronotropism
      • Heart failure rare – decreased CO match metabolic demands
  • Hypothyroidism
    • Diagnostic Studies:
    • ECG
      • sinus bradycardia
      • prolonged PR & QT interval
      • low voltage complexes
      • flattened or inverted T waves
      • Atrial, ventricular or interventricular delay
    • Echocardiography – effusion, ASH
  • Hypothyroidism
    • Diagnostic Studies:
    • Laboratory findings
      • Low T3, T4; high TSH levels
      • Increased cholesterol & triglyceride
      • Hyponatremia
      • Increased CK-MM but not CK-MB
      • anemia
  • Hypothyroidism
    • Initial Diagnosis:
      • Pericardial effusion or decreased contractile performance
      • Clinical suspicion of hypothyroidism
    • Definite Diagnosis:
      • Clinical findings
      • Biochemical evidence of hypothyroidism
      • 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
      • there exists a right-to-left shunt, or
      • 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:
      • Tricuspid regurgitation, pulmonic stenosis or both
      • High-output cardiac state may occur – due to decrease in systemic vascular resistance
      • 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:
      • Attacks of headache
      • Palpitations
      • Tachycardia
      • Sweating
      • Irritability
    • CVS signs and symptoms:
      • Inc. HR, contractility, conduction velocity
      • Orthostatic hypotension
      • Hypertension (85%) – sustained or paroxysmal
      • LVH
      • LV failure – due to focal myocardial necrosis
      • Pulmonary edema
  • Acromegaly
    • Excessive growth hormone
      • CHF due to high cardiac output
      • Diastolic dysfunction due to ventricular hypertrophy – increased LV chamber size or wall thickness
      • Global systolic dysfunction
      • 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