eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Cardiac Tumors
Updated: Nov 10, 2008
Introduction
Background
Cardiac tumors were first described in the 18th century by Boneti; however, many believe the description by Albers in 1835 is the first authentic report. In 1936, the first successful removal of a neoplasm of the heart was performed. In 1952, angiography was first used for the diagnosis of heart tumors. In 1955, bypass was used for the first time to excise an intracavitary tumor. Cardiac tumors, whether primary or metastatic, are rare.Benign neoplasms occur 3 times more often than malignant tumors. By far, the most common type of tumors reported in children and adolescents is rhabdomyoma, followed by fibroma, myxoma, and teratoma. Sarcoma is the largest group of primary cardiac malignant neoplasms. Of these sarcomas, angiosarcomas are the most common histologic type and occur more frequently in males. These tumors directly seed blood, thus metastases are common and widespread. Signs and symptoms of these tumors at presentation are generalized, nonspecific, and mimic several other systemic diseases.
The clinical presentation of a patient with a cardiac tumor is determined more by the tumor's location than by its histologic type. Benign tumors, depending on their location, can present more symptoms than malignant tumors if they critically obstruct a valve or outflow tract. Consider the findings that are typical for each location.
Right-sided tumors may present with congestive heart failure (CHF) manifested by fatigue, edema, jugular venous distention, and ascites. Other symptoms include shortness of breath, syncope, and night sweats. Pericardial effusions may occur. Vena cava syndrome, pulmonary embolism, and restrictive cardiomyopathy are some of the complications.
Left atrial and left ventricular tumors can present various signs and symptoms that include, but are not restricted to, fever, chills, dizziness, dyspnea on exertion, cold sweats during exercise or at night, and nonproductive cough. Because tumors may embolize, they also can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents. Based on their size and position, they may induce arrhythmias and interfere with ventricular compliance.
Benign tumors
Rhabdomyomas are hamartomas and are the most frequently found tumors in children. They are associated with tuberous sclerosis in about 50-80% of patients. Of patients with tuberous sclerosis, 50-60% have rhabdomyomas. These tumors are frequently multiple, involving ventricular free and septal walls, and have a yellowish-gray color. They vary from small to extremely large. They are rarely excised because they tend to regress over time; if patients are asymptomatic, only observation is warranted. Surgical removal is indicated when they present with obstruction leading to cardiac compromise or intractable arrhythmias.
Fibromas, usually single and large, are most commonly found in the left ventricular free wall and rarely involve the septal wall. As many as 40% of fibromas are diagnosed in infants younger than 1 year. These tend to be firm nonencapsulated tumors derived from fibroblasts.
Myxomas are usually seen in adults. They are rarely seen in children, accounting for only 9-15% of all cardiac tumors from birth to adolescence. They are often found attached to the atrial septum and mitral valve apparatus in the left atrium (>85%). Myxomas can appear sporadically or as part of the syndrome myxoma or Carney syndrome, which includes endocrine neoplasms, tumors in other organs, and skin with spotty hyperpigmentation. This type of familial cardiac myxoma accounts for less than 10% of the myxomas appearing in the heart. They have an autosomal dominant transmission and most commonly appear in females. Patients with syndrome myxoma tend to be younger than those with sporadic myxomas. Myxomas may embolize; this may be their first clinical presentation. Peripheral embolization is reported to occur in as many as 70% of patients with myxomas and may even occur in utero.
Teratomas are single, encapsulated, grayish-tan tumors that appear most often in the pericardium. Teratomas develop in the right atrium, right ventricle, and septum of the heart. They are large basal tumors that, in newborns, may be larger than the patient's own heart.
Angiomas are a benign proliferation of endothelial cells, also known as vascular tumors. They can occur in any part of the heart (with a preference for right-sided chambers) and can form blood vessels (hemangiomas) or lymph vessels (lymphangiomas). These vascular vessels communicate between themselves within the myocardium. They can infiltrate the intraventricular septum near the conduction system where they may cause heart block. Hemangiomas are red, hemorrhagic, sessile, or polypoid subendocardial nodules that vary from small to large and occasionally have been associated with hemorrhagic cardiac tamponade.
Other types of tumors that have been less frequently reported in children include lipomas, papillary tumors, accessory cardiac cushion tissue, leiomyomas, mesotheliomas, fibroelastomas, fibroelastic papillomas, and benign cystic tumors.
Malignant tumors
Sarcomas originate from mesenchyme and, therefore, display a wide variety of morphologic types. These tumors are rare in children, with angiosarcoma being the most common type of sarcoma for all ages.
Cardiac angiosarcomas are characteristically lobulated variegated masses that are necrotic or hemorrhagic and are composed of anastomosing vascular channels lined by malignant cells that may contain areas of spindle cells. They tend to be aggressive malignancies, either infiltrative or polypoid, with most arising from the right atrium. Metastases are common and, based on autopsy studies, occur in as many as 88% of patients with malignant tumors.
Rhabdomyosarcomas grow invasively, metastasize, and can recur. These tumors are rare and are more common in children because they may arise from embryonic cell rests in the septum.
Fibrosarcomas, often involving more than one chamber of the heart, contain areas of hemorrhage and necrosis.
Other malignant neoplasms
Other reported malignant cardiac tumors are lymphomas, histiocytoma, leiomyosarcomas, choriocarcinoma, liposarcoma, and osteogenic sarcomas.
Frequency
United States
Primary cardiac tumors are rare, with a frequency of 0.0017-0.28% based on autopsies. About 75% of them are benign and 25% are malignant. Secondary (metastatic) tumors are 10-40 times more frequent. Lam et al reviewed 12,485 autopsies and found only 7 cases of primary neoplasm of the heart versus 154 cases of secondary heart tumors.1
Rhabdomyomas are the most common tumor in children, with approximately 75% diagnosed in children younger than 1 year. Of these tumors, 50-80% are associated with tuberous sclerosis. Fibromas are the second most common tumor in children, with most (40%) diagnosed in children younger than 1 year. Myxomas are seen in 9-15% of patients with cardiac tumors, are the most common primary cardiac neoplasms in all ages, and comprise 50-60% of all heart tumors. The true incidence of teratomas is not known; however, 50% are diagnosed in newborns and 66% in children younger than 1 year. Sarcomas comprise 25% of all cardiac tumors; the most frequent tumor found is angiosarcoma.
Race
No racial predilection is observed.
Sex
Myxomas are more common in females, especially in patients with syndrome myxoma or Carney syndrome. Angiosarcomas have a 2:1 male-to-female ratio. Rhabdomyomas, fibromas, teratomas, other sarcomas, and malignant tumors do not have any sex predilection.
Age
No specific age predilection is noted; however, rhabdomyomas, fibromas, teratomas, and rhabdomyosarcomas are more commonly seen in children and adolescents than in adults. Myxomas comprise 50-60% of all benign tumors of the heart and are found mainly in adults.
Clinical
History
Diagnosis is a challenge for any physician because cardiac tumors have no typical presentation. Typically, patients are asymptomatic or present with nonspecific signs and symptoms. Some authors call heart neoplasms the great masqueraders. Certain symptoms, including irritability, shortness of breath, anorexia, tiredness, or palpitations, may raise suspicion of a neoplastic process.
- Initial symptoms in infants may include irritability, periodic episodes of pallor, fever, tachypnea, tachycardia, anorexia, and failure to thrive.
- Older children and adolescents may present with similar symptoms and may complain of dyspnea on exertion, dizziness, general malaise, syncope, hemoptysis, and shock or experience sudden death.
- For presenting symptoms of specific types of cardiac tumors, see Background.
Physical
Clinical presentation and physical findings relate to location of the tumor. Arrhythmias, heart failure, fever, pericardial effusion, and new or louder heart murmurs are a few of the findings for all these growths (see Background).
- Right-sided tumors: Heart failure, edema, jugular venous distention, ascites, shortness of breath, right-sided third and/or fourth heart sounds, cor pulmonale, pericardial effusions, hepatomegaly, vena cava syndrome, and pulmonary embolism are associated with right-sided tumors.
- Left-sided tumors: With the ability to embolize, these tumors can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents.
- Based on their size and position, left-sided and right-sided tumors may interfere with ventricular compliance.
- Nonproductive cough and hemoptysis have been reported in older children and adults.
- Malar flush, emboli, spotty hyperpigmentation of the skin, and a distinctive apical diastolic sound called a tumor plop are associated with myxoma.
Causes
Mutations in the gene protein kinase A (PKA) were identified in patients with Carney complex and myxomas. A mutation of the gene that causes neurofibromatosis is present in patients who have neurofibromatosis and cardiac tumors. Whether or not this means cardiac tumors are directly related to neurofibromatosis is uncertain, although a relationship is likely.
Studies have described the relationship between angiogenesis and tumor growth.2 Cardiac myxomas produce vascular endothelial growth factor, probably inducing angiogenesis for tumor growth. Neoangiogenesis is involved in the development of masses in the heart, benign or malignant. This knowledge is important for the possible creation of adjuvant therapies for inhibition of the tumor.
Differential Diagnoses
| Acidosis, Metabolic | Mitral Stenosis, Acquired |
| Atrial Septal Defect, General Concepts | Myocarditis, Nonviral |
| Cardiomyopathy, Hypertrophic | Neonatal Lupus and Cutaneous Lupus Erythematosus
in Children |
| Cardiomyopathy, Restrictive | Pulmonary Hypertension, Idiopathic |
| Double Outlet Right Ventricle, Normally Related
Great Arteries | Pulmonary Stenosis, Infundibular |
| Double-Chambered Right Ventricle | Pulmonary Stenosis, Valvar |
| Ebstein Anomaly | Rheumatic Heart Disease |
| Endocardial Fibroelastosis | Systemic Lupus Erythematosus |
| Hypoplastic Left Heart Syndrome | Thrombophlebitis |
| Loffler Syndrome | Vasculitis and Thrombophlebitis |
Other Problems to Be Considered
Collagen vascular diseases
Mural thrombus
Cerebrovascular accident
Vasculitis (ie, lupus, polyarteritis)
Mitral valve stenosis
Workup
Laboratory Studies
- CBC count with differential should be normal in benign cardiac tumors. In malignant tumors, any kind of blood count abnormality can be present in these patients. For example, anemia is not an uncommon finding. Platelet count may be elevated or low, and WBC count may be high.
- Erythrocyte sedimentation rate (ESR) is a nonspecific marker for inflammation that may be normal or elevated; however, the ESR could be low in the presence of congestive heart failure (CHF).
- Liver function test findings are occasionally elevated because of severe right-sided heart failure or metastases.
- Creatine kinase-myocardial band (CK-MB) fraction levels have been found to be higher in patients who have both ventricular dysfunction and a cardiac tumor but not necessarily primary malignancies from the heart.
Imaging Studies
- Echocardiography
- This is the most cost-effective of all tests used to evaluate the anatomy of the heart. Echocardiography can provide important information about size, extension and/or invasion within and outside the heart, valvar involvement and/or competency, ventricular function, and pericardial effusion.
- It is very sensitive but not specific.
- Some of these tumors may be too small for echocardiographic detection, especially those leading to dysrhythmias.
- Transesophageal echocardiogram also is useful in detecting tumors in the left heart, especially in adults.
- Chest roentgenography: Cardiomegaly and pulmonary edema may present in cases of obstructive lesions. Certain neoplasms can have calcium deposition detected on the roentgenogram.
- MRI: MRI is a powerful tool in diagnosing tumors, evaluating their extent, and seeking metastases. It may delineate the area of the mass better than echocardiography because it has a larger field of observation. Contrast enhancement and multislice imaging can provide a 3-dimensional view of tissue. It also can help differentiate tumors from thrombi.
- CT scanning: Ultrafast CT scanning is gaining use in this area because it eliminates artifacts of heart motion seen with conventional CT scanning. CT is a powerful tool in evaluating the extent of the tumor and exhibits a high degree of tissue discrimination.
Other Tests
- ECG: Nonspecific changes are the most common findings; however, ST-T segment changes with strain patterns have been noticed in obstructive rhabdomyomas and fibromas. After surgery, these changes return to normal. Other patterns associated with ischemia also may be present. Diffuse low-voltage QRS, especially in the presence of pericardial effusion, can be seen. Arrhythmias may be the first presentation of an intracardiac tumor and occasionally are resistant to medical therapy. Other findings include intraventricular conduction delay and complete bundle branch block.
Procedures
- Endomyocardial biopsy is controversial and seldom useful in children because of the high possibility of false-negative results and the high possibility of metastasis during the procedure if a malignant tumor is present.
Histologic Findings
Benign tumors
Rhabdomyomas are a glycogenic degeneration of myocardial fibers and are thus considered hamartomas. They contain large vacuolated cells filled with glycogen. Their microscopic hallmark, the spider cell, displays eccentric nuclei, granular cytoplasm with a central cytoplasmic mass, and fine fibrillar processes that radiate to the periphery, giving the appearance of a spider in a web. Because the cytoplasm is rich in glycogen, it stains positive with periodic acid-Schiff reagent. Rhabdomyositis is a rare form of cardiomyopathy. In this pathology, microscopic changes in the muscle and conduction system are evident, yet gross anatomic findings are rare.
Cardiac fibromas consist of elongated fibroblasts, with fibrous tissue and collagen. Occasionally, calcium can be seen using radiography or microscopically within the tumor. Mitosis is rarely seen in this type of tumor.
Myxoma has a characteristic pattern of cells termed lipidic cells embedded in a myxoid stroma rich in glycosaminoglycans. These cells are multinucleated with pink cytoplasm. Typically, they present in clusters surrounding vascular structures. Calcium is present in approximately 10% of patients. Usually, the embolized material has a different pattern from the material at the center of the lesion, and cells resemble embryonic mesenchymal cells, similar to embryonic endocardial cushion tissue.
Teratomas, rarely malignant in children, form from 3 embryonic tissues that usually grow in the anterior mediastinum. These tumors should be differentiated from intrapericardial bronchogenic cysts because they may have the same gross appearance; the histologic difference is that bronchogenic cysts do not contain neuronal tissue, which is usually present in teratomas.
Hemangiomas are classified, based on the predominant type of proliferating vascularity, into hemangiomas (common) or lymphangiomas (extremely uncommon). The tumor contains endothelium-lined spaces that may have blood, lymph, or thrombi.
Malignant tumors
Angiosarcomas have variable vascular channels lined with atypical endothelial cells.
The histologic hallmark of rhabdomyosarcoma is the presence of cross-striations.
Fibrosarcomas are composed of a herringbone pattern of spindle-shaped cells with elongated blunt-ended nuclei and frequent mitoses.
Treatment
Medical Care
Most childhood cardiac tumors are benign, with no treatment necessary unless the tumor severely obstructs blood flow or causes intractable arrhythmias. Most only require close follow-up care.
New data have addressed the use of serum creatine kinase-myocardial band (CK-MB) fraction levels in evaluating ventricular function. Greater ventricular dysfunction correlated with higher CK-MB levels.
Nonetheless, examine the patient extensively and expeditiously because finding a tumor is a stressful situation for both patient and family. By reaching a prompt and precise diagnosis, the physician can proceed with the best treatment approach.
The mode of treatment varies and cannot be easily simplified because the kind of tumor (benign vs malignant, infiltrative vs localized) dictates therapy. Location and extent of the tumor, as well as symptoms, are clinical variables that direct treatment. Observation is sufficient when the mass is small and does not interfere with vascular hemodynamics. However, the clinician must be aggressive when the tumor is causing hemodynamic problems. In occasions, electrical problems may also arise due to the location requiring resection and/or implantation of a pacemaker if atrioventricular block occurs.
Surgical Care
Many advocate excision of the mass as soon as it is found; however, most childhood tumors are benign and do not require resection. Nevertheless, if a tumor is causing severe obstruction or intractable arrhythmias and has been rendered resectable, perform surgery as soon as possible.
- Excision and biopsy: If excision is required, perform it as extensively and completely as possible. Certain tumors recur even after complete excision. An extensive resection carries its own risks since, occasionally, removal of papillary muscles, valves, chordae tendineae, and conduction tissue are necessary.
- Heart transplantation is an option for unresectable benign masses causing hemodynamic compromise. Transplantations have been performed with variable results for primary malignant cardiac tumors in children and adults without evidence of metastases.
Consultations
Consult a cardiologist, thoracic and cardiovascular surgeon, pathologist, radiologist, and hematologist/oncologist.
Diet
No dietary restrictions are needed. Occasionally, certain restrictions such as low-sodium diet for congestive heart failure (CHF) are required.
Activity
Advise no restrictions unless clinical condition merits otherwise.
Medication
Focus medical treatment of a patient with a cardiac neoplasm on symptoms and possible complications resulting from primary disease. Two of the more common complications of patients with heart tumors are development of arrhythmias and congestive heart failure (CHF).
CHF management includes diuretics and afterload reduction. Severe cases with hemodynamic compromise may require intravenous inotropes, afterload reduction, vasodilators, and anticoagulation. Arrhythmias vary; thus, specific antiarrhythmics should be chosen.
Cardiac glycosides
These agents may improve left ventricular function by inhibiting the Na+/K+ -ATPase activity that results in increased myocardial contraction. This leads to sodium accumulation within the myocyte that stimulates the sodium calcium exchange pump. Increased intracellular calcium increases the force of contraction while producing antiarrhythmic effects on cardiac muscle.
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system.
Dosing
Adult
0.125-0.375 mg/d PO
Pediatric
Total digitalizing dose (TDD):
Note: TDD administered within 24 h
Premature infants: 0.02 mg/kg PO divided q8h
Full-term infants: 0.03 mg/kg PO divided q8h
1-24 months: 0.04-0.05 mg/kg PO divided q8h
>2 years: 0.03-0.04 mg/kg PO divided q8h
Maintenance dose:
Infants: 6-8 mcg/kg/d PO
2-5 years: 10-15 mcg/kg/d PO
5-10 years: 7-10 mcg/kg/d PO
>10 years: 3-5 mcg/kg/d PO
<10 years: Divided bid dosing is recommended
Interactions
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Contraindications
Documented hypersensitivity; beriberi heart disease, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, and carotid sinus syndrome
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
TDD may need to be lowered based on drug concentrations obtained; adjust dose for patients with decreased renal function; dosing must be individualized/titrated and serum levels followed; it is arrhythmogenic and interacts with several drugs used commonly to treat arrhythmias; use cautiously in patients with outflow tract obstructions
Hypokalemia may reduce positive inotropic effect of digitalis; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal impairment; highly toxic (overdoses can be fatal)
Diuretic agents
Hypoperfusion of kidneys causes retention of sodium and water, producing peripheral and pulmonary edema. Diuretics decrease intravascular volume overload. They promote excretion of water and electrolytes by the kidneys and are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites. Pay special attention to patients with a combination of CHF and outflow obstruction because a decrement in cardiac output caused by a decrease in cardiac preload produced by diuretics may further compromise patients' conditions.
Furosemide (Lasix)
Loop diuretic that increases excretion of water by interfering with chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. DOC in pediatric patients.
Dosing
Adult
1 mg/kg/dose PO/IV/IM; titrate to effect
Pediatric
0.5-2 mg/kg/dose PO/IV/IM up to tid
Interactions
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide (hearing loss of varying degrees may occur); anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Contraindications
Documented hypersensitivity; hepatic coma, anuria, and state of severe electrolyte depletion
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause profound diuresis and electrolyte loss; metabolic alkalosis is a common complication; should not be given in the same IV line with inamrinone since it may cause precipitation of the compounds; may cause renal stones, especially in premature newborns; coadministration of chlorothiazide may decrease the hypercalciuria
Chlorothiazide (Diuril)
Thiazide diuretic. If given with furosemide, may decrease the hypercalciuria produced by furosemide.
Dosing
Adult
0.5-2 g/d PO or 100-500 mg/d IV
Pediatric
PO:
<6 months: 20-40 mg/kg/d PO divided bid
>6 months: 20 mg/kg/d PO divided bid
IV:
<6 months: 2-8 mg/kg/d IV divided bid
>6 months: 4 mg/kg/d IV divided bid
Interactions
Thiazides may decrease effects of anticoagulants, antigout agents, and sulfonylureas; thiazides may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Contraindications
Documented hypersensitivity; anuria or renal decompensation
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Safety of IV use in children has not been established; may produce electrolyte imbalance; should not be given SC or IM
Spironolactone (Aldactone)
Potassium-sparing diuretic. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.
Dosing
Adult
6.25-25 mg/d PO
Pediatric
2-3 mg/kg/d PO divided bid/tid
Interactions
May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone; concomitant use with indomethacin or ACE inhibitors may cause hyperkalemia
Contraindications
Documented hypersensitivity; anuria, renal failure, or hyperkalemia
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Can cause electrolyte imbalance, especially hyperkalemia; caution in renal and hepatic impairment
Angiotensin converting enzyme inhibitors
ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved. Blood pressure is determined by cardiac output and systemic resistance. When systemic resistance is decreased with afterload reduction, myocardial shortening and stroke volume improve; thus, cardiac output can be maintained at a lower heart rate with lower myocardial oxygen demand. ACE inhibitors decrease production of angiotensin II, a potent vasoconstrictor. As with the diuretics, pay special attention when administering to patients with outflow tract obstruction, since afterload is decreased.
Captopril (Capoten)
Used to reduce afterload. Prevents conversion of Angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion.
Dosing
Adult
6.25-12.5 mg PO tid; not to exceed 150 mg tid
Pediatric
<6 months: 0.05-0.5 mg/kg/dose PO up to tid
>6 months: 0.5-2 mg/kg/dose PO up to tid
Test dose: 0.1 mg/kg/dose PO
Interactions
NSAIDs may reduce hypotensive effects of captopril; may increase digoxin, lithium, and allopurinol levels; rifampin decreases captopril levels; probenecid may increase captopril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics
Contraindications
Documented hypersensitivity; renal impairment
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Dose should be titrated to the patient's tolerance and to effect; dose should be adjusted in patients with renal impairment; may cause idiosyncratic hypotension after first dose in children; test dose should be given and blood pressure monitored frequently after the dose
Adrenergic agonist agents (inotropes)
These agents improve the hemodynamic status by increasing myocardial contractility and heart rate, resulting in increased cardiac output. They also increase peripheral resistance by causing vasoconstriction. Increased cardiac output and increased peripheral resistance lead to increased blood pressure.
Dopamine (Intropin)
Stimulates both adrenergic and dopaminergic receptors. Hemodynamic effect is dependent on the dose. Lower doses predominantly stimulate dopaminergic receptors that, in turn, produce renal and mesenteric vasodilation. Cardiac stimulation and renal vasodilation are produced by higher doses.
After initiating therapy, increase dose by 1-4 mcg/kg/min q10-30min until optimal response is obtained. More than 50% of patients are satisfactorily maintained on doses <20 mcg/kg/min.
Dosing
Adult
2-20 mcg/kg/min IV
Pediatric
Administer as in adults
Interactions
Phenytoin, alpha-adrenergic and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects
Contraindications
Documented hypersensitivity; pheochromocytoma or ventricular fibrillation; subaortic stenosis
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypovolemia should be treated before infusion; extravasation should be treated promptly with SC administration of phentolamine (Regitine); administration through a central vein is recommended; do not use the umbilical artery for infusion; if >20 mcg/kg/min are required, consider a different agent (epinephrine, dobutamine)
Dobutamine (Dobutrex)
Stimulates beta1-adrenergic receptors. Has less alpha1 stimulation than dopamine, producing less increase in systemic vascular resistance.
Dosing
Adult
2-15 mcg/kg/min IV
Pediatric
Administer as in adults
Interactions
Beta-adrenergic blockers antagonize effects of dobutamine; general anesthetics may increase toxicity
Contraindications
Documented hypersensitivity; idiopathic hypertrophic subaortic stenosis and atrial fibrillation or flutter
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Hypovolemia should be treated before infusion; administration through a central vein is recommended; do not use umbilical artery for infusion; may decrease CVP and wedge pressure
Cyclic adenosine monophosphate (cAMP) phosphodiesterase inhibitors
Inotropic effect occurs by inhibiting cAMP phosphodiesterase that increases cellular levels of cAMP. It does not affect the sodium-potassium pump like digitalis. Vasodilatory activity is related to direct relaxation effect on vascular smooth muscle.
Inamrinone - formerly amrinone (Inocor)
Produces vasodilation and increases inotropic state. More likely to cause tachycardia than dobutamine; may exacerbate myocardial ischemia.
Dosing
Adult
0.75 mg/kg IV bolus slowly over 2-3 min; maintenance infusion is 5-10 mcg/kg/min; not to exceed a cumulative dose of 10 mg/kg/d
Adjust dose according to patient response
Pediatric
Administer as in adults
Interactions
Coadministration with diuretics may result in hypovolemia and a decrease in filling pressure; cardiac glycosides have additive effects on inamrinone; inamrinone precipitates in presence of furosemide
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May aggravate outflow tract obstructions; should not be diluted in glucose-containing solutions; discontinue therapy if symptoms of liver toxicity develop; correct hypokalemic states before giving therapy
Milrinone (Primacor)
Bipyridine positive inotrope and vasodilator with little chronotropic activity. Different in mode of action from both digitalis glycosides and catecholamines.
Dosing
Adult
50 mcg/kg IV loading dose over 10 min, followed by continuous infusion at 0.375-0.75 mcg/kg/min
Pediatric
Administer as in adults
Interactions
Milrinone precipitates in presence of furosemide
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor fluids, electrolyte changes, and renal function during therapy; excessive diuresis may increase potassium loss and predispose digitalized patients to arrhythmias; important to correct hypokalemia with potassium supplementation prior to treatment; patients showing excessive decreases in blood pressure should have infusion rates slowed or stopped; previous vigorous diuretic therapy has caused significant decreases in cardiac filling pressure (cautiously administer milrinone and monitor blood pressure, heart rate, and clinical symptomatology)
Antiarrhythmic agents
These agents alter the electrophysiologic mechanisms responsible for arrhythmia.
Procainamide (Procan, Procanbid)
Class I-A antiarrhythmic used for PVCs. Increases refractory period of the atria and ventricles. Myocardiac excitability is reduced by an increase in the threshold for excitation and by inhibition of ectopic pacemaker activity.
Dosing
Adult
Loading dose: 17 mg/kg IV infused over 1 h; followed by 1-6 mg/min IV
PO maintenance dose: 2-4 g/d PO in divided doses
Pediatric
17 mg/kg IV infused over 1 h; followed by 20-80 mcg/kg/min IV
PO maintenance dose: 15-50 mg/kg PO in divided doses
Interactions
Can expect increased levels of procainamide metabolite NAPA in patients taking cimetidine, ranitidine, beta-blockers, amiodarone, trimethoprim, and quinidine; procainamide may increase effect of skeletal muscle relaxants, quinidine and lidocaine, and neuromuscular blockers; ofloxacin inhibits tubular secretion of procainamide and may increase bioavailability; when taken concurrently with sparfloxacin, may increase risk of cardiotoxicity
Contraindications
Documented hypersensitivity; complete heart block or second-degree or third-degree heart block, if a pacemaker is not in place; torsade de pointes; systemic lupus erythematosus
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause QRS widening and ventricular arrhythmias; continuously monitor during first administration; blood dyscrasias have been reported during the administration of this medication; monitor blood levels; lower the dose when administering to patients with renal dysfunction
Lidocaine (Dilocaine, Xylocaine)
Class IB antiarrhythmic that increases the electrical stimulation threshold of the ventricle, suppressing automaticity of conduction through the tissue. Could be used IV/ET/IO.
Dosing
Adult
1-1.5 mg/kg IV infused over 2-3 min; may repeat doses of 0.5-0.75 mg/kg q5-10min; not to exceed a cumulative dose of 3 mg/kg; followed by 2-4 mg/min IV
Pediatric
1-1.5 mg/kg IV infused over 2-3 min; may repeat doses of 0.5-0.75 mg/kg q5-10min; not to exceed a cumulative dose of 3 mg/kg; followed by 20-40 mcg/kg/min IV
Interactions
Coadministration with cimetidine or beta-blockers increases toxicity of lidocaine; coadministration with procainamide and tocainide may result in additive cardiodepressant action; may increase effects of succinylcholine
Contraindications
Documented hypersensitivity; Adams-Stokes syndrome/attacks and Wolff-Parkinson-White syndrome; severe sinoatrial, AV, or intraventricular block if artificial pacemaker not in place
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Use a solution without preservatives; caution in heart failure, hepatic disease, hypoxia, hypovolemia or shock, respiratory depression, and bradycardia; may increase risk of CNS and cardiac adverse effects in elderly patients; high plasma concentrations can cause seizures, heart block, and AV conduction abnormalities
Propranolol (Inderal, Betachron E-R)
Class II antiarrhythmic, nonselective, beta-adrenergic receptor blocker with membrane-stabilizing activity that decreases automaticity of contractions. May be used to treat supraventricular as well as ventricular tachyarrhythmias.
Dosing
Adult
1 mg/dose IV, may repeat q5min; not to exceed a cumulative dose of 5 mg
Maintenance dose: 40-320 mg/d PO in divided doses
Pediatric
0.01-0.1 mg/kg/dose IV infused over 10-15 min, may repeat q6-8h; not to exceed a cumulative dose of 1 mg for infants or 3 mg for children
Maintenance dose: 0.5-4 mg/kg/d PO divided q6-8h
Interactions
Coadministration with aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease propranolol effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity of propranolol; toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines may increase with propranolol
Contraindications
Documented hypersensitivity; uncompensated CHF; bradycardia; cardiogenic shock; AV conduction abnormalities
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Beta-adrenergic blockade may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; slowly withdraw drug and closely monitor
Sotalol (Betapace)
Currently under investigation for use in children. It has class II (ie, beta-blocking effects) and class III action potential prolongation properties. Less negative inotropic effect than class II antiarrhythmics.
Dosing
Adult
80 mg PO bid, may increase dose gradually q2-3d to 240-320 mg/d; not to exceed 320 mg/d
Pediatric
Not established
Interactions
Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels, possibly resulting in decreased pharmacologic effect; cardiotoxicity of sotalol may increase when administered concurrently with other drugs that prolong the QT interval (eg, sparfloxacin, astemizole, calcium channel blockers, quinidine, flecainide, phenothiazines, cisapride, dofetilide)
Contraindications
Documented hypersensitivity; asthma; severe sinus bradycardia; second- or third-degree AV block
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Medication should be initiated in hospital under monitored conditions; beta-adrenergic blockade may decrease signs and symptoms of acute hypoglycemia and clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; slowly withdraw drug and closely monitor patient; caution in hypokalemia, peripheral vascular disease, hypomagnesemia, and CHF
Verapamil (Calan, Isoptin)
Calcium channel blocker. Elicits negative inotropic effect. Can diminish PVCs associated with perfusion therapy and decrease the risk of ventricular fibrillation and ventricular tachycardia. By interrupting reentry at AV node, verapamil can restore normal sinus rhythm in patients with paroxysmal supraventricular tachycardias (PSVT).
Dosing
Adult
80-120 mg PO tid; not to exceed 480 mg/d
Pediatric
<2 years: Contraindicated
>2 years: 4-10 mg/kg/d PO divided tid; alternatively, 0.1-0.3 mg/kg IV over 3 min; not to exceed 5 mg/dose
Interactions
Verapamil may increase carbamazepine, digoxin, and cyclosporine levels; coadministration with amiodarone can cause bradycardia and a decrease in cardiac output; when administered concurrently with beta-blockers, may increase cardiac depression; cimetidine may increase verapamil levels; verapamil may increase theophylline levels
Contraindications
Documented hypersensitivity; severe CHF, sick sinus syndrome or second- or third-degree AV block, and hypotension (<90 mm Hg systolic); age <2 years
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution if coadministered with a beta-blocker; may cause severe hypotension in children; hepatocellular injury may occur; transient elevations of transaminases with and without concomitant elevations in alkaline phosphatase and bilirubin have occurred (elevations have been transient and may disappear with continued verapamil treatment); periodically monitor liver function
Follow-up
Further Inpatient Care
- Discharge patients with cardiac tumors when they are stable.
Further Outpatient Care
- Provide close follow-up care to diagnose recurrence of malignant tumors after resection.
- Monitor ventricular function.
- Monitor medication doses and effects.
Inpatient & Outpatient Medications
- Medications, when indicated, include digitalis, afterload reduction agents, diuretics, antiarrhythmics, and anticoagulants.
Transfer
- Transfer to a facility with the necessary specialties and consultants.
Complications
- Complications include arrhythmia, congestive heart failure (CHF), thromboembolism, decrease in ventricular function, and seeding during surgical removal (metastasis).
- For complications related to specific types of cardiac tumors, see Background.
Prognosis
- Studies provide a wide spectrum of morbidity and mortality statistics.
- Survival rates depend on the type of tumor.
- Benign tumors have a good prognosis unless they are associated with severe obstruction or intractable arrhythmias, which have a slightly higher risk of mortality.
- For primary cardiac malignant tumors, the prognosis is invariably poor.
- Metastatic neoplasms to the heart are more common than primary cardiac neoplasms; the prognosis is dependent on the histologic type of tumor, and survival rates are based on original pathology.
- Certain tumors are known to recur even after resection.
- Most children with cardiac tumors have an excellent prognosis and do not need any intervention.
Miscellaneous
Medicolegal Pitfalls
- Failure to make the diagnosis of cardiac tumor in a timely manner
Multimedia
Media file 1: Echocardiographic parasternal long-axis view showing a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. AO=Aorta, RV=Right ventricle, LA=Left atrium, Arrow=Left ventricular mass.
Media file 2: Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
Media file 3: Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
Media file 4: Echocardiographic apical 5 chamber view of a 1 month old patient with several ventricular tumors filling the ventricular cavities in both sides. The patient was having seizures and diagnosed to have tuberous sclerosis. The tumor associated with this disease is rhabdomyoma (see text). RA= Right Atrium; Ao= Aorta; LA= Left Atrium; RV= Right Atrium; LV= Left Ventricle
Media file 5: Follow-up echocardiographic 4 chamber view from the same patient as in Media file 4. This echocardiogram was done 8 months after the previous view. Note that the tumors have changed in size and some of them have regressed totally, especially in the right ventricle. The arrows show the remaining areas of tumors seen in both ventricles.
References
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Keywords
cardiac tumors, cardiac neoplasm, heart tumors, heart neoplasm, neoplasm of the heart, rhabdomyoma, fibroma, myxoma, teratoma, sarcoma, angiosarcoma, cancer, cardiac mass, congenital heart failure, CHF, jugular venous distention, ascites, pericardial effusion, syncope, shortness of breath, vena cava syndrome, pulmonary embolism, restrictive cardiomyopathy, tuberous sclerosis, Carney syndrome, vascular tumors, hemangiomas, lymphangiomas, heart block, lipomas, papillary tumors, leiomyomas, mesotheliomas, fibroelastomas, fibroelastic papillomas, benign cystic tumors, rhabdomyosarcoma
Contributor Information and Disclosures
Author
Edwin Rodriguez-Cruz, MD, Assistant Professor, Department of Pediatrics, San Juan Bautista Medical School and Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Pediatrics, Hospital El Maestro and San Juan Bautista Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Cardiology, Cardiovascular Center of Puerto Rico and the Caribbean and Veterans Affairs Hospital and Medical Center of Puerto Rico
Edwin Rodriguez-Cruz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, American Society of Echocardiography, Puerto Rico Medical Association, Society of Cardiac Angiography and Interventions, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.
Coauthor(s)
Rosa M Cintrón-Maldonado, MD, Clinical Instructor of Pediatrics, Department of Pediatrics, San Juan Bautista Medical Center
Rosa M Cintrón-Maldonado, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.
Robert D Ross, MD, Co-Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Division of Pediatric Cardiology, Professor, Children's Hospital of Michigan and Wayne State University
Robert D Ross, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.
Medical Editor
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.
Pharmacy Editor
Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation
Managing Editor
Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.
CME Editor
Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.
Chief Editor
Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.
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