Pediatric Cardiac Tumors 

Updated: Jan 04, 2016
Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Syamasundar Rao Patnana, MD 

Overview

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.[1]

The image below demonstrates a large tumor in the left ventricular cavity of the heart.

Echocardiographic parasternal long-axis view showi 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.

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 (see image below), followed by fibroma, myxoma, and teratoma.[2] 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.

Echocardiographic apical 5 chamber view of a 1 mon 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

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 as depicted in the images below. 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.[3]

Echocardiographic apical 5 chamber view of a 1 mon 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
Follow-up echocardiographic 4 chamber view from th Follow-up echocardiographic 4 chamber view from the same patient as shown in the image above. 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.

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.

Epidemiology

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.[4]

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.

 

Presentation

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.

Note the following:

  • Initial symptoms in infants may include irritability, periodic episodes of pallor, fever, tachypnea, tachycardia, anorexia, and failure to thrive.[5]

  • 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).

Note the following:

  • Right-sided tumors (see image below): 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.

    Echocardiographic parasternal long-axis view demon 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.
  • Left-sided tumors (see image below): With the ability to embolize, these tumors can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents.

    Echocardiographic subcostal view of a patient with 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.
  • 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.[6] 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.[7] 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.

 

DDx

 

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. Refer to the following images

Echocardiographic parasternal long-axis view showi 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.
Echocardiographic subcostal view of a patient with 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.
Echocardiographic parasternal long-axis view demon 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.
Echocardiographic apical 5 chamber view of a 1 mon 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
Follow-up echocardiographic 4 chamber view from th Follow-up echocardiographic 4 chamber view from the same patient as shown in the image above. 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.

Echocardiography is very sensitive but not specific. Some of these tumors may be too small for echocardiographic detection, especially those leading to dysrhythmias.

Transesophageal echocardiography 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.

Magnetic resonance imaging

Magnetic resonance imaging (MRI) is a powerful tool in diagnosing tumors, evaluating their extent, and seeking metastases.[8] 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.

Computed tomography scanning

Ultrafast computed tomography (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.

Nuclear medicine imaging

Nuclear medicine studies are used in children to evaluate congenital and developmental disorders of the chest. The same radiopharmaceuticals and imaging techniques are used to evaluate pediatric patients as are used in adults to evaluate cardiac and pulmonary disease, aerodigestive disorders, and pediatric malignancies.[9]

Other Tests

Electrocardiography

Nonspecific changes are the most common findings on electrocardiography (ECG); 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.[10]

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.

Consultations

Consult a cardiologist, thoracic and cardiovascular surgeon, pathologist, radiologist, and hematologist/oncologist.

Transfer

Transfer to a facility with the necessary specialties and consultants.

Diet and activity

No dietary restrictions are needed. Occasionally, certain restrictions such as low-sodium diet for congestive heart failure (CHF) are required.

Advise no restrictions unless the patient's clinical condition merits otherwise.

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.[11]

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.

Cardiac transplantation

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.

 

Medication

Medication Summary

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

Class Summary

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.

Diuretic agents

Class Summary

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.

Chlorothiazide (Diuril)

Thiazide diuretic. If given with furosemide, may decrease the hypercalciuria produced by furosemide.

Spironolactone (Aldactone)

Potassium-sparing diuretic. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Angiotensin converting enzyme inhibitors

Class Summary

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.

Adrenergic agonist agents (inotropes)

Class Summary

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.

Dobutamine (Dobutrex)

Stimulates beta1-adrenergic receptors. Has less alpha1 stimulation than dopamine, producing less increase in systemic vascular resistance.

Cyclic adenosine monophosphate (cAMP) phosphodiesterase inhibitors

Class Summary

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

Produces vasodilation and increases inotropic state. More likely to cause tachycardia than dobutamine; may exacerbate myocardial ischemia.

Milrinone (Primacor)

Bipyridine positive inotrope and vasodilator with little chronotropic activity. Different in mode of action from both digitalis glycosides and catecholamines.

Antiarrhythmic agents

Class Summary

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.

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.

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.

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.

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).

 

Follow-up

Further Outpatient Care

Provide close follow-up care to diagnose recurrence of malignant tumors after resection.

Monitor ventricular function, as well as monitor medication doses and effects.

Further Inpatient Care

Discharge patients with cardiac tumors when they are stable.

Inpatient & Outpatient Medications

Medications, when indicated, include digitalis, afterload reduction agents, diuretics, antiarrhythmics, and anticoagulants.

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, such as the following:

  • 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.