Pediatric Cardiac Tumors Clinical Presentation

  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Dec 1, 2011
 

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.[2]
  • 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.
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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 (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 demonEchocardiographic 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 withEchocardiographic 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.
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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.[3] 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.

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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: NOVARTIS Grant/research funds INVESTIGATOR

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  Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Michigan; Professor of Pediatrics, Wayne State University School of Medicine

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.

Specialty Editor Board

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, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z 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.

References

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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 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 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 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 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.
 
 
 
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