Pediatric primary cardiac tumors: Diagnosis and treatment

  • Avihu Z. Gazit
  • Sanjiv K. Gandhi

Opinion statement

Primary cardiac tumors are rare in pediatrics. Their clinical presentation differs among the various age groups (fetus to the young adult). Our discussion focuses on the diagnosis and treatment of rhabdomyomas, fibromas, myxomas, and pericardial teratomas in the fetus and neonatal stages. Fetal diagnosis has been described in association with rhabdomyomas, fibromas, and pericardial teratomas. It is made by echocardiography prompted by fetal dysrhythmias, nonimmune hydrops, intrauterine growth restriction, and familial tuberous sclerosis. Based on our experience, it is of the utmost importance to refer these patients to a tertiary center for detailed evaluation, follow-up, and delivery. Tumor diagnosis in the neonatal age group may be prompted by a murmur, dysrhythmia, conduction block, and hemodynamic compromise or hypoxemia due to right or left inflow or outflow tract obstruction. Prenatal diagnosis of a hemodynamically significant mass mandates the presence of a neonatal intensivist in the delivery room for prompt initiation of advanced life support (intubation, mechanical ventilation, and central venous access), and early initiation of prostaglandin E1 to establish ductus arteriosus patency. Severe inflow obstruction associated with a restrictive atrial septum may require balloon atrial septostomy. Emergent transvenous pacing is indicated in the face of high-degree atrioventricular block. Early surgical removal of the mass after initial stabilization in the neonatal or cardiac intensive care unit may be indicated.


Left Atrium Teratoma Patent Ductus Arteriosus Main Side Effect Milrinone 
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References and Recommended Reading

  1. 1.
    Fyler DC: Report of the New England Regional Infant Cardiac Program. Pediatrics 1980, 65:375–461.Google Scholar
  2. 2.
    Reynen K: Frequency of primary tumors of the heart. Am J Cardiol 1996, 77:107.PubMedCrossRefGoogle Scholar
  3. 3.
    Marx GR, Moran AM: Cardiac tumors. In Moss and Adams’ Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult, edn 6. Edited by Allen HD, et al. Philadelphia: Lippincott, Williams & Wilkins; 2001:1432–1445.Google Scholar
  4. 4.
    Beghetti M, Gow RM, Haney I, et al.: Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J 1997, 134:1107–1114.PubMedCrossRefGoogle Scholar
  5. 5.
    Becker AE: Primary Heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 2000, 21:317–323.PubMedCrossRefGoogle Scholar
  6. 6.
    Gazit AZ, Singh GK, Shumway J, et al.: Fetal cardiac rhabdomyoma: a sheep or a wolf? J Matern Fetal Neonatal Med 2007, 20:343–348.PubMedCrossRefGoogle Scholar
  7. 7.
    Elderkin RA, Radford DJ: Primary cardiac tumours in a paediatric population. J Paediatr Child Health 2002, 38:173–177.PubMedCrossRefGoogle Scholar
  8. 8.
    de-Montpreville VT, Nottin R, Dulmet E, Serraf A: Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center. Cardiovasc Pathol 2007, 16:22–28.CrossRefGoogle Scholar
  9. 9.
    Freedom RM, Lee KJ, MacDonald C, Taylor G: Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol 2000, 21:299–316.PubMedCrossRefGoogle Scholar
  10. 10.
    Zhou QC, Fan P, Peng QH, et al.: Prenatal echocardiographic differential diagnosis of fetal cardiac tumors. Ultrasound Obstet Gynecol 2004, 23:165–171.PubMedCrossRefGoogle Scholar
  11. 11.
    Burke A, Virmani R: Tumors of the heart and great vessels. In Atlas of Tumor Pathology. Armed Forces Institute of Pathology. Washington DC: 1996:series 3, fascicle 16.Google Scholar
  12. 12.
    Isaacs H Jr: Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004, 25:252–273.PubMedCrossRefGoogle Scholar
  13. 13.
    Al-Mateen M, Hood M, Trippel D, et al.: Cerebral embolism from atrial myxoma in pediatric patients. Pediatrics 2003, 112:162–167.CrossRefGoogle Scholar
  14. 14.
    Farooki ZQ, Hakimi M, Arciniegas E, et al.: Echocardiographic features in a case of intrapericardial teratoma. J Clin Ultrasound 1978, 6:108–110.PubMedCrossRefGoogle Scholar
  15. 15.
    de Bustamante TD, Azpeitia J, Miralles M, et al.: Prenatal sonographic detection of pericardial teratoma. J Clin Ultrasound 2000, 28:194–198.PubMedCrossRefGoogle Scholar
  16. 16.
    Butany J, Nair V, Nassemudin A, et al.: Cardiac tumours: diagnosis and management. Lancet Oncol 2005, 6:219–228.PubMedCrossRefGoogle Scholar
  17. 17.
    Gulati G, Sharma S, Kothari SS, et al.: Comparison of Echo and MRI in the imaging evaluation of intracardiac masses. Cardiovasc Intervent Radiol 2004, 27:459–469.PubMedCrossRefGoogle Scholar
  18. 18.
    Heymann MA: Pharmacologic use of prostaglandin E1 in infants with congenital heart disease. Am Heart J 1981, 101:837–843.PubMedCrossRefGoogle Scholar
  19. 19.
    Marino BS, Wernovski G: Preoperative care. In Pediatric Cardiac Intensive Care. Edited by Chang AC, et al. Baltimore, MD: Lippincott, Williams & Wilkins; 1998:151–162.Google Scholar
  20. 20.
    Westfall TC, Westfall DP: Adrenergic agonists and antagonists. In Goodman & Gilman’s the Pharmacologic Basis of Therapeutics. Edited by Brunton LL, Lazo IS, Parker KL. New York: McGraw Hill; 2005:237–295.Google Scholar
  21. 21.
    Siwy BK, Sadove AM: Acute management of dopamine infiltration injury with Regitine. Plast Reconstr Surg 1987, 80:610–612.PubMedCrossRefGoogle Scholar
  22. 22.
    Notterman DA: Inotropic agents, catecholamines, digoxin, amrinone. Crit Care Clin 1991, 7:583–613.PubMedGoogle Scholar
  23. 23.
    Johnston RR, Eger EI II, Wilson C: A comparative interaction of epinephrine with enflurane, isoflurane, and halothane in man. Anesth Analg 1976, 55:709–712.PubMedCrossRefGoogle Scholar
  24. 24.
    Hoffman TM, Wernovsky G, Atz AM, et al.: Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003, 107:996–1002.PubMedCrossRefGoogle Scholar
  25. 25.
    Edelson J, Stroshane R, Benziger DP, et al.: Pharmacokinetics of the bipyridines amrinone and milrinone. Circulation 1986, 73(3 Pt 2):III145–III152.PubMedGoogle Scholar
  26. 26.
    Syverud SA, Dalsey WC, Hedges JR: Transcutaneous and transvenous cardiac pacing for early bradyasystolic cardiac arrest. Ann Emerg Med 1986, 15:121–124.PubMedCrossRefGoogle Scholar
  27. 27.
    Lumia FJ, Rios JC: Temporary transvenous pacemaker therapy: an analysis of complications. Chest 1973, 64:604–608.PubMedCrossRefGoogle Scholar
  28. 28.
    Austin JL, Preis LK, Crampton RS, et al.: Analysis of pacemaker malfunction and complications of temporary pacing in the coronary care unit. Am J Cardiol 1982, 49:301–306.PubMedCrossRefGoogle Scholar
  29. 29.
    Takach TJ, Reul GJ, Ott DA, Cooley DA: Primary cardiac tumors in infants and children: immediate and long-term operative results. Ann Thorac Surg 1996, 62:559–564.PubMedCrossRefGoogle Scholar
  30. 30.
    Jamieson SW, Gaudiani VA, Reitz BA, et al.: Operative treatment of an unresectable tumor of the left ventricle. J Thorac Cardiovasc Surg 1981, 81:797–799.PubMedGoogle Scholar
  31. 31.
    Giamberti A, Giannico S, Squitieri C, et al.: Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve. Ann Thorac Surg 1995, 59:1219–1221.PubMedCrossRefGoogle Scholar
  32. 32.
    Reddy SC, Fenton KM, Gandhi SK, et al.: Intrapericardial teratoma in a neonate. Ann Thorac Surg 2003, 76:626.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  1. 1.Department of PediatricsWashington University in Saint LouisSt. LouisUSA

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