title: Pediatric primary cardiac tumors: diagnosis and treatment.
abstract: 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 E(1) 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.
authors: Department of Pediatrics, Washington University in Saint Louis, One Children's Place, NWT Box 8116, St. Louis, MO 63110, USA. gazit_a@kids.wustl.edu
authors: Avihu Z
authors: AZ
authors: Gazit
authors: Sanjiv K
authors: SK
authors: Gandhi