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Published on: February 6, 2019
Pediatric primary cardiac tumors: diagnosis and treatment
Avihu Z Gazit1, Sanjiv K Gandhi
1Department of Pediatrics, Washington University in Saint Louis, One Children's Place, NWT Box 8116, St. Louis, MO 63110, USA. gazit_a@kids.wustl.edu
Insights
Pediatric primary cardiac tumors like rhabdomyomas and fibromas are rare, with distinct fetal and neonatal presentations. Early diagnosis and specialized care at tertiary centers are crucial for optimal outcomes.
Area of Science:
- Pediatric Cardiology
- Fetal and Neonatal Medicine
- Pediatric Oncology
Background:
- Primary cardiac tumors are uncommon in children.
- Clinical manifestations vary significantly across pediatric age groups, from fetus to young adult.
- This review focuses on specific tumors: rhabdomyomas, fibromas, myxomas, and pericardial teratomas in fetuses and neonates.
Purpose of the Study:
- To outline the diagnostic approaches for fetal and neonatal primary cardiac tumors.
- To describe the management strategies for these rare pediatric conditions.
- To emphasize the importance of specialized care in tertiary centers.
Main Methods:
- Review of diagnostic methods including echocardiography for fetal diagnosis.
- Discussion of clinical presentations prompting neonatal diagnosis (murmur, dysrhythmia, obstruction).
- Outline of emergent interventions and surgical considerations.
Main Results:
- Fetal diagnosis is possible for rhabdomyomas, fibromas, and pericardial teratomas, often linked to specific fetal conditions.
- Neonatal diagnosis may be triggered by symptoms of cardiac compromise or obstruction.
- Prenatal diagnosis of significant masses requires immediate neonatal intensive care and potential interventions like prostaglandin E(1) or balloon atrial septostomy.
Conclusions:
- Referral to a tertiary center is essential for comprehensive evaluation and management of fetal and neonatal cardiac tumors.
- Prompt multidisciplinary intervention, including advanced life support and potential surgery, is critical for neonates with hemodynamically significant tumors.
- Early diagnosis and management significantly impact the prognosis for pediatric patients with primary cardiac tumors.
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.
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