Objectives: The aim of this study was to describe a large experience with primary cardiac tumors in pediatric patients, characterize associated arrhythmias, and expand knowledge of natural history and treatment options.
Background: Primary cardiac tumors in children are rare. The incidence of arrhythmias is not well-defined, and management plans vary widely.
Methods: We employed a retrospective single-center review of patients ≤21 years of age diagnosed with a primary cardiac tumor between 1968 and 2010. Clinically significant arrhythmias were defined as: 1) sudden cardiac arrest; 2) nonsustained and sustained ventricular tachycardia (VT); 3) pre-excitation; and 4) sustained supraventricular tachycardia of any mechanism.
Results: A total of 173 patients were identified: 106 rhabdomyoma, 25 fibroma, 14 myxoma, 6 vascular, 4 teratoma, 3 lipoma, and 15 other. Median age at diagnosis was 7 months (prenatal to 21 years). Of these, 42 (24%) had clinically significant arrhythmias. Patients with large fibromas were the highest-risk group, with VT occurring in 64%. Among rhabdomyoma patients, 10% had pre-excitation, and 6% had VT. Over a mean follow-up of 6 years (1 day to 34 years, median 4 years), surgical excision was performed in 62 cases, with rhythm treatment being 1 of the indications in 20. Post-operatively, clinically significant arrhythmias were eliminated in 18 of these 20, including all 13 fibroma patients.
Conclusions: Clinically significant arrhythmias occurred in 24% of pediatric patients with cardiac tumors, VT being the most common type. Surgical excision for VT associated with rhabdomyomas and fibromas in selected patients is an important and effective management strategy in these patients.
Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.