Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study

John P. Jones, Tristan Ramcharan, Milind Chaudhari, Vinay Bhole, Karen Mcleod, Shankar Sadagopan, Orhan Uzun, Andrew Parry, Jan Till, Colin J. McMahon, Alan G. Stuart, Mark A. Walsh*

*Corresponding author for this work

Research output: Contribution to journalArticle (Academic Journal)

5 Citations (Scopus)

Abstract

Background: Although ventricular fibromas are rare, they are the second most common type of cardiac tumor in children. While histologically benign, they have a propensity to cause malignant arrhythmias, with cardiac arrest often being the first presentation. Objective: The purpose of this study was to evaluate the arrhythmia risk and management strategies for pediatric ventricular fibromas. Methods: Fifteen centers in the British Paediatric Arrhythmia Group network were contacted to partake in the study to contribute cases. A detailed database search was performed at 2 hospitals for cases of ventricular fibromas. Results: A total of 19 patients were included in the study. Arrhythmias were common, with 5 patients presenting with cardiac arrest and 5 others having documented ventricular tachycardia. Nine patients have undergone surgical resection at various hospitals, and all these patients have survived with good long-term outcomes. One patient who did not have any treatment died, presumably of a ventricular arrhythmia; another died of metastatic disease. There were no recurrences of arrhythmia after surgery, and the need for a defibrillator was alleviated in all cases. Conclusion: Ventricular fibromas have a high propensity to cause malignant arrhythmias, and if they are not managed appropriately, mortality is high. The outcomes of surgical resection are good, regardless of size, and this represents the best therapeutic option, with most patients being symptom free in the longer term.

Original languageEnglish
Pages (from-to)1507-1512
Number of pages6
JournalHeart Rhythm
Volume15
Issue number10
DOIs
Publication statusPublished - 1 Oct 2018

Keywords

  • Arrhythmia
  • Cardiac arrest
  • Pediatric
  • Ventricular fibrillation
  • Ventricular fibroma

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