John P Jones1, Tristan Ramcharan2, Milind Chaudhari2, Vinay Bhole2, Karen Mcleod3, Shankar Sadagopan4, Orhan Uzun5, Andrew Parry1, Jan Till6, Colin J McMahon7, Alan G Stuart1, Mark A Walsh8. 1. Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom; Bristol Heart Institute, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom. 2. Department of Paediatric Cardiology, Birmingham Children's Hospital, Birmingham, United Kingdom. 3. Department of Cardiology, Royal Hospital for Sick Children, Glasgow, United Kingdom. 4. Department of Paediatric Cardiology, Southampton General Hospital, Southampton, United Kingdom. 5. Department of Cardiology, University Hospital Wales, Cardiff, United Kingdom. 6. Department of Paediatric Cardiology, Royal Brompton & Harefield NHS Foundation Trust, London, United Kingdom. 7. Department of Paediatric Cardiology, Our Lady's Hospital Crumlin, Dublin, Republic of Ireland. 8. Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom; Bristol Heart Institute, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom. Electronic address: mark.walsh4@nhs.net.
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.
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.