Abstract
Objective
To describe our surgical techniques and early outcomes after resection of cardiac fibromas in symptomatic children.
Methods
A retrospective analysis was performed including all patients who underwent surgical resection of cardiac fibroma at a single institution between May 2021 and April 2024. Parameters reviewed were age, weight, genetic predisposition, clinical presentations, and results of investigations, including 3-dimensional virtual reality imaging. Completeness of resection, intraoperative complications, immediate postoperative outcomes and follow-up parameters including redo operations, arrhythmias, and mortality were analyzed.
Results
The median age at surgery was 12 months (interquartile range [IQR], 8-129 months), and the median weight was 8.7 kg (IQR, 7.5-48.1 kg). The most common clinical presentation was ventricular arrhythmia (n = 5), and the left ventricular chamber was involved in all cases. Transmural resection was done in 5 cases, and complete resection was possible in 3. The cavity after resection was obliterated by layered closure using polypropylene. Loop recorders were implanted simultaneously in 4 cases. All patients had normal sinus rhythm at discharge, and the mean left ventricular ejection fraction was 55 ± 7%. The median duration of follow-up was 12 months (IQR, 11-32 months). One patient had recurrent arrhythmia and underwent reoperation owing to growth of the residual tumor. One patient with incomplete resection required implantation of an implantable cardioverter-defibrillator at 30 months after surgery. All patients were on anti-arrhythmic drugs and had a normal ECG on loop recorder analysis.
Conclusions
Complete resection of cardiac fibroma is preferred but can be limited by tumor location. Arrhythmia-free survival can be achieved in most cases.
Key Words: cardiac fibroma, transmural resection, arrhythmia, pediatric, ventricular fibrillation
Complete resection minimizes recurrence; surgery can achieve arrhythmia-free survival.
Central Message.
Complete resection is preferred, although it may be restricted by tumor location. In most cases, arrhythmia-free survival can be achieved. Surgical removal is associated with minimal adverse events.
Perspective.
Cardiac fibroma is a rare benign tumor in children that carries a risk of fatal arrhythmias and sudden death. Imaging studies such as echocardiography, CT, and MRI are crucial for surgical planning. Complete resection prevents recurrence, but partial removal is considered when vital structures are involved. Surgery is associated with low mortality, and recurrence may present as new arrhythmia or sudden collapse.
Cardiac fibroma is a rare, benign tumor that affects predominantly infants and children. It is the second most common primary cardiac tumor in children after rhabdomyoma. These tumors originate from fibroblasts and myofibroblasts, forming connective tissue masses.1, 2, 3 Symptoms of cardiac fibromas are linked to intracardiac obstruction, conduction system disturbances, valvular dysfunction, embolization, and systemic manifestations. There is a significant risk of ventricular arrhythmias, with cardiac arrest a common presentation. The rarity of this condition poses unique challenges in determining the best clinical management practices.4,5
Various strategies have been described for managing cardiac fibromas, including surgical resection, single ventricle palliation, cardiac transplantation, and conservative management.6 Although the long-term risk of sustained ventricular arrhythmias after surgery is unknown, surgical resection has been shown to offer curative treatment in some reports, reducing the need for long-term antiarrhythmic medications and defibrillator placement.7 Although this practice is well established in some centers, even for the largest fibromas, it is less common in the United Kingdom. Here we describe our early outcomes following the surgical excision of cardiac fibromas.
Methods
This study is a retrospective single-unit analysis of all patients who underwent surgical resection of cardiac fibroma between May 2021 and April 2024. Demographic and patient-related data were obtained from hospital records. Preoperative parameters, such as age at presentation, weight at surgery, genetic predisposition, clinical presentation, and results of investigations including 12-lead electrocardiogram, 24-hour Holter monitoring, echocardiography, computed tomography (CT) scans, and magnetic resonance imaging (MRI), were reviewed. Additionally, a virtual reality model was generated to review the anatomy of the mass and its adjacent structures and to simulate the operation in a virtual space.
Surgical details, including the duration of cardiopulmonary bypass (CPB), cross-clamp time, completeness of resection, and intraoperative complications, were analyzed. Immediate postoperative outcomes, including the duration of ventilation, intensive care unit stay, hospital stay, and postoperative complications, were reviewed. Follow-up data, such as redo operations, arrhythmias, and mortality, were analyzed. This study was deemed exempt from ethical review by the local Institutional Review Board, as it involved retrospective analysis of anonymized clinical data. Informed written consent was not required, as no identifiable patient information was included and the study met the institutional criteria for exemption.
Results
Between May 2021 and April 2024, 7 patients underwent surgery for cardiac fibroma. The median age at the time of surgery was 12 months (IQR, 8-129 months), and the median weight was 8.7 kg (IQR, 7.5-48.1 kg). The most common clinical presentations were ventricular arrhythmia (n = 5) and sudden collapse necessitating cardiopulmonary resuscitation (n = 3) (Table 1). One patient had left ventricular outflow obstruction, and another patient exhibited features of cardiac failure. None of the patients was diagnosed antenatally or had any specific genetic predisposition. A 24-hour Holter monitor revealed multifocal ventricular tachycardia and ectopic beats in 4 patients.
Table 1.
Preoperative and intraoperative parameters of the 7 study patients
| Patient | Age, mo | Weight, kg | Presenting symptoms | Chambers involved | Preop biopsy | Transmural resection | Complete resection | Open chest/ECMO |
|---|---|---|---|---|---|---|---|---|
| 1 | 129 | 34.5 | Syncope/arrhythmia | IVS + left and right ventricles | No | Transmural | No | |
| 2 | 7 | 8.74 | Collapse, CPR | Anterior septum and anterolateral walls, from base to apex | No | Transmural | No | |
| 3 | 185 | 48.1 | Arrhythmia | Posterior inferior LV wall | No | Transmural | Yes | |
| 4 | 109 | 70.25 | Arrhythmia | LV fibroma extending from the base to the inferior surface | Yes | No | No | Open chest |
| 5 | 12 | 7.5 | Tachypnea, pericardial effusion | LV lateral wall | Yes | No | Yes | |
| 6 | 8 | 5.9 | Collapse, CPR/VF | LV lateral wall | No | Transmural | Yes | |
| 7 | 9 | 8.5 | Collapse, CPR | Ventricle (lateral/apical wall) + IVS | No | Transmural | No | Open chest |
ECMO, Extracorporeal membrane oxygenation; IVS, interventricular septum; CPR, cardiopulmonary resuscitation; LV, left ventricular; VF, ventricular fibrillation.
Detailed transthoracic echocardiography was performed in all patients to identify the location and extent of the tumor, assess the degree of left and right ventricular outflow tract obstruction, and evaluate the involvement of the mitral valve apparatus. One patient had an increased left ventricular outflow tract gradient (2.7 m/s). Additionally, CT scans were performed in all patients to categorize the tumor, identify its location and extent, and assess the involvement of the coronary arteries, and MRI was performed in 4 patients to confirm the diagnosis and evaluate ventricular volume. The left ventricular chamber was involved in all cases, with extension to the interventricular septum (IVS) noted in 3 cases. Extensive involvement of the left ventricle, IVS, and right ventricle was observed in 1 case. Biopsy specimens were obtained and analyzed to confirm fibroma in 2 cases. Preoperatively, 5 patients (71%) were started on antiarrhythmic drugs, and 1 patient had a previous loop recorder implantation.
Median sternotomy with the use of CPB and arresting the heart using cold blood cardioplegia was the preferred surgical approach. One patient was operated on with a beating heart. The median duration of CPB was 167 minutes (IQR, 97-173 minutes), and the median aortic cross-clamp time was 107 minutes (IQR, 72-143 minutes). Transmural resection was performed in 5 cases, with macroscopically complete resection achieved in 3 cases. Entry into the left ventricular cavity during fibroma resection was noted in 1 patient, and the defect was closed directly. No residual ventricular septal defects were observed. The cavity after resection was obliterated by layered closure using polypropylene, and the epicardial margins were approximated using strips of autologous pericardium or Teflon.
One patient experienced recurrent ventricular tachycardia after weaning from CPB owing to stretching of the epicardium overlying the left anterior descending artery. CPB was reinstated, and the stretched epicardium overlying the left anterior descending artery was incised along its entire length. Simultaneous implantation of a loop recorder was performed in 4 cases. The sternum was not closed in 2 cases owing to poor ventricular function postresection. None of the patients required ECMO support postsurgery. The median duration of mechanical ventilation was 18 hours (IQR, 7-61 hours). The median intensive care unit stay was 2 days (IQR, 1-6 days), and the median hospital stay was 10 days (IQR, 7-12 days). All patients had normal sinus rhythm at discharge, with a mean left ventricular ejection fraction of 55 ± 7% (Table 2). The patient with a preoperative left ventricular outflow tract gradient of 2.7 m/s demonstrated a reduced gradient of 1.7 m/s postoperatively.
Table 2.
Postoperative parameters and follow-up data of the 7 study patients
| Patient | ICU stay, d | Hospital stay, d | Rhythm (discharge) | EF at discharge, % | Follow up, mo | Subsequent surgery | Antiarrhythmic drugs | Status |
|---|---|---|---|---|---|---|---|---|
| 1 | 1 | 10 | NSR | 60 | 42 | LR implant (5 mo post- Sx) ICD implant (30 mo post-Sx) |
Amiodarone + nadolol | Alive |
| 2 | 2 | 9 | NSR | 45 | 45 | Redo resection (3 mo post-Sx) | Amiodarone + nadolol + mexiletine | Alive |
| 3 | 3 | 5 | NSR | 60 | 14 | Nil | Nadolol + mexiletine | Alive |
| 4 | 6 | 12 | NSR | 45 | 11 | Nil | Amiodarone + bisoprolol | Alive |
| 5 | 2 | 10 | NSR | 60 | 6 | Nil | Propranolol | Alive |
| 6 | 1 | 7 | NSR | 57 | 12 | Nil | Amiodarone + propranolol | Alive |
| 7 | 8 | 14 | NSR | 60 | 11 | Nil (awaiting ICD implant) | Amiodarone + mexiletine + propranolol | Alive |
ICU, Intensive care unit; EF, ejection fraction; NSR, normal sinus rhythm; LR, loop recorder; Sx, surgery; ICD, implantable cardioverter-defibrillator.
The median duration of follow-up was 12 months (IQR, 11-42 months). There was no mortality following surgical resection of cardiac fibroma. One patient with incomplete resection experienced recurrent arrhythmia and out-of-hospital arrest after 3 months of follow-up; subsequent imaging showed an increase in the size of the residual tumor. The patient underwent reoperation, and the tumor was adequately debulked uneventfully. Another patient with extensive involvement of the IVS after partial resection had recurrent arrhythmia with syncopal episodes and received a subcutaneous implantable cardioverter-defibrillator. At the time of this report, all patients were on antiarrhythmic drugs, with regular ECG and loop recorder monitoring showing good ventricular function.
Discussion
Although cardiac fibromas can arise in the atria or other regions of the heart, all cases evaluated at our institution were confined to the ventricles. The predilection for ventricular involvement—the left ventricle more frequently than the right—might be attributed to the relative distribution and composition of myocardium and fibroblast-rich interstitial tissue. The clinical presentations of our patients varied widely, aligning with findings in prior literature reviews.
Tumor biopsy and histologic evaluation remain the gold standard for confirming a diagnosis. Nevertheless, advancements in imaging techniques have led to widespread reliance on noninvasive modalities, such as echocardiography, CT, and MRI, as primary diagnostic tools, with cardiac catheterization seldom indicated. On MRI, cardiac fibromas typically manifest as well-defined, homogeneous masses that appear isointense to hyperintense relative to the adjacent myocardium on T1-weighted MRI sequences and hyperintense on T2-weighted MRI sequences.8 On CT scans, they often appear as solitary, hypervascular tumors originating in the ventricular wall, displaying homogeneous or heterogeneous enhancement following contrast administration. CT is especially effective at identifying calcifications, which occur in approximately 25% of cases, signifying regions of limited blood supply.9,10 A virtual reality model offers enhanced spatial understanding of the mass and its anatomic context and may surpass traditional imaging methods.
Surgical intervention is generally recommended for symptomatic ventricular fibromas, though there is no consensus regarding surgery for asymptomatic cases. The primary risk even in asymptomatic cases is supported by reports linking ventricular fibromas to sudden cardiac death, often attributed to fatal ventricular tachycardia. In some cases, sudden death occurred without preceding symptoms or documented arrhythmias. Surgical removal is also advised in asymptomatic patients with enlarging tumors to prevent progressive cardiac deformity and atrioventricular valve dysfunction—an approach supported by multiple studies.11, 12, 13
Ventricular fibromas lack a true capsule and cannot be easily excised intact. The clear transition from normal red-colored soft myocardium to white-colored firm tumor allows establishment of a dissection plane between the myocardium and the tumor tissues (Video 1). Because fibromas are benign, their clearly defined boundaries eliminate the need to excise the adjacent myocardium.
To ensure safe and precise tumor removal, optimal conditions include moderate hypothermia, aortic cross-clamping, and intermittent cold-blood cardioplegia. Together, these measures safeguard the myocardium, assist in tumor resection, protect surrounding structures, and enable effective cardiac repair.
After resection of ventricular septal fibromas, reconstruction of the septum can be achieved by closure of the septum in layers using autologous pericardial patches or by placement of a prosthetic patch. Entry into one or both ventricular cavities is common; reconstruction is usually possible by direct closure, although prosthetic patch repair is feasible if necessary.
Once large tumors are removed, the ventricular defects are closed in layers using polypropylene, and the epicardial margins were approximated using strips of autologous pericardium or Teflon patches (Video 2). While some advocate cardiac transplantation for large fibromas,14,15 others may offer an alternative to transplant listing, such as subtotal resection, staged surgical interventions, or palliative shunting.
In our case series, partial resection was done in 3 cases owing to extensive involvement of multiple cardiac chambers and coronary artery. In an infant with a large cardiac tumor arising from the free wall of the left ventricle and IVS and with the left anterior descending artery (LAD) coursing inside the mass, left and right ventriculotomy incisions were made on either sides of the LAD course, and most of the mass was shelled out, leaving only residual fibrotic tissue under the left main coronary artery and along the LAD (Figure 1, Video 3). The interventricular communication was closed directly using autologous pericardial strips, and the ventricular cavity was reconstructed.
Figure 1.
Cardiac fibroma involving the left ventricle and interventricular septum, encasing the mid and distal left anterior descending artery (LAD) in a 9-month-old infant. Incomplete excision of the tumor done leaving only residual fibrotic tissue under the left main coronary artery and along the LAD. ∗Denotes the course of the LAD.
In our view, subtotal resection is a viable option for tumors extending into critical areas such as the cardiac crux or involving major coronary arteries, with cardiac transplantation reserved for rare cases where complete excision would severely compromise essential cardiac structures. In pediatric patients, surgical resection of ventricular fibromas substantially reduces the risk of life-threatening arrhythmias; however, a small subset of patients remains at risk, underscoring the need for long-term rhythm monitoring. For children with unresectable cardiac fibromas, ICDs have been used to mitigate this risk.16
Limitations
We acknowledge that the outcomes presented in this study reflect a median follow-up of 12 months, which offers only an early snapshot of postoperative trajectories. Although our initial results are encouraging, particularly in terms of arrhythmia resolution and ventricular function recovery, this timeframe might not capture delayed complications, recurrence, or the need for future interventions—especially in cases involving subtotal resection.
Conclusions
Cardiac fibroma is a rare benign tumor of children that carries a potential risk of fatal ventricular tachycardia and sudden death. The left ventricle is the most common site involved. Detailed echocardiography and cardiac CT and MRI images are vital in planning surgical excision. Complete resection is preferred to eliminate the risk of recurrence, but partial resection is acceptable if the fibroma involves vital structures or in an effort to preserve ventricular function. Ventricular fibromas have no true capsule, but the borders can be readily recognized; thus, the plane between the firm white tumor and soft red myocardium can be readily developed with sharp dissection or use of cautery. Because fibromas are benign, R0 resection is not mandatory. New onset of arrhythmia or sudden collapse in patients with history of partial resection of fibroma indicates recurrence or increase in size of the tumor. Surgical resection of pediatric cardiac fibroma can be performed with low morbidity and mortality.
Conflict of Interest Statement
The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
Footnotes
This study was deemed exempt from ethical review by the local Institutional Review Board, as it involved retrospective analysis of anonymized clinical data. Informed written consent was not required, as no identifiable patient information was included and the study met institutional criteria for exemption.
Supplementary Data
Surgical video demonstrating the dissection plan involving creation of a dissection plane between the myocardium and cardiac fibroma on the left ventricular lateral wall, followed by complete excision of a cardiac fibroma involving the left ventricular lateral wall in a 1-year-old patient. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.
Surgical video demonstrating complete resection of a large left ventricular fibroma and obliteration of defect in a 15-year-old patient. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.
Surgical video of partial resection of the cardiac fibroma of the left ventricular and interventricular septum encasing the mid and distal left anterior descending artery in a 9-month-old infant. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Surgical video demonstrating the dissection plan involving creation of a dissection plane between the myocardium and cardiac fibroma on the left ventricular lateral wall, followed by complete excision of a cardiac fibroma involving the left ventricular lateral wall in a 1-year-old patient. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.
Surgical video demonstrating complete resection of a large left ventricular fibroma and obliteration of defect in a 15-year-old patient. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.
Surgical video of partial resection of the cardiac fibroma of the left ventricular and interventricular septum encasing the mid and distal left anterior descending artery in a 9-month-old infant. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00358-X/fulltext.