Abstract

Cardiac fibromas are rare primary cardiac tumors. Although they constitute a small portion of cardiac neoplasms, they are the most common primary cardiac neoplasm group in childhood after rhabdomyomas. Cardiac fibromas, composed of connective tissue and fibroblasts, are benign. They may be asymptomatic, cause symptoms such as intracavitary obstruction, coronary artery compression, thromboembolic events, and conduction defects, or result in sudden death. Approximately 180 to 200 cases of cardiac fibromas have been reported in the literature, and most are located in the ventricles. Since fibromas are rare, optimal treatment strategies are unclear. Surgical excision is recommended for tumors that cause arrhythmias, heart failure, or intracavitary obstruction. This video article presented the surgical excision of a 6.2×5×4.3 cm cardiac fibroma originating from the anterior wall of the right ventricle in an 11-month-old infant. Postoperative transesophageal echocardiography showed that the tumor was completely removed and that the right ventricular and tricuspid valve functions were good.

Cardiac fibromas are rare primary cardiac tumors. Although they constitute a small portion of cardiac neoplasms, they are the most common primary cardiac neoplasm group in childhood after rhabdomyomas. Cardiac fibromas, composed of connective tissue and fibroblasts, are benign.[1] They may be asymptomatic or may cause symptoms such as intracavitary obstruction, coronary artery compression, thromboembolic events, conduction defects, and may cause sudden death. They are typically intramural and are often found in the ventricles. Atrial fibromas are quite rare. In addition to echocardiography, computed tomography angiography and magnetic resonance imaging are valuable diagnostic tools.[2,3] These diagnostic methods may help in deciding on surgical excision.

Video 1. Removal of huge cardiac fibroma from right ventricle video presentation.

Given the rarity of cardiac fibromas, the optimal treatment strategy remains uncertain. In cases where surgical excision is not possible, single ventricle palliation and even heart transplantation are among the treatment options.[3,4] In the long-term follow-up study conducted by Cho et al.[3] after cardiac fibroma resection, the most common reason for reoperation was mitral valve insufficiency, while no recurrence was observed in patients who underwent complete resection. In this video article, we reported successful surgical excision of a huge cardiac fibroma originating from the anterior wall of the right ventricle in a small infant.

TECHNIQUE
The 11-month-old patient underwent a standard median sternotomy in the supine position. The thymus was removed. A large piece of pericardium was removed from the anterior surface and prepared with 0.6% glutaraldehyde solution. Cardiopulmonary bypass was initiated with aortic and bicaval cannulation. Cardioplegic arrest was achieved under moderate hypothermia. The right atrium was opened parallel to the atrioventricular groove. The location of the tumor and its relationship with the cardiac structures were examined from within the tricuspid valve. The tumor was located in the anterior wall of the right ventricle, with no involvement of the ventricular septum or tricuspid valve. Since exposure was insufficient, intervention could not be performed from within the tricuspid valve. The right ventricle infundibulum was opened from a position away from the mass. The tumor was reached. The mass, which was observed to be unencapsulated, gray in color, and elastic in consistency, was dissected and stripped from the myocardial tissue. A second incision was made in the right ventricular free wall for the mass that was highly adherent to the anterior wall of the right ventricle and was observed to have thinned the ventricular wall to a great extent. The tumor was completely excised together with the thinned ventricular tissue in the anterior wall. The removed tumor was 6.2×5×4.3 cm in size. During the dissections, damage to the coronary arteries, tricuspid valve, and conduction system was avoided. The defect formed by the excision of the highly thinned right ventricular myocardium was closed with 5-0 Prolene sutures and supported by an autogenous pericardial patch to prevent the reduction of the right ventricular cavity and aneurysmal dilatation. Cardiopulmonary bypass was terminated without any issues. Cross-clamp and cardiopulmonary bypass times were 78 and 126 min, respectively. Transesophageal echocardiography revealed that the tumor was completely removed, and the right ventricular and tricuspid valve functions were good. A written informed consent was obtained from the parent of the patient.

The patient was discharged on the eighth postoperative day without any issues. The patients remained under follow-up by a pediatric cardiologist for approximately one year, during which no tricuspid valve insufficiency, ventricular dysfunction, arrhythmia, ventricular aneurysm formation, or recurrence was observed.

Discussion

Primary cardiac neoplasms are rare in pediatric cardiac surgery practice. The prevalence reported in autopsy series is between 0.0017% and 0.28%.[5] Cardiac fibromas are usually 3 to 8 cm in size, single, multilobulated, gray-white in color, and hard in consistency. Approximately 180 to 200 cases of cardiac fibroma have been reported in the literature, and the majority of these fibromas are located in the ventricles. Fibromas originating from the atrium, septum, and other cardiac tissues are rare.[6] Even if they do not invade, they can strongly adhere to the myocardial tissue due to their lack of encapsulation, irregular growth, and intramural nature.[3]

Cardiac fibromas are usually asymptomatic and can be detected incidentally. Since fibromas are rare, optimal treatment strategies are unclear. Benign cardiac tumors such as fibromas are often diagnosed when they reach large sizes and become symptomatic. Surgical excision is recommended for tumors that cause arrhythmias, heart failure, or intracavitary obstruction. Surgical treatment is curative, but sometimes total excision may be very difficult or unfeasible. Since procedures such as valve replacement and coronary artery bypass grafting may be impossible, particularly in infants, procedures that may be alternatives to complete resection can be performed. In such cases, even if the mass cannot be completely removed, partial resections also have a favorable prognosis. Waller et al.[4] applied atrial septostomy and systemic pulmonary artery shunt in a newborn with cardiac fibroma in the right ventricle that was deemed unresectable. Thus, they provided a bridge to heart transplantation by providing single ventricle palliation. Since involvement of the ventricular septum causes conduction defects and arrhythmias, it is associated with a poor prognosis. In our case, the fibroma was in the right ventricle anterior and lateral walls. There was no ventricular septum involvement and coronary artery occlusion. It caused stenosis in right ventricular inflow and outflow tracts. The fact that the tumor size was very large and that it was seen in a baby were also factors that made the case difficult. On magnetic resonance imaging, no surgical plane could be observed between the mass, which had low vascularity, and the myocardium. Despite this, a decision was made for surgical resection due to the intracavitary stenosis and its large size. It was possible to completely separate the tumor from the myocardial tissue with sharp dissection. It was very large in size compared to the infant's heart. During surgery, it was decided to remove the entire tumor tissue together with extremely thinned a portion of the right ventricular anterior wall instead of partial resection to prevent recurrence and preserve right ventricular functions. The resected right ventricular anterior wall tissue was reconstructed with a glutaraldehyde pericardial patch, and ventricular functions were preserved.

In conclusion, for surgical planning, the proximity to critical structures such as the septum, valves, conduction system, and coronary arteries should be well evaluated. For good exposure, the incision sites should be well planned, and the resection should be performed with sharp dissection. In this way, surgical excision of large and symptomatic fibromas can be successfully performed. Surgical resection is at the forefront for successful results, particularly in infants, and if achieved, total excision can be curative.

Data Sharing Statement: The data that support the findings of this study are available from the corresponding author upon reasonable request.

Author Contributions: Idea, literatüre review, writing article: Y.T.; Design, critical review: B.T.; Analysis, references and fundings: S.A.; Control, supervision, writing article: E.E.

Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Funding: The authors received no financial support for the research and/or authorship of this article.

References

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2) Rajput FA, Bishop MA, Alahmadi MH, Limaiem F. Cardiac fibroma. In: StatPearls [Internet]. StatPearls Publishing; 2024. [Accessed: 25.08.2024].

3) Cho JM, Danielson GK, Puga FJ, Dearani JA, McGregor CG, Tazelaar HD, et al. Surgical resection of ventricular cardiac fibromas: Early and late results. Ann Thorac Surg 2003;76:1929-34. doi: 10.1016/s0003-4975(03)01196-2.

4) Waller BR, Bradley SM, Crumbley AJ, Wiles HB, McQuinn TC, Bennett AT. Cardiac fibroma in an infant: Single ventricle palliation as a bridge to heart transplantation. The Annals of Thoracic Surgery 2003;75:1306-8. doi:10.1016/ S0003-4975(02)04655-6

5) Xu ZH, Wu QY, Li HY, Xue H, Zhang MK, Jin YQ. Surgical treatment of large left ventricular fibroma in children. Chin Med J (Engl) 2017;130:1737-8. doi: 10.4103/0366- 6999.209901.

6) Teng F, Yang S, Chen D, Fang W, Shang J, Dong S, et al. Cardiac fibroma: A clinicopathologic study of a series of 12 cases. Cardiovasc Pathol 2022;56:107381. doi: 10.1016/j. carpath.2021.107381.