Ann Thorac Surg 2004;78:328-330
© 2004 The Society of Thoracic Surgeons
Case report
Surgical issues in giant right ventricular fibroma
Brojendra N. Agarwala, MDa,
Joanne P. Starr, MDb,
Eloise Walker, MDa,
Emile A. Bacha, MDa*
a Section of Pediatric Cardiology, Chicago, Illinois, USA
b Section of Cardiothoracic Surgery, Pediatric and Congenital Cardiac Surgery, University of Chicago Children's Hospital, Chicago, Illinois, USA
Accepted for publication June 13, 2003.
* Address reprint requests to Dr Bacha, Pediatric and Congential Cardiac Surgery, The University of Chicago Children's Hospital, 5841 S Maryland Ave, MC 5040, Chicago, IL, USA 60637
e-mail: ebacha{at}surgery.bsd.uchicago.edu
 |
Abstract
|
|---|
Cardiac fibroma is a rare and benign tumor usually seen in infants and children. We report a 3-year-old asymptomatic child who presented with a giant right ventricular (RV) fibroma. He underwent complete surgical resection and is doing well at follow-up. Because of the size of the tumor and the potential need for resection of RV free wall, it is essential to have contingency plans to deal with postoperative RV failure.
|
Introduction
|
|---|
Cardiac fibroma, while overall an exceedingly rare lesion, is the second most common tumor in the pediatric population after rhabdomyomas [1, 2]. The tumor consists of benign growth of myocardial connective tissue and more commonly originates from the left ventricular free wall. It is predominantly a solitary lesion. Patients can be asymptomatic, or present with congestive heart failure, sudden death, or arrhythmias [1, 2]. When feasible, complete resection is usually recommended [1, 2]. Debulking (partial resection), heart transplantation, or conservative management have also been advocated [2, 3]. This report describes a patient with a giant right ventricular (RV) free wall fibroma. Pertinent surgical issues addressed in this report are whether surgery is indicated, the need (and risk) of partial versus complete resection, and the surgical management of RV failure resulting from an extensive RV resection.
A 3-year-old previously healthy child presented with a heart murmur. Physical examination revealed a healthy appearing child with normal vital signs, and a prominent left precordium. A grade 1–2/6 atypical ejection systolic murmur was heard along the left upper sternal border. Electrocardiogram revealed normal sinus rhythm, left axis deviation, and right bundle branch block. Twenty-four hour Holter monitor showed no evidence of arrhythmia. Crosssectional echocardiogram (Fig 1)
and magnetic resonance imaging (MRI) of the heart (Fig 2)
demonstrated a 7 x 5 cm cardiac tumor located in the inlet portion of the RV free wall, severely compressing the RV cavity. The extent of the tumor in-growth into the endocardium or involvement of the anterior papillary muscle of the tricuspid valve could not be determined. Color-flow Doppler at the RV inflow indicated mild obstruction. At surgery a 7 x 4.5 cm sessile white appearing poorly vascularized intramural tumor mass was noted in the inlet portion of the anterior wall of the RV, severely compressing the RV cavity. After cardioplegic arrest and right atriotomy, the RV endocardium was noted to be completely intact. Frozen section biopsy of the tumor was consistent with benign fibroma. Starting at the margin between tumor and normal myocardium, the tumor was completely excised using sharp dissection, staying within 1 to 2 mm of healthy myocardium. The endocardium was breached at the lowest point of the tumor creating a 5-mm hole. The right coronary artery and atrioventricular groove were preserved. Once the resection was completed, the remaining cavity was collapsed onto itself and the epicardium closed with a double layer of 5-0 polypropelene. Cross clamp time was 57 minutes. His postoperative course was uneventful. Postoperative echocardiogram did not reveal residual tumor and the RV cavity was normal in size with normal tricuspid Doppler color-flow pattern (Fig 3).
At 18-months follow-up, he is doing well.
View larger version (96K):
[in this window]
[in a new window]
|
Fig 1. Two-dimensional echocardiogram in 4-chamber view revealing a giant (4.71 x 3.80 cm) extracavitary tumor compressing the right ventricular (RV) cavity with narrow RV inflow area.
|
|
View larger version (70K):
[in this window]
[in a new window]
|
Fig 2. Magnetic resonance imaging scans of the heart revealing a giant capsulated tumor just behind the sternum arising from the anterior wall of the right ventricle (RV) causing severe RV cavity compression. (A) Transverse view. (B) Longitudinal view.
|
|
View larger version (125K):
[in this window]
[in a new window]
|
Fig 3. Postoperative two-dimensional echocardiogram in 4-chamber view demonstrating normal ventricles.
|
|
|
Comment
|
|---|
Although surgical resection of symptomatic cardiac fibromas is universally accepted, the role of surgery in asymptomatic fibromas is less clear [2, 4]. They can remain dormant for many years and even regress [5]. The present patient did have a solitary tumor, which differentiates it from hamartomas, which are usually multiple and should be observed. There was mild RV inflow obstruction from this giant tumor. In addition to the above, the increasingly well-documented risk of significant arrhythmias and sudden death convinced us that an operation was indicated.
Indeed, the largest series of cardiac fibromas reports 9 of 23 patients (39%) with either documented arrhythmias or sudden death [2], and thus we felt that the remaining RV scar would be less arrhythmogenic than this huge mass.
Preoperatively, we were concerned with creating RV failure from a massive RV free wall resection. The MRI could not demonstrate whether the tumor was involving the endocardial surface, thus necessitating transmural RV free wall resection. In case of postoperative RV failure, we were prepared to perform a bidirectional Glenn shunt [1] as a first step, or, in case a massive RV resection was needed, a RV exclusion procedure [6]. However, by respecting the tumor margins, resection of only small amounts of RV muscle per se and closure of the defect over itself, the RV function was maintained. Partial resection would have been another alternative. This has been done before with long-term success, but is not recommended if complete resection is at all possible safely [1, 2]. In conclusion, this case demonstrates that even very large RV fibromas can be resected completely and safely. However, it is essential to have contingency plans for postoperative RV failure.
|
References
|
|---|
- Beghetti M., Gow R.M., Haney I., Mawson J., Williams W.G., Freedom R.M. Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J 1997;134:1107-1114.[Medline]
- Burke A.P., Rosado-de-Christenson M., Templeton P.A., Virmani R. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994;108:862-870.[Abstract/Free Full Text]
- Valente M., Cocco P., Thiene G., et al. Cardiac fibroma and heart transplantation. J Thorac Cardiovasc Surg 1993;106:1208-1212.[Abstract]
- Tahernia A.X., Bricker J.T., Ott D.A. Intracardiac fibroma in an asymptomatic infant. Clin Cardiol 1990;13:506-512.[Medline]
- Lee Y.C., Singleton R.T., Tang C.K. Benign mesenchymal tumor of the heart: spontaneous regression and disappearance of pulmonary artery stenosis. Chest 1982;82:503-505.[Free Full Text]
- Sano S., Ishino K., Kawada M., et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002;123:640-647.[Abstract/Free Full Text]
Top
Abstract
Introduction
