|Year : 2017 | Volume
| Issue : 2 | Page : 154-156
Large right ventricular fibroma
Sachin Kuthe1, Manish Sonkusale2, Anil Wanjari3, Prasad Panbude2
1 Department of Cardiovascular and Thoracic Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Cardiac Anesthesia, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India
3 Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India
|Date of Web Publication||8-Sep-2017|
Department of Cardiac Anesthesia, Jawaharlal Nehru Medical College and Acharya Vinoba Bhave Rural Hospital, DMIMS, Sawangi (M), Wardha - 442 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Primary cardiac tumors are extremely rare in infants and children. They are generally benign histologically, but clinical spectrum varies from asymptomatic patient to ventricular arrhythmia, sudden cardiac death. This report describes the case of a 6-year-old girl with a large cardiac fibroma in the right ventricle. Surgical resection was indicated because of the right ventricular outflow tract obstruction caused by progression of the tumor and symptoms to patient. The fibroma was successfully completely resected and further follow-up evaluation was uneventful.
Keywords: Asymptomatic patient, primary cardiac tumors, ventricular fibroma
|How to cite this article:|
Kuthe S, Sonkusale M, Wanjari A, Panbude P. Large right ventricular fibroma. J Datta Meghe Inst Med Sci Univ 2017;12:154-6
| Introduction|| |
Tumors of the heart are rare across all age group of patients. Incidence of primary tumor of the heart is 0.001–0.03. Secondary involvement of the heart by extra cardiac tumors is 20–40 times more common than by primary cardiac tumors. Cardiac myxoma is the most common of these primary cardiac tumors. Patient's symptom varies according to size and site of tumor.,,
Cardiac fibroma is a rare and benign tumor usually seen in infants and children. These are tumors of connective tissue, mostly solitary. Cardiac fibroma treatment varies depending on tumor size and location, influence on cardiac function, and symptom. We are reporting here a case of the right ventricular tumor occupying whole of the right ventricular cavity.
| Case Report|| |
A 6-year-old girl presented with breathlessness on exertion for 2 years and syncope for 6 months. Physical examination reveals a healthy appearing child with normal vital signs and prominent left precordium. A soft systolic murmur was heard along the left upper parasternal border. Electrocardiogram showed normal sinus rhythm, right axis deviation, incomplete right bundle branch block, and no ectopic complexes. Transthoracic echocardiography revealed a large echogenic mass (5 cm × 4 cm) in the right ventricle (RV). The mass was attached to the RV-free wall with broad base and occupying almost entire cavity of RV. There was a small gap between mass margins and interventricular septum 2–3 mm allowing turbulent blood flow [Figure 1]. The pulmonic and tricuspid leaflets were free from the mass. Cardiac magnetic resonance imaging (MRI) was done for further evaluation, which confirmed echocardiography finding [Figure 2].
|Figure 1: Transthoracic echocardiography images (a) large tumor occupying most of the right ventricle cavity and attached to free wall and (b) turbulent blood flow between tumor and septum|
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|Figure 2: Magnetic resonance imaging images (a) cross-section of tumor and (b) sagittal section of tumor|
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Intraoperative after cardioplegic arrest, right atriotomy was done and tumor removal was attempted through tricuspid valve but was abandoned because of the size of mass and extensive attachment to the right ventricular wall and possible damage to tricuspid valve. Tumor was then approached through the right ventricular incision, and large 5 cm × 7 cm, sessile, firm, whitish, egg-shaped tumor was then excised. Tumor was found to be arising from the intramural part of RV-free wall, poorly vascularized, severely compromising RV cavity [Figure 3]. The overlying eight ventricular wall was thinned out, which was also excised with tumor. The right ventricular incision was closed primarily using soft PTFE felt. The patient came off bypass in sinus rhythm with minimal inotropic support, levosimendan 0.05 mcg/kg/min. Histological findings were consistent with cardiac fibroma.
|Figure 3: Intraoperative images of tumor excision (a and b) and tumor size (c and d)|
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Postoperative course in the intensive care unit (ICU) was uneventful. The patient extubated in 4 h after shifting to ICU. She did not show any sign of low cardiac output syndrome. Inotropes were tapered off on the 2nd postoperative day. She remained in normal sinus rhythm throughout ICU stay. Postoperative echocardiography showed normal RV cavity and function and no remnant of tumor mass.
| Discussion|| |
Primary cardiac tumors in infants and children are extremely rare and reflect a benign process in more than 90% of the cases. The incidence is reported to be 0.03%–0.32%. Atrial myxoma is by far the most common primary cardiac tumor in adults, and rhabdomyosarcoma is most common in children. Fibroma is second only to rhabdomyosarcoma accounting for approximately 25% benign cardiac tumors in infants and children. There is no sex predominance, and the frequency of such tumors tends to decline with age.
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., These are firm gray-white masses, range from 1 to 10 cm in diameter. The echocardiographic appearance is of discrete, often obstructive masses. Grossly, they appear sharply demarcated, with multiple foci of calcification.
Because of their rare prenatal diagnosis, rapid growth, and crucial localization, large cardiac fibromas often present with cardiac arrhythmias and heart failure, and they can lead to sudden death., The evolution and refinement of noninvasive imaging techniques, such as ultrasonography, echocardiography, MRI, and multidimensional echocardiography, have allowed optimal visualization and decision-making at earlier stages of tumor development.
Parks et al. reported the first successful excision of a ventricular fibroma (then called fibrous hamartoma) in 1962. Although the treatment of symptomatic fibromas consists of complete excision, the management of asymptomatic fibromas remains controversial. Surgical practices vary from partial resection to complete resection and reconstruction. Valente et al. reported heart transplantation in some cases due to giant tumor occupying the heart. However, other shows partial excision resulted in good morbidity and long-term survival.,,
Our patient was a 6-year-old girl, symptomatic due to intracardiac tumor, and echocardiography shows solitary mass severely compromising RV cavity and causing outflow obstruction. Considering risk of congestive heart failure, sudden death, or arrhythmias, complete surgical resection was considered. Intraoperative, complete excision of tumor along with thinned out RV wall was performed. Meticulous dissection was done to preserve normal myocardium around tumor attachment without compromising RV cavity.
Our two major concerns preoperatively were postoperative RV failure and arrhythmias, attributed to right ventricular incision and resection of free wall. Previously, bidirectional Glenn shunt was described in RV failure following tumor resection.
| Conclusion|| |
Although fibromas are solitary, benign, slowly growing tumors with tendency to grow huge and having no malignant potential. Considering untoward complications such as cardiac failure, arrhythmias, and sudden cardiac death, surgical resection of fibroma should be attempted in symptomatic patients. Complete surgical excision of tumor with minimal RV resection is a key to successful surgical outcome and minimal postoperative morbidity.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]