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Ann Thorac Surg 1996;62:882-884
© 1996 The Society of Thoracic Surgeons

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Case Report

Massive Right Ventricular Fibroma Treated With Partial Resection and a Cavopulmonary Shunt

Divisions of Cardiology , Radiology and Cardiovascular Surgery, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada

Accepted for publication March 25, 1996.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
Cardiac fibromas in infants and children may present as intramural or intracavitary masses. Total or partial resection as well as transplantation have been reported as treatment for symptomatic patients. We report an infant, diagnosed prenatally, who underwent partial excision of a massive obstructive right ventricular fibroma and creation of a bidirectional cavopulmonary shunt. The palliative approach has allowed the child to be followed up with the option of transplantation should the tumor continue to increase in size.

	Introduction

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Primary cardiac tumors are uncommon in infants and children [1, 2]. Cardiac fibromas are the second most frequent tumors following rhabdomyomas, and occur almost exclusively in pediatric patients. More than 75% are diagnosed by 2 years of age, which suggests that the tumor may be congenital [3, 4]. The left ventricular free wall and the septum are most often involved. Right ventricular fibromas are rare [1–3]. Congestive heart failure, heart murmurs, and arrhythmias may be seen as presenting symptoms [3, 5]. Fibromas are benign tumors but can be clinically important when they cause inflow or outflow obstruction or arrhythmias. Total or partial resection allows relief of hemodynamic impairment [6–8]. Recently cardiac transplantation has been used for management of large nonresectable cardiac fibromas [4].

A 36-week-old baby girl with a prenatal diagnosis of pericardial effusion and thickened myocardium was referred to our center. A two-dimensional echocardiogram repeated shortly after birth showed a large right ventricular mass attached to the free wall and measuring 4.5 x 2.2 cm. The mass did not extend into the right atrium or cause right ventricular outflow tract obstruction. Magnetic resonance imaging confirmed the two-dimensional echocardiographic findings. The electrocardiogram showed sinus tachycardia at a rate of 190 beats/min, right-axis deviation, and right bundle-branch block. The chest roentgenogram showed mild cardiomegaly. The family history was negative, fundoscopy and Wood's lamp examination were normal, and magnetic resonance imaging of the head was also normal, excluding tuberous sclerosis. At hospital discharge, she had no sign of heart failure, and the 24-hour Holter monitoring showed occasional monomorphic premature ventricular contractions.

At age 3 months, repeat two-dimensional echocardiogram and magnetic resonance imaging showed that the tumor had increased in size to 5.6 x 3.1 cm (Fig 1). The right ventricular inflow was mildly obstructed and the right atrium was mildly enlarged. An echocardiogram showed that the mitral valve inflow was inverted with filling abnormalities of the left ventricle secondary to compression. She was asymptomatic but because of the rapid growth of the tumor and the presence of inflow obstruction, resection of the mass with creation of a right bidirectional cavopulmonary anastomosis, to ensure pulmonary blood flow, was recommended. The heart was arrested for 60 minutes and protected with cold blood cardioplegia and reinfusion of warm cardioplegia. The endocardial appearance of the right ventricle was normal but the bulk of the tumor was easily palpable in the wall and was found to occupy the entire anterior and diaphragmatic free wall of the right ventricle. The anatomically normal tricuspid valve and the left ventricle were compressed by the tumor. A 5 x 4 cm portion of the mass was resected from the anterior wall through a right atrial approach and a right ventricular infundibulum incision of about 5 cm. Complete resection was precluded by the size and location of the mass, as well as the long bypass and cross-clamp time used to perform the partial resection. Histology was consistent with the diagnosis of a fibroma. The child's hospital course was uneventful and she was discharged 8 days later.

View larger version (46K): [in this window] [in a new window]   Fig 1. . T1-weighted spin-echo magnetic resonance images of the chest. Axial image at age 3 months showing the right ventricular free wall tumor (*) compressing the right ventricle (arrows) and the right atrioventricular valve. Note the transverse lie of the heart within the chest due to the tumor bulk. (LA = left atrium; LV = left ventricle; RA = right atrium.)

View larger version (52K): [in this window] [in a new window]   Fig 2. . Sagittal image 6 months after the first partial resection showing the tumor mass (*) and the patent bidirectional cavopulmonary anastomosis (**). (RPA = right pulmonary artery; RV = right ventricle.)

At age 2 years, she is a very active child with normal development. Physical examination reveals only a nonspecific 1/6 systolic ejection murmur at the left sternal border. The electrocardiogram shows right bundle-branch block. The 24-hour Holter monitoring is normal. The two-dimensional echocardiogram shows no inflow or outflow obstruction and a tumor involving the free wall of the right ventricle. The magnetic resonance imaging shows that the tumor is slightly smaller compared with the previous examination.

	Comment

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Complete surgical resection of cardiac fibromas may be precluded by their size and location [2, 4]. Partial excision may relieve obstruction or compression and long-term survival has been reported [5, 8]. Operation for asymptomatic tumors is controversial but in a large review, cardiac fibroma was found incidentally at autopsy (9%) or sudden death was the initial manifestation (14%) in 144 patients reviewed, indicating its lethal potential [2, 3]. Inflow or outflow tract obstruction as well as arrhythmias are the main indications for operation [4]. Cardiac fibromas may remain stable for many years, but some have been found to show aggressive growth [3].

Operation in this child was recommended on the basis of rapid growth and mild inflow obstruction of the right ventricle. A bidirectional cavopulmonary anastomosis was performed to ensure pulmonary blood flow if the mass continued to grow and become more obstructive, as well as to guard against right ventricular failure due to the incomplete resection. Further resection or transplantation remain options if the tumor progresses.

Close noninvasive follow-up with repeated two-dimensional echocardiogram or magnetic resonance imaging and 24-hour Holter monitoring is mandatory to detect changes in tumor size or rhythm disturbances.

Whenever possible, cardiac fibromas causing symptoms should be totally excised. If total excision is not possible, partial resection may be beneficial. The addition of a cavopulmonary shunt may provide extra assurance and safety in ensuring long-term pulmonary blood flow in massive right ventricular fibromas.

	Footnotes

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Address reprint request to Dr Gow, Division of Cardiology, The Hospital for Sick Children, 555 University Ave, Toronto, Ont, Canada M5G 1X8.

	References

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1. Abushaban L, Denham B, Duff D. 10 year review of cardiac tumours in childhood. Br Heart J 1993;70:166–9.[Abstract/Free Full Text]
2. Bertolini P, Meisner H, Paek SU, Sebening F. Special considerations on primary cardiac tumors in infancy and childhood. Thorac Cardiovasc Surg 1990;38:164–7.
3. Parmley LF, Salley RK, Williams JP, Head GB III. The clinical spectrum of cardiac fibroma with diagnostic and surgical considerations: noninvasive imaging enhances management. Ann Thorac Surg 1988;45:455–65.[Abstract/Free Full Text]
4. Valente M, Cocco P, Thiene G, et al. Cardiac fibroma and heart transplantation. J Thorac Cardiovasc Surg 1993;106:1208–12.[Abstract]
5. Burke AP, Rosado-de-Christensen M, Templeton PA, Virmani R. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994;108:862–70.[Abstract/Free Full Text]
6. Ceithaml EL, Midgley FM, Perry LW, Dullum MK. Intramural ventricular fibroma in infancy: survival after partial excision in 2 patients. Ann Thorac Surg 1990;50:471–2.[Abstract/Free Full Text]
7. Murphy MC, Sweeney MS, Putnam JB, et al. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac Surg 1990;49:612–8.[Abstract/Free Full Text]
8. Yamaguchi M, Hosokawa Y, Ohashi H, Imai M, Oshima Y, Minamiji K. Cardiac fibroma. Long-term fate after excision. J Thorac Cardiovasc Surg 1992;103:140–5.[Abstract]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Beghetti, M. Articles by Gow, R. M. Search for Related Content PubMed PubMed Citation Articles by Beghetti, M. Articles by Gow, R. M.