Giant left ventricular fibroma presenting as ventricular tachycardia in a child

Introduction

Primary cardiac tumors are rare in children, with a prevalence of 0.0017% to 0.28% in autopsy series.^1,2 Rhabdomyoma is the most common cardiac tumor in children. Fibromas of the heart are exceedingly rare. ³ Fibroma as a cause of ventricular tachycardia is exceptionally rare.^4,5

Case report

A previously healthy 14-month-old girl was referred from a local hospital to a tertiary center because of chest infection and ventricular tachycardia. She had been unwell for 2 weeks with poor feeding and a fast heart rate noticed by her mother. On presentation to a local hospital, she was febrile, mildly tachypneic, and tachycardiac. She had mild subcostal recessions and crepitations on auscultation. She was diagnosed as having a chest infection and ventricular tachycardia of unknown origin. She was managed medically and referred to tertiary center for further management. She was clinically stable when seen initially in our tertiary center. Hematological and biochemical investigations were normal. An electrocardiogram showed ventricular tachycardia (Figure 1(a)). Chest radiography indicted cardiomegaly (Figure 1(b)). An echocardiogram demonstrated a large mass located at the apex of the left ventricle, pushing the apex upwards (Figure 1(c)). Chest computed tomography with and without contrast revealed a large well-defined pericardial mass lesion of regular contour, infracardiac in location, producing upward elevation of the cardiac apex (Figure 2). The mass measured 4 × 3 cm. In addition, there was central calcification. Cardiac catheterization showed a huge mass in the left ventricular apex without coronary artery involvement. The patient underwent a median sternotomy with cardiopulmonary bypass and cold cardioplegic arrest. The pericardium was opened, and a huge greyish mass was seen bulging through it. The mass extended from the apex of heart to the base of the left ventricle and from the anterior descending artery to the obtuse margin of the heart. The epicardium was incised 3 cm lateral to the anterior descending artery; a cleavage plane could be developed between the tumor and normal muscle. The mass was enucleated with careful dissection. The tumor measured 8 × 7 × 5 cm and weighed 300 g (Figure 3). There was no intra- or postoperative complication. Postoperative electrocardiography (Figure 4(a)) showed normal sinus rhythm with no evidence of chamber hypertrophy and no signs of ischemia; a 72-h Holter recording was normal. Echocardiography showed normal ventricular size and function with no residual mass. The patient was discharged home after 6 days in a good condition on no medication. Histopathological examination confirmed the diagnosis of left ventricular fibroma (Figure 4(b), 4(c)). The patient has been followed up for 4 years and is doing fine. The last echocardiogram showed normal left ventricular size and function and no evidence of tumor recurrence.

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Figure 1.

(a) Electrocardiogram showing ventricular tachycardia. (b) Chest radiograph in anteroposterior view and (c) Two-dimensional echocardiogram in apical 4-chamber view showing a huge mass in the apical area.

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Figure 2.

Chest computed tomography in coronal view showing a mass in the apical area.

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Figure 3.

The huge mass after excision.

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Figure 4.

(a) Postoperative electrocardiogram showing sinus arrhythmia and no left ventricular hypertrophy. (b) Photomicrograph of tissue from mass showing features of fibroma. Green Masson trichrome stain and (c) Photomicrograph of tissue from mass showing typical features of fibroma. Hematoxylin and eosin stain.

Discussion

Rhabdomyoma is the most common primary cardiac tumor in children.^1,2 Rhabdomyomas are usually multiple and involve the ventricular myocardium. ⁶ Cardiac fibroma is the second most common cardiac tumor in children. ⁵ It usually involves the left ventricular free wall. ⁷ These tumors are usually solitary masses and are generally completely resectable. ⁸ Intrapericardial teratoma arises from within the pericardium from the pedicle at the base of great vessels, and is mostly associated with pericardial effusion.^7,8 Surgical resection of this tumor is usually curative.^7,8 Myxoma is more common in adults than children. The majority of myxomas arise from the left atrium.^1,8 Surgical removal is usually effective.^1,8

The clinical presentation of cardiac fibroma depends on the size and location of the tumor. ⁸ Children usually present with palpitation, chest pain, arrhythmia, and heart failure. ⁸ Diagnosis is usually established by echocardiography, computed tomography, and magnetic resonance imaging. ⁸ In our case, echocardiography and computed tomography could delineate the extent of the mass in the left ventricular wall, and cardiac catheterization was performed to assess the mass and its relation to the coronaries. The management strategy for cardiac fibroma is complete resection because it does not recur.^3,8 Any child with arrhythmia, especially ventricular tachycardia, should undergo thorough investigation including echocardiography and chest computed tomography to exclude a cardiac tumor as a treatable cause of ventricular tachycardia.