(Tex Heart Inst J 1995;22:261-4). Pirimary tumors of the heart are very .... Parmley LF, Salley RK, Williams JP, Head GB III. The clinical spectrum of cardiac ...
Case Reports
Removal of a Giant Cardiac Fibroma from a 4-Year-Old Child
Ulrich Busch, MD Christoph Kampmann, MD Rudolf Meyer, PhD Karl H. Sandring, MD Gerd Hausdorf, PhD Wolfgang Konertz, PhD
In an 8-month-old infant presenting with a systolic heart murmur, a cardiac tumor was diagnosed by means of 2-dimensional echocardiography, cardiac catheterization, and magnetic resonance imaging. In the following years, the child developed ventricular arrhythmias, which were controlled with antiarrhythmic drugs. The operation was undertaken when the boy was 4 years and 3 months of age. A tumor of the interventricular septum measuring 45 x 13 x 25 mm was resected in toto. The defect in the ventricular septum was closed with a Dacron patch. The postoperative course was uneventful. Histologic examination classified the tumor as a fibroma. (Tex Heart Inst J 1995;22:261-4)
Pirimary tumors of the heart are very uncommon lesions. The 1st case of a cardiac fibroma was documented by Luschka in 1855, and about 95 cases of cardiac fibroma had been reported in the English literature by 1992.' To date, over 100 cases have been published worldwide. The 1st successful excision of a cardiac fibroma was performed by Parks and colleagues2 in 1962. As recently as 1983, only 22 cases of successful removal of the tumor had been reported.3 However, the improved diagnostic techniques of angiocardiography and echocardiography have increased the frequency of identification of these tumors.4 We report our recent experience in treating a 4-yearold boy with a fibroma arising from the interventricular septum.
Case Report The boy was born in October of 1989. He exhibited normal growth and development. During a routine physical examination at the age of 8 months, an uncharacteristic systolic heart murmur was heard at the left sternal border. Cardiac ultrasound examination disclosed a solid mass originating from the interventricular septum and protruding into the left ventricular cavity. It measured 3.4 by 3.7 cm.
Key words: Fibroma/diagnosis; fibroma/surgery; heart neoplasms/diagnosis; heart neoplasms/surgery From: The Departments of Cardiac Surgery (Drs. Busch and Konertz), Pediatric Cardiology (Drs. Hausdorf and Kampmann), Radiology (Dr. Sandring), and Institute of Pathology (Dr. Meyer),
Humboldt-University, Charite, D-1 0098 Berlin, Germany Address for reprints: Dr.med. U. Busch, Universitatsklinik und Poliklinik fur Herzchirurgie, Charit6, Schumannstr. 20/21, D-10098 Berlin, Germany Texas Heail Institutejournal
This finding was subsequently confirmed by magnetic resonance imaging (MRI), which showed a well-defined, oval tumor occupying both right and left ventricular chambers (Fig. 1). The tumor and the myocardial signal were similar; therefore, rhabdomyoma was suspected. An attempt to perform an endomyocardial biopsy failed to obtain tumor cells, despite computed tomographic guidance. Thereafter, heart catheterization revealed dilatation of the left ventricle, but hemodynamic data did not show significant obstruction of either the inflow or the outflow tract. A 2nd attempt to retrieve tumor tissue by means of endomyocardial biopsy failed also. In January of 1991, ventricular tachycardias were recorded during electrocardiographic monitoring. The infant was treated with propafenon, and no ventricular tachycardias occurred during subsequent examinations. During the following years, the child grew normally. He underwent cardiologic examinations at 6-month intervals. The mass did not appear to increase in diameter after the 1st echocardiographic exam. When the boy was referred to surgery, he was 4 years and 3 months of age. At surgery, the heart was found to be moderately enlarged. After institution of cardiopulmonary bypass and cardioplegic arrest, a central tumor as large as a hen's egg could be palpated. Removal of Giant Cardiac Fibroma from a 4-Year-Old
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Fig. 1 Magnetic resonance image of the chest before surgery. A tumor of the interventricular septum is shown occupying both ventricular chambers.
A small apical left ventriculotomy was performed for the purpose of inspection. A large protrusion into the chamber was seen, but the tumor was covered with myocardium. Therefore, the heart was incised on the right side along the anterior descending coronary artery, in the vicinity of the septum. Here a firm, grey-white tumor was seen to be protruding into both ventricular chambers (Fig. 2A). Careful separation of the well-defined surface allowed resection of the tumor without substantial loss of functional myocardium (Fig. 2B). In this manner, the tumor was resected in 2 parts (4.5 x 1.3 x 2.5 cm and 2.5 x 2.0 x 2.0 cm; 25.5 g), leaving a large interventricular septal defect. The defect was closed with a Dacron patch and continuous suture. After closure of the ventriculotomies, cardiopulmonary bypass was discontinued easily and normal sinus rhythm resumed spontaneously. The aortic cross-clamp time was 97 minutes. The patient was extubated 6 hours after surgery and transferred from the intensive care unit the next day. Postoperative electrocardiography (ECG) exhibited sinus rhythm with a heart rate of 115 b/min and QRS axis deviation of S,Q1,, type. There was no bundle branch block (QRS time was 85 ms). As seen on the preoperative ECG, there were ST segment abnormalities and negative T waves in leads aVR, aVL, and V,-6. During Holter monitoring, no ventricular arrhythmias occurred. Postoperative echocardiographic examination showed a cavity filled by a hematoma in the area of the tumor resection. The ventricles contracted normally. A small pericardial effusion extending along the right ventricle decreased in subsequent examinations. On the 8th postoperative day, ECG-triggered MRI of the chest was performed. The axial views demonstrated the patch and a small hematoma in the interventricular septum. The ensuing postoperative 262
Removal of Giant Cardiac Fibroma from a 4-Year-Old
Fig. 2 A) Operative view through a right ventriculotomy. The tumor was mobilized with stay sutures. B) The dissection of the tumor from the interventricular septum is nearly complete. Note the size of the tumor in comparison with the size of the heart.
course was uneventful. The child was sent home in excellent condition on the 12th postoperative day. Histologic examination of the tumor showed the features typical of fibroma, i.e., bundles of collagen fibers and a small number of fibroblasts (Fig. 3). Immunohistologic and electron microscopic studies classified the collagen as types 1, 111, and V, and found a normal content of collagen fibers.
Discussion Atrial myxomas, the most common cardiac tumors in adults, are exceedingly rare in infants and children.',' On the other hand, approximately 90% of all fibromas (which themselves account for less than 5% of all cardiac tumors) occur in infants and children under 12 years of age.36,' In this age group, rhabdomyomas and fibromas are the most frequently encountered tumors.89 Vblume 22. Numbo-3, 1995
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