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Cardiac fibroma
Cardiac fibroma Long-term fate after excision Between 1980 and July 1983 three infants and children with cardiac fibromas underwent surgical resection at Kobe Children's Hospital. Two of them survived and have an excellent clinical result 6 years and 7 years postoperatively. The results of late follow-up with the use of 24-hour dynamic electrocardiography, two-dimensional echocardiography, thallium-2Ot myocardial scintillation scan, and technetium 99m sodium pertechnetate-gated blood pool imaging have proved that the patients are free of arrhythmic episodes, free of recurrence of tumor, have no significant myocardial perfusion defect, and have normal left ventricular function. (J THoRAc CARDIOVASC SURG 1992;103:140-5)
Masahiro Yamaguchi, MD, Yuhei Hosokawa, MD, Hidetaka Ohashi, MD, Masanao Imai, MD, Yoshihiro Oshima, MD, and Katsumi Minamiji, MD, Kobe, Japan
Cardiac fibromas are rare lesions 1,2 and until 1962 were not amenable to surgical treatment.' The advances made during the past two decades in the diagnosis and surgical management of cardiac defects have given renewed importance to their early recognition and proper surgical treatment. Little is known, however, about the late course of patients after the operation. Between 1980 and July 1983, three patients with cardiac fibromas underwent surgical treatment at Kobe Children's Hospital. This is a report of findings of these patients and longterm follow-up of two survivors with the use of a 24-hour dynamic electrocardiogram, two-dimensional echocardiography, thallium-201 myocardial scintillation scan, and technetium 99m sodium pertechnetate-gated blood pool imaging (radionuclide angiography).
Case report CASE 1. A 2-month-oldfemale infant in no apparent distress was referred to Kobe Children's Hospital because of a heart murmur and cardiomegaly (cardiothoracic ratio 67%). The electrocardiogram showed right axis deviation, slight left ventricular hypertrophy, abnormal Q wavein precordial leads, and negative T wave in leads V4 and V5. Cardiac catheterization
From the Divisionof Cardiothoracic Surgery, Kobe Children's Hospital, and the Department of Cardiology, Himeji Cardiovascular Center, Kobe, Japan. Received for publication April 16, 1990. Accepted for publication Sept. II, 1990. Address for reprints: Masahiro Yamaguchi, MD, Chief, Division of Cardiothoracic Surgery, Kobe Children's Hospital, Takakuradai I-I-I, Suma-ku, Kobe 654, Japan.
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demonstrated no gross intracardiac abnormalities except for an elevated left atrial pressure with a mean of 13 mm Hg and a V wave of 29 mm Hg. Angiocardiographic examination revealed an extensive mass in the interventricular septum, extending from the apex up to the subaortic area, displacement and narrowing of both ventricular cavities, and severe mitral valve regurgitation caused by displacement of papillary muscles by the mass (Fig. 1). A coronary angiogram showed normal arterial distribution with scanty blood supply to the intracardiac mass. The patient was scheduled for thallium-201 myocardial scintigraphy in another hospital and was temporarily discharged from Kobe Children's Hospital. At her first visit to the outpatient clinic 2 weeks later, she had an episode of ventricular fibrillationthat was successfullymanaged by electric defibrillation. Surgical intervention was determined to be necessary because of recurrent ventricular fibrillation and persistent supraventricular premature beats; also, there was no improvement in pulmonary congestion despite respirator care with endotracheal intubation for 20 days. An operation was performed on May 11, 1980. Profound hypothermic circulatory arrest and cardiopulmonary bypass were used.The intraseptal tumor was rounded, firm, white-gray, and it had markedly thinned the overlying myocardium along the diaphragmatic and apical surfaces of the heart. Inspection of the ventricular cavities through incisionsof the apical aspects of both ventricles revealed that the intraseptal tumor extended from the apex to the base of the heart and from the anterior to the posterior free walls, occupying most of the right and left ventricular cavities. The papillary muscles of the mitral valve were markedly displaced by the tumor, and the valve leaflets were thickened and deformed. Rhabdomyoma was suspected after intraoperative frozen section. Total excision of the tumor was judged not to be feasible, and the tumor (3.3 by 5.5 by 1.3 em) was removed partially. The anterior papillary muscles of the mitral valvewere reattached to the septal wall near the apex. The ventricular incisions were closed by 4-0 Tevdek buttress
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Fig. 1. Case I. Angiocardiograms. Right ventriculogram (left) and left ventriculogram (right) by left atrial injection in lateral views. Arrows indicate margin of filling defect caused by compression by interventricular mass.
sutures (Deknatel, a division of Pfizer Hospital Products, Fall River, Mass.). After termination of warming by cardiopulmonary bypass, normal sinus rhythm resumed, but the patient could not be weaned from bypass because of low cardiac output. Permission for autopsy limited to the heart only was obtained. Histopathologic study of the heart revealed a huge, well-delineated, unencapsulated homogeneous mass occupying most of the interventricular septum. Microscopically it consisted of interlacing bundles of dense collagen and elastic fibrils that stained blue with Azan-Mallory stain. There were scattered areas of enclosed muscle fibers. No "primitive" cardiac muscle cells were recognized. Diagnosis of cardiac fibroma was also confirmed by electron microscopic findings of characteristic unit fibrils of collagen with the 640 Aperiodic banding. CASE 2. A 7-year-old boy was referred to us because of an arrhythmia discovered recently. The chest roentgenogram revealed a cardiothoracic ratio of 52% and a prominent bulge at the left cardiac border. The electrocardiogram showed multifocal ventricular and supraventricular extrasystoles occurring 5 to 10 times per minute. The 24-hour dynamic electrocardiogram disclosed bouts of ventricular tachycardia, seen 211 times an hour maximum, and frequent supraventricular extrasystoles (2230 times/4600 QRSs, maximum). The two-dimensional echocardiogram demonstrated a large intramural homogeneous mass in the lateral free wall of the left ventricle protruding into the left ventricular cavity (Fig. 2). Cardiac catheterization showed normal hemodynamic data except for a pressure gradient of 30 mm Hg between the apex and the outflow of the right ventricle. Angiography of the right ventricle revealed marked displacement and deformity of the cavity. A left ventriculogram showed a mass originating from the base and extending to the lateral free wall, with compression and displacement of the
upper portion of the left ventricular chamber. The left coronary artery was displaced and stretched around this mass but was free of obstruction. On September 16, 1982, a 6.0 by 4.5 by 4.5 em (45 gm) fibroma was excised with the aid of cardiopulmonary bypass, a crystalloid cardioplegic solution, and topical cardiac cooling. The yellow-white-gray intramural tumor was found to extend from the base to the lateral wall of the left ventricle, and it had markedly thinned the overlying myocardium. The overlying myocardium was incised longitudinally, and sharp dissection with a narrow margin was used to remove the tumor from the ventricular wall. The left ventricular cavity was not entered. The defect was reconstructed by apposition of the myocardial layers with an inner row of interrupted sutures and an outer (epicardial) row of continuous sutures. The patient tolerated the procedure well. The 24-hour dynamic electrocardiogram on the thirty-ninth postoperative day showed a marked decrease of premature ventricular contraction (95 times/4814 beats/hr. maximum), which was not seen in 17 of 24 hours. CASE 3. A 6-year-old boy was first seen at Kobe Children's Hospital for evaluation of a heart murmur. There was a grade 3/6 systolic ejection murmur along the left sternal border. A slightly increased cardiothoracic ratio (55%), with a prominent bulge at the left cardiac border, was revealed on chest x-ray examination. The electrocardiogram exhibited normal sinus rhythm with a QRS axis of -45 degrees, signs of left ventricular hypertrophy, and a negative T wave in leads I and aVL. The 24-hour dynamic electrocardiogram revealed the presence of occasional ventricular and supraventricular extrasystoles (I2 times/6457 beats, maximum). Two-dimensional echocardiography demonstrated a homogeneous mass extending from the anterior aspect of the ventricular septum to the anterior free wall
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MV Fig. 2. Case 2. Two-dimensional echocardiogram (fourchamber view) shows large intramural homogeneous mass in lateral free wall of left ventricle. IVS, Interventricular septum; LVand RV, left and right ventricles; LA and RA, left and right atria; PM, papillary muscle; MV, mitral valve. of the left ventricle located close to the aortic valve. Cardiac catheterization showed no gross abnormalities except for the pressure gradients of 6 mm Hg between the right ventricle and the pulmonary artery and 14 mm Hg between the left ventricle and the ascending aorta. Biventricular angiograms revealed a narrowing of the right ventricular outflow tract by a compression from the left side and an avascular intramural mass in the anterior free wall of the left ventricle, near the ventricular septum, extending from the base to the lower portion of the left ventricular free wall (Fig. 3). The thickest portion of the avascular area measured 25 mm. The left anterior descending coronary artery and its diagonal branch were stretched around this mass, but they were free of obstruction. In July 1983 a 66 by 40 by 37 mm (51 gm) fibroma was removed from the junction between the ventricular septum and the anterior free wall of the left ventricle. The tumor extended from the base of the heart to the apex of the left ventricle. The left anterior descending coronary artery was found to be stretched and dislocated to the right by the underlying tumor. The apical aspect of the tumor was incised along the anterior descending coronary artery. During dissection the diagonal branch of the left anterior descending coronary artery was not-
ed to run deep in the sulcus made by the impingement of the tumor on the artery, pinched and stretched by the tumor mass, which was preserved intact and freed from the tumor by extremely meticulous sharp dissection. This artery was noted hanging as a suspension bridge in the huge defect made by the excision of the tumor. The left ventricular cavity was not entered, and the defect was reconstructed in the same fashion as in patient 2. The child was discharged from the hospital 2 months after the operation after confirmation of satisfactory correction and satisfactory hemodynamic state by cardiac catheterization. Late follow-up. Both of the surviving patients were seen at the outpatient clinic once every 3 months until 2 years after the operation and once every 6 months thereafter. Standard electrocardiograms at rest and after exercise loading by Master's method, repeated at every visit, have revealed no abnormalities in either of the patients. The 24-hour dynamic electrocardiograms repeated at 5 years postoperatively disclosed no arrhythmias in either of the patients. Late postoperative two-dimensional echocardiography repeated at 5 years in patient 2 and at 4 years in patient 3 revealed normal configuration, with no space-occupying mass, and normal function of the left ventricle. Thallium-20l myocardial scintillation scan and radionuclide angiogram were ordered at 7 and 6 years after the operation in patients 2 and 3, respectively.Small areas of hypoperfusionwere noted at the anterolateral free wall near the base of the left ventricle in patient 2 and at the anterior wall, midportion to the base of the left ventricle near the interventricular septum in patient 3 (Fig. 4). These findings suggested scar formations at the sites coinciding with the area of tumor excisions. The radionuclide angiogram revealed normal wall motion of the left ventricle, with an ejection fraction of 71% in patient 2, and very mild localized hypokinesisat the midportion of the anterolateral wall, with a normal global ejection fraction of 62% in patient 3 (Fig. 5). Both of the patients are developing normally and enjoying full range of physical activity. .
Discussion Primary cardiac tumors are uncommon lesions in all age groups. The general prevalence was estimated at 0.01 % to 0.02% from a large series of un selected autopsies.l-? These tumors occur even more infrequently in children, but their clinical recognition is important because many of them are amenable to surgical resection. 3- 5 During infancy and childhood, rhabdomyomas, fibromas, and teratomas are the most common/' whereas in adults 35% to 50% of all tumors are myxomas.I: 8 To our knowledge about 95 cases of cardiac fibromas have been reported in .the English literaturev 5, 9-23 since the first documented case reported by Luschka in 1855 4; 83% of the reported cases were in children. Surgical excision was attempted in 41 cases (not including those in this report) since the first report by Svejda and Tomasek/"; 30 of the 41 patients had successful complete or subtotal resection and II had unsuccessful operations. The prevalence of cardiac fibroma was 0.14% of total cardiac operations in children in our institution (3/2175 cardiac operations in the past 19 years), which was high
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Fig. 3. Case 3. Left ventriculogram in anteroposterior (left) and lateral (right) views. There is a large and apparently singlefilling defect. Arrows indicate margin of filling defect.
Fig. 4. Case 3. Thallium-201 myocardialscintillation scan. ANT, Anteroposterior view; LAO, left anterior oblique view; LAT, lateral view. Arrows indicate area of hypoperfusion. compared with information from other reports. The fact that two of our patients were advised to have complete cardiac evaluation by a preschool routine check, although they had been free of any subjective symptoms, and that two-dimensional echocardiography suggested cardiac fibroma may indicate that the prevalence of operations in patients with cardiac fibroma will certainly increase in the
future because of the popularity of routine cardiac examination during infancy and early childhood and because of the advent of two-dimensional echocardiography and other noninvasive diagnostic modalities. The results of surgical intervention in patients with cardiac fibromas have been satisfactory in recent years.f- 5.10 except for patients who have (1) huge masses
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ANT
LAO F!g. 5. Case 3.Radionuclide angiogram. ANT, Anteroposterior view; LAO, leftanterioroblique view. Arrows show area of slight hypokinesis.
occupying most of both ventricular cavities, (2) severe congestive heart failure at initial presentation, or (3) recurrent ventricular tachycardia or fibrillation in the neonatal period or in early infancy!': 12 (as in patient I in this report). Retrospectively, a hard, boardlike, noncontractile mass left unresected in the interventricular septum as a result of incomplete excision should be blamed for the death of the first patient in this report. We believe that she would have had a better chance of survival if aggressive, complete removal of the fibroma and reconstruction of the entire ventricular septum (with a prosthetic patch, if necessary), together with reattachment of the anterior papillary muscle of the mitral valve, had been achieved successfully. With current refinement in techniques of cardiopulmonary bypass and of myocardial protection, successful resection oflarge intramural tumors has been increasingly realistic. Because of the invariably fatal prognosis of babies who have congestive cardiac failure or ventricular fibrillation (or both) resulting from large intramural cardiac fibromas.lv !3.!4 surgical excision of these tumors should be attempted even in the neonatal period. Every effort should be made to achieve complete resection so that the heart will be contractile after the operation. The prognosis not only for an incompletely resected tumor but also for a completely resected tumor must be guarded. There is always a possibility of residual tumor tissue because of the narrow margin of resection to preserve as much myocardium as possible for reconstruction
of the ventricle. Excellent long-term survival after complete resection of the fibroma was reported by Williams and associates" and by others.v 10 based on clinical observations, with survival up to 18 years after the operation. However, none of these reports have referred to the objective evidences of long-term cardiac reevaluation. The promising results of long-term follow-up of two survivors with the use of various noninvasive ·diagnostic methods in this report, in terms of absence of arrhythmic episodes and of signs of recurrence of residual tumor and preservation of a normally functioning left ventricle, should provide strong objective support to the clinical observations of the authors mentioned, that is, the patients who have undergone successful excision of a cardiac fibroma have an excellent probability of remaining free of disease. REFERENCES 1. Nadas HS, Ellison RC. Cardiac tumors in infancy. Am J Cardiol 1968;21 :363-6.
2. EngleMA, GlennF. Primarymalignanttumorofthe heart in infancy: casereport and review of the subject. Pediatrics 1955;15:562-70.
3. Park FR Jr, Adams F, Longmire WR Jr. Successful excisionof a left ventricular hamartoma: report of a case.Circulation 1962;26:1316-20. 4. Williams DB, Danielson GK, McGoon DC, Feldt RH, Edwards WD. Cardiac fibroma: long-term survival after excision. J THORAC CARDIOVASC SURG 1982;84:230-6. 5. Reece IJ, Cooley DA, Frazier OH, Hallman GL, Powers
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6. 7. 8. 9.
10.
II.
12.
13.
14. 15.
PL, Montero CG. Cardiac tumors: clinical spectrum and prognosisof lesionsother than classic benign myxoma in 20 patients. J THORAC CARDIOVASC SURG 1984;88:439-46. Schmaltz AA, Apitz J. Primary heart tumors in infancy and childhood. Cardiology 1981;67:12-22. Griffiths Gc. A reviewof primary tumors of the heart. Prog Cardiovasc Dis 1965;7:465-79. Wood P. Diseases of the heart and circulation. 3rd ed. London: Eyre & Spottiswoode, 1969. Geha AS, Weidman WH, Soule EH, McGoon DC. Intramural ventricular cardiac fibroma. Circulation 1967; 36: 427-40. Chan HS, Sonley MJ, Moes CAF, Daneman A, Smith CR, Martin OJ. Primary and secondary tumors of childhood involvingthe heart, pericardium and great vessels.Cancer 1985;56:825-36. Marin-Garcia J, Fitch CW, Shenefelt RE. Primary right ventricular tumor (fibroma) simulating cyanotic heart disease in a newborn. J Am Coli CardioI1984;3:868-71. Kutayli F, Malouf J, Slim M, Mufarrij A, Hatem J. Cardiac fibroma with tumor involvement of the mitral valve: diagnosis by cross-sectionalechocardiography. Eur Heart J 1988;9:563-6. Schwartz J, Saldivar V, Tio F, Kohler C. Interventricular fibroma and cystic renal dysplasia in a newborn. Pediatr PathoI1984;2:187-95. Van der Hauert LG. Cardiac tumors in infancy and childhood. Br Heart J 1971;33:125-32. Calhoun TR, Terry EE, Best EB, Sunbury TR. Myocardial fibroma or fibrous hamartoma. Ann Thorac Surg 1981; 32:407-10.
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16. Blumhardt R, Telepak RJ, Hartshorne MF, Crooks LA. Thallium imaging of benign cardiac tumor. Clin Nucl Med 1983;8:297-8. 17. Bini RM, Westaby S, Bargeron LM Jr, Pacifico AD, Kirklin JW. Investigation and management of primary cardiac tumors in infants and children. J Am Coli Cardiol 1983;2:351-7. 18. Charuzi Y, Mills H, Buchbinder NA, Marshall LA. Primary intramural cardiac tumor: long-term follow-up. Am Heart J 1983;106:414-9. 19. Zerkowski HR, Hentrich F, Doetsch NW, Rohm N. Partial replacement of the left ventricular wall for a large intramural fibroma. Pediatr Cardiol 1985;6:43-5. 20. Helmer S, Abghari R, Stone AJ, Lee CC. Detection of benign cardiac fibroma on thallium-20l imaging in an adult. Clin Nucl Med 1987;12:365-7. 21. AmrSS,AbuAI-RaghebSY,SoleimanNA,AI-DebsNR. Sudden, unexpected death due to cardiac fibroma. Am J Forensic Med PathoI1987;8:142-7. 22. Kimura M, Fujino T, Kawachi Y, Kitazato K. Successful excision of right ventricular fibroma associated with ventricular tachycardia: report of a six year survival. Jpn Heart J 1988;29:753-9. 23. Christano RA, Wolf BHM, Nijveld A, Lubbers LJ. An asymmetric bulging of the chest as a leading sign of a cardiac fibroma in a child. Eur J Pediatr 1988;147:96-8. 24. Svejda J, Tomasek V. Fibrous hamartoma or so-called fibroma of the myocardium. J Pathol Bacteriol 1960; 80: 430-2.
Masahiro Yamaguchi, MD, Yuhei Hosokawa, MD, Hidetaka Ohashi, MD, Masanao Imai, MD, Yoshihiro Oshima, MD, and Katsumi Minamiji, MD, Kobe, Japan
Cardiac fibromas are rare lesions 1,2 and until 1962 were not amenable to surgical treatment.' The advances made during the past two decades in the diagnosis and surgical management of cardiac defects have given renewed importance to their early recognition and proper surgical treatment. Little is known, however, about the late course of patients after the operation. Between 1980 and July 1983, three patients with cardiac fibromas underwent surgical treatment at Kobe Children's Hospital. This is a report of findings of these patients and longterm follow-up of two survivors with the use of a 24-hour dynamic electrocardiogram, two-dimensional echocardiography, thallium-201 myocardial scintillation scan, and technetium 99m sodium pertechnetate-gated blood pool imaging (radionuclide angiography).
Case report CASE 1. A 2-month-oldfemale infant in no apparent distress was referred to Kobe Children's Hospital because of a heart murmur and cardiomegaly (cardiothoracic ratio 67%). The electrocardiogram showed right axis deviation, slight left ventricular hypertrophy, abnormal Q wavein precordial leads, and negative T wave in leads V4 and V5. Cardiac catheterization
From the Divisionof Cardiothoracic Surgery, Kobe Children's Hospital, and the Department of Cardiology, Himeji Cardiovascular Center, Kobe, Japan. Received for publication April 16, 1990. Accepted for publication Sept. II, 1990. Address for reprints: Masahiro Yamaguchi, MD, Chief, Division of Cardiothoracic Surgery, Kobe Children's Hospital, Takakuradai I-I-I, Suma-ku, Kobe 654, Japan.
12/1/26451 140
demonstrated no gross intracardiac abnormalities except for an elevated left atrial pressure with a mean of 13 mm Hg and a V wave of 29 mm Hg. Angiocardiographic examination revealed an extensive mass in the interventricular septum, extending from the apex up to the subaortic area, displacement and narrowing of both ventricular cavities, and severe mitral valve regurgitation caused by displacement of papillary muscles by the mass (Fig. 1). A coronary angiogram showed normal arterial distribution with scanty blood supply to the intracardiac mass. The patient was scheduled for thallium-201 myocardial scintigraphy in another hospital and was temporarily discharged from Kobe Children's Hospital. At her first visit to the outpatient clinic 2 weeks later, she had an episode of ventricular fibrillationthat was successfullymanaged by electric defibrillation. Surgical intervention was determined to be necessary because of recurrent ventricular fibrillation and persistent supraventricular premature beats; also, there was no improvement in pulmonary congestion despite respirator care with endotracheal intubation for 20 days. An operation was performed on May 11, 1980. Profound hypothermic circulatory arrest and cardiopulmonary bypass were used.The intraseptal tumor was rounded, firm, white-gray, and it had markedly thinned the overlying myocardium along the diaphragmatic and apical surfaces of the heart. Inspection of the ventricular cavities through incisionsof the apical aspects of both ventricles revealed that the intraseptal tumor extended from the apex to the base of the heart and from the anterior to the posterior free walls, occupying most of the right and left ventricular cavities. The papillary muscles of the mitral valve were markedly displaced by the tumor, and the valve leaflets were thickened and deformed. Rhabdomyoma was suspected after intraoperative frozen section. Total excision of the tumor was judged not to be feasible, and the tumor (3.3 by 5.5 by 1.3 em) was removed partially. The anterior papillary muscles of the mitral valvewere reattached to the septal wall near the apex. The ventricular incisions were closed by 4-0 Tevdek buttress
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Fig. 1. Case I. Angiocardiograms. Right ventriculogram (left) and left ventriculogram (right) by left atrial injection in lateral views. Arrows indicate margin of filling defect caused by compression by interventricular mass.
sutures (Deknatel, a division of Pfizer Hospital Products, Fall River, Mass.). After termination of warming by cardiopulmonary bypass, normal sinus rhythm resumed, but the patient could not be weaned from bypass because of low cardiac output. Permission for autopsy limited to the heart only was obtained. Histopathologic study of the heart revealed a huge, well-delineated, unencapsulated homogeneous mass occupying most of the interventricular septum. Microscopically it consisted of interlacing bundles of dense collagen and elastic fibrils that stained blue with Azan-Mallory stain. There were scattered areas of enclosed muscle fibers. No "primitive" cardiac muscle cells were recognized. Diagnosis of cardiac fibroma was also confirmed by electron microscopic findings of characteristic unit fibrils of collagen with the 640 Aperiodic banding. CASE 2. A 7-year-old boy was referred to us because of an arrhythmia discovered recently. The chest roentgenogram revealed a cardiothoracic ratio of 52% and a prominent bulge at the left cardiac border. The electrocardiogram showed multifocal ventricular and supraventricular extrasystoles occurring 5 to 10 times per minute. The 24-hour dynamic electrocardiogram disclosed bouts of ventricular tachycardia, seen 211 times an hour maximum, and frequent supraventricular extrasystoles (2230 times/4600 QRSs, maximum). The two-dimensional echocardiogram demonstrated a large intramural homogeneous mass in the lateral free wall of the left ventricle protruding into the left ventricular cavity (Fig. 2). Cardiac catheterization showed normal hemodynamic data except for a pressure gradient of 30 mm Hg between the apex and the outflow of the right ventricle. Angiography of the right ventricle revealed marked displacement and deformity of the cavity. A left ventriculogram showed a mass originating from the base and extending to the lateral free wall, with compression and displacement of the
upper portion of the left ventricular chamber. The left coronary artery was displaced and stretched around this mass but was free of obstruction. On September 16, 1982, a 6.0 by 4.5 by 4.5 em (45 gm) fibroma was excised with the aid of cardiopulmonary bypass, a crystalloid cardioplegic solution, and topical cardiac cooling. The yellow-white-gray intramural tumor was found to extend from the base to the lateral wall of the left ventricle, and it had markedly thinned the overlying myocardium. The overlying myocardium was incised longitudinally, and sharp dissection with a narrow margin was used to remove the tumor from the ventricular wall. The left ventricular cavity was not entered. The defect was reconstructed by apposition of the myocardial layers with an inner row of interrupted sutures and an outer (epicardial) row of continuous sutures. The patient tolerated the procedure well. The 24-hour dynamic electrocardiogram on the thirty-ninth postoperative day showed a marked decrease of premature ventricular contraction (95 times/4814 beats/hr. maximum), which was not seen in 17 of 24 hours. CASE 3. A 6-year-old boy was first seen at Kobe Children's Hospital for evaluation of a heart murmur. There was a grade 3/6 systolic ejection murmur along the left sternal border. A slightly increased cardiothoracic ratio (55%), with a prominent bulge at the left cardiac border, was revealed on chest x-ray examination. The electrocardiogram exhibited normal sinus rhythm with a QRS axis of -45 degrees, signs of left ventricular hypertrophy, and a negative T wave in leads I and aVL. The 24-hour dynamic electrocardiogram revealed the presence of occasional ventricular and supraventricular extrasystoles (I2 times/6457 beats, maximum). Two-dimensional echocardiography demonstrated a homogeneous mass extending from the anterior aspect of the ventricular septum to the anterior free wall
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MV Fig. 2. Case 2. Two-dimensional echocardiogram (fourchamber view) shows large intramural homogeneous mass in lateral free wall of left ventricle. IVS, Interventricular septum; LVand RV, left and right ventricles; LA and RA, left and right atria; PM, papillary muscle; MV, mitral valve. of the left ventricle located close to the aortic valve. Cardiac catheterization showed no gross abnormalities except for the pressure gradients of 6 mm Hg between the right ventricle and the pulmonary artery and 14 mm Hg between the left ventricle and the ascending aorta. Biventricular angiograms revealed a narrowing of the right ventricular outflow tract by a compression from the left side and an avascular intramural mass in the anterior free wall of the left ventricle, near the ventricular septum, extending from the base to the lower portion of the left ventricular free wall (Fig. 3). The thickest portion of the avascular area measured 25 mm. The left anterior descending coronary artery and its diagonal branch were stretched around this mass, but they were free of obstruction. In July 1983 a 66 by 40 by 37 mm (51 gm) fibroma was removed from the junction between the ventricular septum and the anterior free wall of the left ventricle. The tumor extended from the base of the heart to the apex of the left ventricle. The left anterior descending coronary artery was found to be stretched and dislocated to the right by the underlying tumor. The apical aspect of the tumor was incised along the anterior descending coronary artery. During dissection the diagonal branch of the left anterior descending coronary artery was not-
ed to run deep in the sulcus made by the impingement of the tumor on the artery, pinched and stretched by the tumor mass, which was preserved intact and freed from the tumor by extremely meticulous sharp dissection. This artery was noted hanging as a suspension bridge in the huge defect made by the excision of the tumor. The left ventricular cavity was not entered, and the defect was reconstructed in the same fashion as in patient 2. The child was discharged from the hospital 2 months after the operation after confirmation of satisfactory correction and satisfactory hemodynamic state by cardiac catheterization. Late follow-up. Both of the surviving patients were seen at the outpatient clinic once every 3 months until 2 years after the operation and once every 6 months thereafter. Standard electrocardiograms at rest and after exercise loading by Master's method, repeated at every visit, have revealed no abnormalities in either of the patients. The 24-hour dynamic electrocardiograms repeated at 5 years postoperatively disclosed no arrhythmias in either of the patients. Late postoperative two-dimensional echocardiography repeated at 5 years in patient 2 and at 4 years in patient 3 revealed normal configuration, with no space-occupying mass, and normal function of the left ventricle. Thallium-20l myocardial scintillation scan and radionuclide angiogram were ordered at 7 and 6 years after the operation in patients 2 and 3, respectively.Small areas of hypoperfusionwere noted at the anterolateral free wall near the base of the left ventricle in patient 2 and at the anterior wall, midportion to the base of the left ventricle near the interventricular septum in patient 3 (Fig. 4). These findings suggested scar formations at the sites coinciding with the area of tumor excisions. The radionuclide angiogram revealed normal wall motion of the left ventricle, with an ejection fraction of 71% in patient 2, and very mild localized hypokinesisat the midportion of the anterolateral wall, with a normal global ejection fraction of 62% in patient 3 (Fig. 5). Both of the patients are developing normally and enjoying full range of physical activity. .
Discussion Primary cardiac tumors are uncommon lesions in all age groups. The general prevalence was estimated at 0.01 % to 0.02% from a large series of un selected autopsies.l-? These tumors occur even more infrequently in children, but their clinical recognition is important because many of them are amenable to surgical resection. 3- 5 During infancy and childhood, rhabdomyomas, fibromas, and teratomas are the most common/' whereas in adults 35% to 50% of all tumors are myxomas.I: 8 To our knowledge about 95 cases of cardiac fibromas have been reported in .the English literaturev 5, 9-23 since the first documented case reported by Luschka in 1855 4; 83% of the reported cases were in children. Surgical excision was attempted in 41 cases (not including those in this report) since the first report by Svejda and Tomasek/"; 30 of the 41 patients had successful complete or subtotal resection and II had unsuccessful operations. The prevalence of cardiac fibroma was 0.14% of total cardiac operations in children in our institution (3/2175 cardiac operations in the past 19 years), which was high
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Fig. 3. Case 3. Left ventriculogram in anteroposterior (left) and lateral (right) views. There is a large and apparently singlefilling defect. Arrows indicate margin of filling defect.
Fig. 4. Case 3. Thallium-201 myocardialscintillation scan. ANT, Anteroposterior view; LAO, left anterior oblique view; LAT, lateral view. Arrows indicate area of hypoperfusion. compared with information from other reports. The fact that two of our patients were advised to have complete cardiac evaluation by a preschool routine check, although they had been free of any subjective symptoms, and that two-dimensional echocardiography suggested cardiac fibroma may indicate that the prevalence of operations in patients with cardiac fibroma will certainly increase in the
future because of the popularity of routine cardiac examination during infancy and early childhood and because of the advent of two-dimensional echocardiography and other noninvasive diagnostic modalities. The results of surgical intervention in patients with cardiac fibromas have been satisfactory in recent years.f- 5.10 except for patients who have (1) huge masses
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ANT
LAO F!g. 5. Case 3.Radionuclide angiogram. ANT, Anteroposterior view; LAO, leftanterioroblique view. Arrows show area of slight hypokinesis.
occupying most of both ventricular cavities, (2) severe congestive heart failure at initial presentation, or (3) recurrent ventricular tachycardia or fibrillation in the neonatal period or in early infancy!': 12 (as in patient I in this report). Retrospectively, a hard, boardlike, noncontractile mass left unresected in the interventricular septum as a result of incomplete excision should be blamed for the death of the first patient in this report. We believe that she would have had a better chance of survival if aggressive, complete removal of the fibroma and reconstruction of the entire ventricular septum (with a prosthetic patch, if necessary), together with reattachment of the anterior papillary muscle of the mitral valve, had been achieved successfully. With current refinement in techniques of cardiopulmonary bypass and of myocardial protection, successful resection oflarge intramural tumors has been increasingly realistic. Because of the invariably fatal prognosis of babies who have congestive cardiac failure or ventricular fibrillation (or both) resulting from large intramural cardiac fibromas.lv !3.!4 surgical excision of these tumors should be attempted even in the neonatal period. Every effort should be made to achieve complete resection so that the heart will be contractile after the operation. The prognosis not only for an incompletely resected tumor but also for a completely resected tumor must be guarded. There is always a possibility of residual tumor tissue because of the narrow margin of resection to preserve as much myocardium as possible for reconstruction
of the ventricle. Excellent long-term survival after complete resection of the fibroma was reported by Williams and associates" and by others.v 10 based on clinical observations, with survival up to 18 years after the operation. However, none of these reports have referred to the objective evidences of long-term cardiac reevaluation. The promising results of long-term follow-up of two survivors with the use of various noninvasive ·diagnostic methods in this report, in terms of absence of arrhythmic episodes and of signs of recurrence of residual tumor and preservation of a normally functioning left ventricle, should provide strong objective support to the clinical observations of the authors mentioned, that is, the patients who have undergone successful excision of a cardiac fibroma have an excellent probability of remaining free of disease. REFERENCES 1. Nadas HS, Ellison RC. Cardiac tumors in infancy. Am J Cardiol 1968;21 :363-6.
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