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Successful partial excision of an intramural fibroma of the left ventricle
Successful Partial Excision of an Intramural Fibroma of the Left Ventricle
GEORGE J. REUL, Jr., MD, FACC’ JIMMY F. HOWELL, MD, FACC PEDRO A. RUBIO, MD PAUL K. PETERSON, MD, FACC Houston,
Texas
A report of the only surviving adult to undergo partial excision of an intramural cardiac left ventricular fibroma is presented. Good results were obtained by relieving outflow tract obstruction through partial excision of the tumor from the septum and left ventricle. The need for conservative surgical treatment of patients with this rare, benign tumor is emphasized.
Intramural fibromas of the heart are thought to comprise less than 5 percent of primary heart tumors. l Since the first case of fibroma of the heart was reported by Luschka2 in 1855, more than 40 others have been reported. s-l5 This report describes successful partial resection of a massive intramural fibroma involving the posterior wall of the left ventricle and most of the interventricular septum.
Case Report
From the Cora and Webb Mading .Department of Surgery, Baylor College of Medicine, Houston, Texas. Manuscript accepted June 19, 1974. * Present address and address for reprints: George J. Reul. Jr., MD, Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
‘?
August 1975
A 31 year old man first presented to the Methodist Hospital in July 1969 with the chief complaints of syncopal episodes and chest pain of 4 years’ duration; the chest pain was described as dull and squeezing, followed by a sensation of dizziness. Attacks were precipitated by rapid changes in position or sudden exertion and were relieved by squatting. Past history revealed that a heart murmur was first heard at age 11 years, when a diagnosis of mitral stenosis was made. One year before admission, chest roentgenograms showed calcification in the mitral valve area. Examination of the patient was essentially normal except for the presence of a grade 2/6 systolic ejection murmur over the entire precordium. Heart rhythm was regular and there was no detectable cardiomegaly. Laboratory studies, including complete blood count, blood urea nitrogen, fasting blood sugar, serum electrolytes, serum lipids, uric acid and liver profile, were within normal limits. An electrocardiogram was normal except for occasional premature ventricular contractions. The chest roentgenogram revealed calcification in the area of the left ventricle and a 6.5 cm well marginated calcific lesion in the lower lobe of the right lung that was thought to be a hamartoma (Fig. 1). Chest fluoroscopy disclosed a large mass of calcium in the left ventricular wall. A kft r?entriculur angiogram (Fig. 2) demonstrated a calcified tubular mass arising from the free wall of the left ventricle just proximal to the aortic valve, descending on the outflow tract and projecting into it. Coronary arteriograms were normal and did not show involvement with the mass or staining in the area of mass. Left and right heart catheterization data were relatively normal, but exercise pressure data were not obtained. Surgical findings and treatment: A preoperative diagnosis of left ventricular tumor and lower right pulmonary lobe hamartoma was made and the chest was explored through a median sternotomy on August 20, 1969. The posterior left ventricle was nodular to palpation and, on elevation of the heart, a bulky tumor could be seen in this area. Total cardiopulmonary bypass was established and the aorta cross-clamped. Through a transverse aortotomy, a large, glistening nodular mass could be seen projecting from the endocardium just inferior to the aortic valve. The mass obstructed the outflow tract, which measured approximately 1.5 cm in diameter. A left atriotomy was performed and the hard mass was palpated through the mitral valve. It
The American Journal of CARDIOLOGY
Volume 36
INTRAMURAL
then became apparent that the nodular calcific mass involved the entire posterior left ventricular wall and most of the interventricular septum extending just below the mitral valve to the apex; the bulk of this mass occupied approximately one half of the left ventricular cavity. A biopsy specimen was taken, a frozen section examined, and the diagnosis of benign fibroma made. The left ventricular outflow tract was cleared by excision of the nodular mass to normal myocardium where possible. Nodules extending into the left atrium were also resected. Satisfactory withdrawal of the projecting portion of the tumor from the left ventricular cavity was performed without left ventriculotomy; because of the extent of involvement, the septum and remainder of the posterior wall were left intact with some of the benign tumor remaining. The aortotomy and atriotomy were closed, and the heart was returned to a normal sinus rhythm after defibrillation. The mass in the lower lobe of the right lung was then removed by wedge resection. Diagnosis, after examination of a frozen section of the lung mass, was fibrous mesothelioma. The sternotomy was closed. The patient’s postoperative course remained uneventful and a left ventricular angiogram 1 month postoperatively revealed no outflow tract obstruction despite persistence of part of the calcified mass in the left ventricular wall. The patient has now returned to gainful employment and is asymptomatic 4 l/2 years after surgery. Permanent sections of the fibrous tumor of the left ventricle are shown in Figure 3.
Discussion Patients reported to have intramural ventricular cardiac fibroma have ranged from newborns to 65 years of age but most have been children. Approximately 30 percent of the patients in previously reported cases died from “sudden death,” presumably because of interference with cardiac conduction or because of outflow tract obstruction.l The clinical diagnosis of intracardiac fibroma has been elusive. Findings have been confused with those of endocardial fibroelastosis4 and pulmonary stenosis.’ Our patient’s condition was diagnosed as mitral stenosis and insufficiency for several years. Most intramural fibromas present with ventricular calcifica-
LEFT VENTRIWLAR
FIBROMA-REUL
ET AL.
FIGURE 1. Preoperative chest roentgenogram. There is calcification in the left ventricle. Arrow points to a large calcified mass in the lower lobe of the right lung. later proved to be a fibrous mesothelioma of the lung.
tion and cardiomegaly; made by ventriculogram,
the final diagnosis has been in most cases preoperative-
lY*
Surgical excision: Because of the difficulty in diagnosis and the rarity of this tumor, only a few cases with successful surgical treatment have been reported. The first successful excision was reported by Parks et a1.3 in 1962. Their patient was a 26 month old boy with a left ventricular hamartoma composed of fibrous and cardiac muscle elements. The specimen weighed 61.5 g and calcification was present.
FIGURE 2. Left ventricular angiograms. A, during diastole, demonstrating the fibroma encroaching on the left ventricular outflow tract. The calcification can be noted in the anterior wall of the left ventricle (LV). B, during systole, there is further encroachment on the outflow tract. Note the large amount of calcium in the anterior wall and septum of the left ventricle.
August 1675
The American Journal of CARDIOLOGY
Volume 36
263
FIGURE 3. Microscopic sections of the excised tumor. A, the fibroma has the appearance of normal fibrous tissue with thick bundles of collagen surrounding normal fibrocytes. Note the lack of cardiac muscle. (Hematoxalin-eosin stain X90, reduced by 25 percent.) 8, high power view. Normal appearing fibrocytes are surrounded by large amounts of collagen. (Hematoxylin-eosin stain X900, reduced by 25 percent.)
Excision was accomplished from the lateral and posterior aspects of the ventricular wall. The patient was followed up for 2 years and remained symptom-free. Geha et al.’ reported successful excision of fibroma in two children. The first, a 27 month old boy, presented with syncopal attacks and arrhythmia. A ventriculogram revealed a partly calcified intramyocardial mass. The excised mass had occupied the interventricular groove, weighed 72 g and was 6 cm in diameter. The second patient was a 38 month old boy with a systolic murmur, left ventricular calcification and cardiomegaly; a 118 g fibroma was removed from the left ventricle. Both patients apparently fared well postoperatively. In 1968 Kay et a1.5 reported excision of an intramural fibroma of the right ventricle and interventricular septum in a 9 year old boy. The child presented with atria1 tachycardia, cardiomegaly and calcification of the right ventricle; a 115 g fibroma was removed from the right ventricle and septum necessitating closure of the defect with a Teflon@ patch. After 4 years of follow-up, the child was doing well despite slight calcification in the patch area. Thomsen et a1.7 and Williams et a1.8 reported successful excision of a left ventricular fibroma in a 17
A
August 1075
The American Journal of CARDtOLOGY
year old girl and a 30 month old boy. Hall6 at,tempted resection of an extensive fibroma of the left ventricle but was unsuccessful because excision of the entire fibroma compromised left ventricular function. Thus, only partial excision is indicated in some patients. Edlund and Holmadahl13 were the first to make a gross surgical diagnosis of intramyocardial fibroma, discovered in the left ventricle of a 32 month old child. Valledor14 attempted to remove an interventricular septal fibroma in an 8 month old infant, but the patient did not survive. Svejda and Tomasek12 accomplished partial excision of a left ventricular and septal fibroma in a 17 month old infant, who did not survive. Our patient represents the seventh successful operation for this rare condition and the first successful partial excision in an adult. Because of the biological activity of the fibroma and the extensive involvement of the left ventricle and septum, we elected to clear the outflow tract and increase left ventricular volume by excising only part of the tumor. Although it has been assumed that intramural cardiac fibromas grow by displacing myocardium, complete resection could not be accomplished in this case. Sufficient muscle mass remained to maintain good cardiac output, ade-
Volume 36
INTRAMURAL LEFT VENTRKMAR FtBRDMA-REUL ET AL.
quate left ventricular volume, a normal conduction pattern and an unimpeded outflow tract. Moreover, the avoidance of ventriculotomy in this case preserved the remaining left ventricular muscle and its blood supply. Four years postoperatively the patient has returned to full time employment and remains symptom-free.
The incidental finding of a fibrous mesothelioma of the lung probably bears no relation to the intracardisc fibroma. T’so and Teoh15 described a newborn infant with intramural cardiac fibroma who, at autopsy, was also found to have fibromas of the larynx and tongue and multiple fibromas of the lungs. However, these lesions were of uniform character.’
References 1. Geha AS, Weldman WH, Soule EH, et al: Intramural ventricular cardiac fibroma: successful removal in two cases and review of the literature. Circulation 36:427-440,. 1967 2. Luschka H: Ein Fibroid in.Herzfleische. Virchows Arch (Pathol Anat) 61343-347, 1655 3. Parks FR, Adams F, Longmlre WP Jr: Successful excision of a left ventricular hamartoma: report of a case. Circulation 26: 13151320, 1962 4. Hoen AG, Ellls EJ: Intramural fibroma of the heart. Am J Cardiol 17:579-564, 1966 5. Kay JH, lrwln G, Tsujl HK, et al: Successful excision of an intramural fibroma of the right ventricle and ventricular septum: four year follow-up. J Cardiovasc Surg 9:434-439, 1966 6. Hall JI: Intramural fibroma of the left ventricle. Thorax 26:461485, 1971 7. Thornsen JH, Corllss RJ, Selfers RD, et al: Left ventricular intramural fibroma. Am J Cardiol 26:726-730, 1971 a. Wllllams WG, Trusler GA, Fowler RS, et al: Left ventricular
9. 10.
11. 12. 13. 14. 15.
August 1975
myocardial fibroma: a case report and review of cardiac tumors in children. J Pediatr Surg 7:324-326. 1972 James U, Stanfleld MH: Case of fibroma of left ventricle in a child of 4 years. Arch Ois Childhood 30:167-192, 1955 McCue CM, Hennlngar GR, Davls E, et al: Congenital subaortic stenosis caused by a fibroma of the left ventricle. Pediatrics 16:372-377, 1955 Jernstrom P, Cremln JH: Intramural fibroma of the heart. Am J Clin Pathol 32250-256, 1959 Svejda J, Tomasek V: Fibrous hamartoma or so-called fibroma of the myocardium. J Pathol Bacterial 66:430-432. 1960 Edlund S, Holmadhl K: Primary tumor of the heart: report of a case. Acta Paediatr Stand 46:59, 1957 Valledor T, Barbolla L, Satsnowsky F: Fibroma of the heart: case report. Dis Chest 37:696-701, 1960 Ts’o TO, Teoh TB: Fibromatosis in an infant. J Pathol Bacterial a5:52i-523, 1963
The American Journal of CARDIOLOGY
Volume 36
286
GEORGE J. REUL, Jr., MD, FACC’ JIMMY F. HOWELL, MD, FACC PEDRO A. RUBIO, MD PAUL K. PETERSON, MD, FACC Houston,
Texas
A report of the only surviving adult to undergo partial excision of an intramural cardiac left ventricular fibroma is presented. Good results were obtained by relieving outflow tract obstruction through partial excision of the tumor from the septum and left ventricle. The need for conservative surgical treatment of patients with this rare, benign tumor is emphasized.
Intramural fibromas of the heart are thought to comprise less than 5 percent of primary heart tumors. l Since the first case of fibroma of the heart was reported by Luschka2 in 1855, more than 40 others have been reported. s-l5 This report describes successful partial resection of a massive intramural fibroma involving the posterior wall of the left ventricle and most of the interventricular septum.
Case Report
From the Cora and Webb Mading .Department of Surgery, Baylor College of Medicine, Houston, Texas. Manuscript accepted June 19, 1974. * Present address and address for reprints: George J. Reul. Jr., MD, Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
‘?
August 1975
A 31 year old man first presented to the Methodist Hospital in July 1969 with the chief complaints of syncopal episodes and chest pain of 4 years’ duration; the chest pain was described as dull and squeezing, followed by a sensation of dizziness. Attacks were precipitated by rapid changes in position or sudden exertion and were relieved by squatting. Past history revealed that a heart murmur was first heard at age 11 years, when a diagnosis of mitral stenosis was made. One year before admission, chest roentgenograms showed calcification in the mitral valve area. Examination of the patient was essentially normal except for the presence of a grade 2/6 systolic ejection murmur over the entire precordium. Heart rhythm was regular and there was no detectable cardiomegaly. Laboratory studies, including complete blood count, blood urea nitrogen, fasting blood sugar, serum electrolytes, serum lipids, uric acid and liver profile, were within normal limits. An electrocardiogram was normal except for occasional premature ventricular contractions. The chest roentgenogram revealed calcification in the area of the left ventricle and a 6.5 cm well marginated calcific lesion in the lower lobe of the right lung that was thought to be a hamartoma (Fig. 1). Chest fluoroscopy disclosed a large mass of calcium in the left ventricular wall. A kft r?entriculur angiogram (Fig. 2) demonstrated a calcified tubular mass arising from the free wall of the left ventricle just proximal to the aortic valve, descending on the outflow tract and projecting into it. Coronary arteriograms were normal and did not show involvement with the mass or staining in the area of mass. Left and right heart catheterization data were relatively normal, but exercise pressure data were not obtained. Surgical findings and treatment: A preoperative diagnosis of left ventricular tumor and lower right pulmonary lobe hamartoma was made and the chest was explored through a median sternotomy on August 20, 1969. The posterior left ventricle was nodular to palpation and, on elevation of the heart, a bulky tumor could be seen in this area. Total cardiopulmonary bypass was established and the aorta cross-clamped. Through a transverse aortotomy, a large, glistening nodular mass could be seen projecting from the endocardium just inferior to the aortic valve. The mass obstructed the outflow tract, which measured approximately 1.5 cm in diameter. A left atriotomy was performed and the hard mass was palpated through the mitral valve. It
The American Journal of CARDIOLOGY
Volume 36
INTRAMURAL
then became apparent that the nodular calcific mass involved the entire posterior left ventricular wall and most of the interventricular septum extending just below the mitral valve to the apex; the bulk of this mass occupied approximately one half of the left ventricular cavity. A biopsy specimen was taken, a frozen section examined, and the diagnosis of benign fibroma made. The left ventricular outflow tract was cleared by excision of the nodular mass to normal myocardium where possible. Nodules extending into the left atrium were also resected. Satisfactory withdrawal of the projecting portion of the tumor from the left ventricular cavity was performed without left ventriculotomy; because of the extent of involvement, the septum and remainder of the posterior wall were left intact with some of the benign tumor remaining. The aortotomy and atriotomy were closed, and the heart was returned to a normal sinus rhythm after defibrillation. The mass in the lower lobe of the right lung was then removed by wedge resection. Diagnosis, after examination of a frozen section of the lung mass, was fibrous mesothelioma. The sternotomy was closed. The patient’s postoperative course remained uneventful and a left ventricular angiogram 1 month postoperatively revealed no outflow tract obstruction despite persistence of part of the calcified mass in the left ventricular wall. The patient has now returned to gainful employment and is asymptomatic 4 l/2 years after surgery. Permanent sections of the fibrous tumor of the left ventricle are shown in Figure 3.
Discussion Patients reported to have intramural ventricular cardiac fibroma have ranged from newborns to 65 years of age but most have been children. Approximately 30 percent of the patients in previously reported cases died from “sudden death,” presumably because of interference with cardiac conduction or because of outflow tract obstruction.l The clinical diagnosis of intracardiac fibroma has been elusive. Findings have been confused with those of endocardial fibroelastosis4 and pulmonary stenosis.’ Our patient’s condition was diagnosed as mitral stenosis and insufficiency for several years. Most intramural fibromas present with ventricular calcifica-
LEFT VENTRIWLAR
FIBROMA-REUL
ET AL.
FIGURE 1. Preoperative chest roentgenogram. There is calcification in the left ventricle. Arrow points to a large calcified mass in the lower lobe of the right lung. later proved to be a fibrous mesothelioma of the lung.
tion and cardiomegaly; made by ventriculogram,
the final diagnosis has been in most cases preoperative-
lY*
Surgical excision: Because of the difficulty in diagnosis and the rarity of this tumor, only a few cases with successful surgical treatment have been reported. The first successful excision was reported by Parks et a1.3 in 1962. Their patient was a 26 month old boy with a left ventricular hamartoma composed of fibrous and cardiac muscle elements. The specimen weighed 61.5 g and calcification was present.
FIGURE 2. Left ventricular angiograms. A, during diastole, demonstrating the fibroma encroaching on the left ventricular outflow tract. The calcification can be noted in the anterior wall of the left ventricle (LV). B, during systole, there is further encroachment on the outflow tract. Note the large amount of calcium in the anterior wall and septum of the left ventricle.
August 1675
The American Journal of CARDIOLOGY
Volume 36
263
FIGURE 3. Microscopic sections of the excised tumor. A, the fibroma has the appearance of normal fibrous tissue with thick bundles of collagen surrounding normal fibrocytes. Note the lack of cardiac muscle. (Hematoxalin-eosin stain X90, reduced by 25 percent.) 8, high power view. Normal appearing fibrocytes are surrounded by large amounts of collagen. (Hematoxylin-eosin stain X900, reduced by 25 percent.)
Excision was accomplished from the lateral and posterior aspects of the ventricular wall. The patient was followed up for 2 years and remained symptom-free. Geha et al.’ reported successful excision of fibroma in two children. The first, a 27 month old boy, presented with syncopal attacks and arrhythmia. A ventriculogram revealed a partly calcified intramyocardial mass. The excised mass had occupied the interventricular groove, weighed 72 g and was 6 cm in diameter. The second patient was a 38 month old boy with a systolic murmur, left ventricular calcification and cardiomegaly; a 118 g fibroma was removed from the left ventricle. Both patients apparently fared well postoperatively. In 1968 Kay et a1.5 reported excision of an intramural fibroma of the right ventricle and interventricular septum in a 9 year old boy. The child presented with atria1 tachycardia, cardiomegaly and calcification of the right ventricle; a 115 g fibroma was removed from the right ventricle and septum necessitating closure of the defect with a Teflon@ patch. After 4 years of follow-up, the child was doing well despite slight calcification in the patch area. Thomsen et a1.7 and Williams et a1.8 reported successful excision of a left ventricular fibroma in a 17
A
August 1075
The American Journal of CARDtOLOGY
year old girl and a 30 month old boy. Hall6 at,tempted resection of an extensive fibroma of the left ventricle but was unsuccessful because excision of the entire fibroma compromised left ventricular function. Thus, only partial excision is indicated in some patients. Edlund and Holmadahl13 were the first to make a gross surgical diagnosis of intramyocardial fibroma, discovered in the left ventricle of a 32 month old child. Valledor14 attempted to remove an interventricular septal fibroma in an 8 month old infant, but the patient did not survive. Svejda and Tomasek12 accomplished partial excision of a left ventricular and septal fibroma in a 17 month old infant, who did not survive. Our patient represents the seventh successful operation for this rare condition and the first successful partial excision in an adult. Because of the biological activity of the fibroma and the extensive involvement of the left ventricle and septum, we elected to clear the outflow tract and increase left ventricular volume by excising only part of the tumor. Although it has been assumed that intramural cardiac fibromas grow by displacing myocardium, complete resection could not be accomplished in this case. Sufficient muscle mass remained to maintain good cardiac output, ade-
Volume 36
INTRAMURAL LEFT VENTRKMAR FtBRDMA-REUL ET AL.
quate left ventricular volume, a normal conduction pattern and an unimpeded outflow tract. Moreover, the avoidance of ventriculotomy in this case preserved the remaining left ventricular muscle and its blood supply. Four years postoperatively the patient has returned to full time employment and remains symptom-free.
The incidental finding of a fibrous mesothelioma of the lung probably bears no relation to the intracardisc fibroma. T’so and Teoh15 described a newborn infant with intramural cardiac fibroma who, at autopsy, was also found to have fibromas of the larynx and tongue and multiple fibromas of the lungs. However, these lesions were of uniform character.’
References 1. Geha AS, Weldman WH, Soule EH, et al: Intramural ventricular cardiac fibroma: successful removal in two cases and review of the literature. Circulation 36:427-440,. 1967 2. Luschka H: Ein Fibroid in.Herzfleische. Virchows Arch (Pathol Anat) 61343-347, 1655 3. Parks FR, Adams F, Longmlre WP Jr: Successful excision of a left ventricular hamartoma: report of a case. Circulation 26: 13151320, 1962 4. Hoen AG, Ellls EJ: Intramural fibroma of the heart. Am J Cardiol 17:579-564, 1966 5. Kay JH, lrwln G, Tsujl HK, et al: Successful excision of an intramural fibroma of the right ventricle and ventricular septum: four year follow-up. J Cardiovasc Surg 9:434-439, 1966 6. Hall JI: Intramural fibroma of the left ventricle. Thorax 26:461485, 1971 7. Thornsen JH, Corllss RJ, Selfers RD, et al: Left ventricular intramural fibroma. Am J Cardiol 26:726-730, 1971 a. Wllllams WG, Trusler GA, Fowler RS, et al: Left ventricular
9. 10.
11. 12. 13. 14. 15.
August 1975
myocardial fibroma: a case report and review of cardiac tumors in children. J Pediatr Surg 7:324-326. 1972 James U, Stanfleld MH: Case of fibroma of left ventricle in a child of 4 years. Arch Ois Childhood 30:167-192, 1955 McCue CM, Hennlngar GR, Davls E, et al: Congenital subaortic stenosis caused by a fibroma of the left ventricle. Pediatrics 16:372-377, 1955 Jernstrom P, Cremln JH: Intramural fibroma of the heart. Am J Clin Pathol 32250-256, 1959 Svejda J, Tomasek V: Fibrous hamartoma or so-called fibroma of the myocardium. J Pathol Bacterial 66:430-432. 1960 Edlund S, Holmadhl K: Primary tumor of the heart: report of a case. Acta Paediatr Stand 46:59, 1957 Valledor T, Barbolla L, Satsnowsky F: Fibroma of the heart: case report. Dis Chest 37:696-701, 1960 Ts’o TO, Teoh TB: Fibromatosis in an infant. J Pathol Bacterial a5:52i-523, 1963
The American Journal of CARDIOLOGY
Volume 36
286