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Ann Thorac Surg 2003;76:1929-1934
© 2003 The Society of Thoracic Surgeons

Original article: cardiovascular

Surgical resection of ventricular cardiac fibromas: early and late results

John M. Cho, MDa, Gordon K. Danielson, MDa*, Francisco J. Puga, MDa, Joseph A. Dearani, MDa, Christopher G. A. McGregor, MDa, Henry D. Tazelaar, MDb, Donald J. Hagler, MDc

a Division of Cardiovascular Surgery, Mayo Medical Center, Rochester, Minnesota, USA
b Division of Anatomic Pathology, Mayo Medical Center, Rochester, Minnesota, USA
c Division of Pediatric Cardiology, Mayo Medical Center, Rochester, Minnesota, USA

Accepted for publication June 30, 2003.

* Address reprint requests to Dr Danielson, Division of Cardiovascular Surgery, Mayo Clinic, Joseph 5-200, 200 First St SW, Rochester, MN 55905, USA.
e-mail: danielson.gordon{at}mayo.edu


   Abstract
Top
 Abstract
Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 References
 
BACKGROUND: Cardiac fibromas are rare tumors. Indications for and preferred type of operation are controversial, and little is known about early and late results of operation.

METHODS: We retrospectively reviewed records of 18 patients who underwent resection of ventricular cardiac fibromas from 1964 to October 2002. Follow-up was obtained from current medical records and recent telephone and written correspondence.

RESULTS: Seventeen patients had complete resection and 1 had subtotal resection of one or more fibromas. Tumors were located in the left ventricle (n = 12), septum (n = 4), or right ventricle (n = 2). A 2-month-old infant died intraoperatively. None of the surviving 17 patients had complete heart block. Follow-up lasted up to 33.7 years for a total of 172.1 patient-years. There were no late deaths. Fourteen patients were asymptomatic, 1 was in New York Heart Association functional class II, and 2 were in class III. There was no recurrence of tumor after complete resection and no change in size of residual tumor in the 1 patient who underwent subtotal resection.

CONCLUSIONS: Although cardiac transplantation has been suggested by some as a preferable operation, most ventricular fibromas, even though extensive, can be completely resected with excellent early and late results. For patients with tumors extending into critical locations, subtotal excision can also give excellent late survival.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 Acknowledgments
 References
 
Parks and associates reported the first successful excision of a ventricular fibroma (then called fibrous hamartoma) in 1962 [1]. Since that time, numerous case reports and reviews of the literature have defined the clinical spectrum and pathologic features of this tumor [2–5]. The report of Williams and colleagues of 4 patients who underwent excision of a ventricular fibroma constituted our early institutional experience [6]. We now add 14 patients to that series and provide recent follow-up of all patients to further define the early and late outcomes of patients after surgical resection of ventricular fibromas.


   Material and methods
Top
 Abstract
 Introduction
 Material and methods
Results
 Comment
 Acknowledgments
 References
 
We retrospectively reviewed 18 consecutive patients who underwent surgical excision of ventricular fibromas at the Mayo Clinic between July 7, 1964, and October 31, 2002. Demographic and other patient-related data were obtained from hospital records.

Recent follow-up was obtained by telephone for 11 patients, by written correspondence from the primary care physician or cardiologist for 9 patients, and by current clinic records for 2 patients. A descriptive analysis of the data was performed. No statistical analyses were run because of the small patient sample size. This review was approved by our Institutional Review Board on December 28, 1999.


   Results
Top
 Abstract
 Introduction
 Material and methods
 Results
Comment
 Acknowledgments
 References
 
Symptoms before referral for operation included dyspnea (n = 6), syncope (n = 5), easy fatigability (n = 5), angina/chest pain (n = 4), congestive heart failure (n = 2), palpitations (n = 2), cough (n = 2), and cardiac arrest (n = 1). Five patients had episodes of ventricular tachycardia; 1 had slow ventricular tachycardia 40% of the time. One patient was found to have a cardiac mass in utero by fetal echocardiography. Five patients were asymptomatic.

Preoperative tumor biopsies were performed elsewhere for 5 patients by left thoracotomy (n = 3, 1 of whom had ventricular tachycardia and fibrillation during biopsy, requiring resuscitation), median sternotomy (n = 1), or cardiac catheterization (n = 1). Two patients were referred to our institution for cardiac transplantation, as the tumor was considered to be too large for safe resection; another patient was offered transplantation elsewhere.

The most significant physical finding in 9 patients was a systolic murmur along the left lower sternal border. A diastolic murmur at the apex was noted in 2 patients.

Electrocardiography demonstrated conduction abnormalities in 8 patients. Left ventricular hypertrophy was noted in 4 patients and right ventricular hypertrophy in 1 patient. Findings suggestive of ischemia were recorded in 6 patients. Cardiomegaly was noted on preoperative chest radiograph in 12 of 18 patients. The cardiothoracic ratio ranged from 0.48 to 0.76 (mean 0.65). Calcification and an irregular cardiac silhouette were noted on five radiographs each.

A ventricular mass was first confirmed by echocardiography in 15 patients, cardiac catheterization in 2 patients, and computed tomography scan in 1 patient. Preoperative echocardiography confirmed calcification in the same 5 patients found to have calcification on chest radiograph. Mitral regurgitation was moderate in 4 patients and mild in 2 patients. The left ventricular ejection fraction was 0.50 or higher in all 15 patients who underwent preoperative echocardiography. Cardiac catheterization demonstrated a filling defect in the left ventricle only in 5 patients, in the right ventricle only in 1 patient, and in both ventricles in 2 patients. The ventricular septum was widened in 2 patients. Displacement of the left anterior descending coronary artery was noted in 4 patients and displacement of the posterior descending coronary artery was noted in 1 patient. Progressive enlargement of the fibroma was noted in 3 patients on serial echocardiograms (numbers 12, 13, 18 in Tables 1 through 3).


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  		Table 1. Operative Findings and Tumor Location

 

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  		Table 2. Tumor Characteristics

 

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  		Table 3. Follow-Up Data (17 Patients)

 
The time from initial symptoms until operation for fibroma resection ranged from 12 days to 7.1 years (mean 1.2 ± 2.1 years). Age at operation varied from 2 months to 61.4 years (median 7.5 years, mean 18.0 years). All 18 operations were performed on an elective basis through a median sternotomy utilizing cardiopulmonary bypass with standard aortic and right atrial or bicaval cannulation. The aorta was not cross-clamped in 4 early cases; 1 patient had a 9-minute cross-clamp under moderate hypothermia; the ventricular fibromas in the remaining 13 patients were resected with the aid of aortic cross-clamping and cold blood cardioplegia for myocardial protection. Aortic cross-clamp time ranged from 9 to 109 minutes (mean 67.2 minutes) and bypass times ranged from 59 to 171 minutes (mean 101.6 minutes). Tumors were located primarily in the left ventricle in 12 patients, the ventricular septum in 4 patients, and the right ventricle in 2 patients. Operative findings and tumor locations in relation to cardiac structures are shown in Table 1.

The ventricular fibromas were solitary in 17 patients and multiple [3] in 1 patient. The cardiac fibromas could be excised completely in 17 patients. One patient (number 6) had a subtotal resection because of extension of tumor into critical areas near the mitral valve; a 4.5-cm remnant of tumor was left in situ. Entry into the cavity of the left ventricle, right ventricle, or both was common; only 4 patients had sufficient myocardium between the fibroma and the ventricular cavity for resection to be performed without entrance into the cavity (patients 5, 10, 14, 17). Postexcision ventricular septal defects were closed primarily in 3 patients and with a prosthetic patch in 1 patient. Associated procedures included electrophysiologic mapping, sequential saphenous vein graft to the proximal and distal anterior descending coronary artery, coronary sinus repair, mitral anterior leaflet plication and annuloplasty, and mitral annuloplasty in 1 patient each.

A large ventricular fibroma in a 2-month-old infant extended into the crux of the left ventricle posteriorly (patient 16, Tables 1 and 2). Tumor resection in that area and subsequent ventricular repair probably compromised the left coronary artery, and the patient could not be separated from cardiopulmonary bypass. There was no complete heart block in the surviving 17 patients, which included the 3 patients referred for or offered cardiac transplantation. Morbidity included atrial fibrillation, post-pericardiotomy syndrome, and left lower lobe collapse necessitating bronchoscopy in 1 patient each. Early postoperative echocardiography showed mild or no mitral regurgitation in 10 patients and moderate residual mitral regurgitation in 2 patients, 1 of whom had undergone mitral annuloplasty. Mean hospital stay was 9 days (range 5 to 12 days).

Microscopic examination confirmed the diagnosis of ventricular fibroma. Characteristics of the tumor specimens are found in Table 2. The left ventricular tumor in one 3-year-old child weighed 106 g, 1.4 times the expected normal heart weight (76 g) (patient 4), and the right ventricular tumor in one 8-month-old infant weighed 100 g, 2.9 times the expected normal heart weight (35 g) (patient 8). All 17 surviving patients were in New York Heart Association functional class I before or at the time of follow-up 1 year after operation.

Late follow-up ranged up to 33.7 years (mean 10.1 years, median 8.6 years) for a total of 172.1 patient-years (Table 3). There were no late deaths. One patient had a left ventricular ejection fraction of 0.45 at 2-year follow-up. All recent echocardiograms on the other patients demonstrated an ejection fraction of more than 0.50. Fourteen patients had no symptoms. One 62-year-old patient (number 10) who had chronic fatigue for 10 years before operation was in New York Heart Association functional class II. Two patients (numbers 3 and 9) were in class III; one weighed 400 pounds and the other was 66 years old and had severe mitral regurgitation that had increased from mild regurgitation 6 years earlier. To our knowledge, no patient has had a recurrence of or has developed new ventricular tachycardia.

Mitral valve replacement was performed for severe mitral regurgitation in 2 patients 2.5 and 24.9 years after tumor resection (patients 11 and 2, respectively). Patient 2 was noted to have a 2-cm mass that was thought to be a recurrent tumor at 11-year follow-up. The mass was followed for another 13 years until symptoms of mitral regurgitation developed. At the time of mitral valve replacement, the mass was removed and found to be only scar tissue. Including this patient, there has been no recurrence of tumor after complete resection in all 16 late survivors. The 1 patient with residual tumor after subtotal resection (number 6) had no change in tumor size during a 14.8-year follow-up.


   Comment
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
Acknowledgments
 References
 
Cardiac fibromas have pathologic features similar to those of fibromas occurring in other parts of the body. These benign tumors are characteristically solitary, circumscribed, firm, gray-white, and often centrally calcified (Fig 1). They are considered neoplasms of fibroblastic origin and do not have a distinct capsule. Although not invasive, they may entrap islands of myocardium during expansile growth of the irregular advancing edge of the tumor, giving the appearance of infiltration of the myocardium [5, 6] (Fig 2). Microscopically, the tumors are of low to intermediate cellularity and are composed of spindle cells arranged in fascicles with abundant intervening collagen; mitotic figures are rare, and foci of dystrophic calcification are common.



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  		Fig 1. Cut surface of excised ventricular fibroma (patient 12).

 


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  		Fig 2. Ventricular fibroma (blue) interdigitating with myocardium (red) at edges of the fibroma giving appearance of tumor infiltration. (Masson's trichrome stain x 25 before 46% reduction.)

 
Although cardiac fibromas are known to occur also in the atria or other cardiac structures, all of the cardiac fibromas evaluated at our institution have occurred in the ventricles. The propensity of cardiac fibromas to involve the ventricles, left more often than right, may simply reflect the relative amount and distribution of myocardium and fibroblast-rich interstitium within the heart.

Today, echocardiography is the most expedient way to diagnose the presence of a cardiac mass. In 15 patients in this series, echocardiography first identified the cardiac fibroma. Echocardiography also provides other valuable information including function of the cardiac valves, particularly the mitral valve, which is frequently distorted by left ventricular fibromas. Additional studies including magnetic resonance imaging, computed tomography, and cardiac angiography may help define tissue characteristics and extent of tumor involvement (Fig 3). The coronary arteries, especially the left anterior descending artery, are frequently displaced or occluded by ventricular fibromas; coronary angiography can identify or exclude these complications. A chest radiograph which shows cardiomegaly, an abnormal cardiac contour, and calcification is highly suggestive of a ventricular fibroma. However, only 2 patients had all three findings. Five patients had two findings and 12 patients had one of these radiographic findings.



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  		Fig 3. Magnetic resonance imaging (patient 18). Axial T1-weighted MR image demonstrating a large ventricular septal fibroma (wide white arrow). At this level, the right atrium (open arrow) and a small portion of the right ventricle (white triangle) are seen, but the left ventricular cavity is not visualized due to the large mass effect of the fibroma. The right coronary artery (narrow black arrow with white underscore) is noted in the atrioventricular groove between the right atrium and right ventricle.

 
The clinical findings in our patients were varied and similar to those reported in previous literature reviews [2–4]. They included dyspnea, syncope, easy fatigability, and angina/chest pain. Five patients experienced episodes of ventricular tachycardia, and 1 infant had a cardiac arrest. Five patients were asymptomatic. There is general agreement that operation should be performed when ventricular fibromas are causing symptoms. However, opinions diverge regarding operation for asymptomatic patients [4, 7–10]. The main concern stems from numerous reports of sudden death associated with ventricular fibromas [3, 4, 11]. The mechanism appears to be fatal ventricular tachycardia [12–15]. In some cases, the fatal event had no preceding symptoms or known arrhythmia. We are aware of an asymptomatic pediatric patient elsewhere who was known to have a ventricular fibroma but who died suddenly before operation was scheduled. In view of that case and the fact that 1 patient in this series had a preoperative cardiac arrest and 5 patients had runs of ventricular tachycardia, we favor offering operation to patients with ventricular fibromas, especially when the tumors are large, even though the patients are asymptomatic. Additionally, operation is favored for asymptomatic patients with enlarging tumors to prevent progressive cardiac deformity and atrioventricular valve dysfunction. Similar opinions have been expressed by others [2–4, 16, 17].

Admittedly, the behavior of cardiac fibromas is unpredictable. We know of one 5-year-old patient who died from Reye's disease and who had an incidental 1.8-cm apical left ventricular fibroma. Additionally, we know of another patient, aged 20 years, who was found to have a probable 2-cm ventricular fibroma near the crux of the heart posteriorly; the tumor was echo-dense and had flecks of calcium. The patient declined operation and has been followed elsewhere. He had been asymptomatic for 4 years, when he had an episode of tachycardia and fell to the floor without loss of consciousness. Electrocardiographic monitoring showed two episodes of nonsustained ventricular tachycardia during sleep. An electrophysiologic study showed no inducible atrial or ventricular tachycardia. Subsequently, he has been asymptomatic for 9 years; there has been no change in size of the ventricular tumor during the 13 years of observation. Because the prevalence of asymptomatic ventricular fibromas in the general population is unknown and fibroma behavior is unpredictable, it is not possible to define the exact risk of sudden death for patients newly diagnosed with a ventricular fibroma.

Ventricular fibromas do not have a true capsule and do not shell out. However, a plane can be readily developed by sharp dissection between the firm white tumor and the softer red myocardium (Fig 4). Fibroma borders are easy to discern, and resecting a margin of myocardium is unnecessary because the tumor is benign. Moderate hypothermia (30° to 32°C), aortic cross-clamping, and intermittent cold-blood cardioplegia provide optimal conditions for myocardial protection, accurate tumor resection, preservation of surrounding structures, and cardiac repair. The 1 patient early in this series who had an injury to the left anterior descending coronary artery necessitating coronary artery bypass grafting had undergone fibroma resection without the use of aortic cross-clamping and cardioplegia. This patient was the same one who had an incomplete resection of tumor (number 6).



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  		Fig 4. Dissection of ventricular fibroma (patient 12; patient's head is at top).

 
After resection of ventricular septal fibromas, reconstruction of the septum can be achieved by closure of the septum in layers or by placement of a prosthetic patch. No residual ventricular septal defects were noted in this series. Entry into one or both ventricular cavities is common; reconstruction is usually possible by direct closure, although prosthetic patch repair is feasible, if necessary. Large ventricular fibromas may extend close to the atrioventricular node and conduction system. Although no patient in this series developed complete heart block or required a pacemaker, the possible need for a pacemaker after tumor resection should be recognized.

An anatomic and surgical challenge can arise when the tumor distorts or involves the papillary muscles. In the left ventricle, this development may cause mitral regurgitation either preoperatively or after tumor resection. Current techniques for mitral valve repair are usually successful in restoring a competent mitral valve. However, 2 patients in this series required mitral valve replacement during follow-up, and a third patient has been advised to have mitral valve repair or replacement for increasing mitral regurgitation. In the right ventricle, fibromas more commonly cause obstruction of the tricuspid valve or right ventricular outflow tract [18, 19].

Perhaps the greatest surgical challenge is presented by a ventricular fibroma that involves the superior septum and crux of the heart posteriorly. The left coronary artery courses in the atrioventricular groove close to this area and so is vulnerable to surgical trauma. Additionally, defects created in the heart posteriorly may require reconstruction with a prosthetic patch, and they typically are difficult to repair without injury to adjacent coronary arteries. The only death in this series was after resection of a tumor in this location in an infant. For such tumors, subtotal excision of the fibroma, leaving the portion at greatest risk for injury to the heart in situ, might provide a better option than complete resection. The 1 patient in this series who underwent a subtotal excision of ventricular fibroma was alive and well 14.8 years later without an increase in size of the residual tumor. Favorable late results after subtotal excision of cardiac fibromas, including abolishment of symptoms and ventricular arrhythmias, have also been reported by others [3, 4, 10, 20].

Two patients with large ventricular fibromas were explored and biopsied elsewhere and were referred to our institution for cardiac transplantation, and a third was endocardially biopsied elsewhere and offered transplantation. All 3 patients underwent successful complete tumor resections. Although cardiac transplantation has been suggested by some as the preferable operation for large fibromas [4, 21, 22], we believe that most ventricular fibromas, even though extensive, can be completely resected with excellent late patient survival. We agree subtotal resection is a good option for tumors with extension into critical areas such as the crux of the heart, and reserve cardiac transplantation for the rare complex fibroma whose excision would cause irreparable damage to essential cardiac structures.


   Acknowledgments
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
References
 
We appreciate the opportunity of including data on patients operated on by Dr Thomas A. Orszulak, Dr James R. Pluth, and Dr Robert B. Wallace and the contribution of Figure 3 by Drs Jan Bogaert and Paul R. Julsrud.


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 Acknowledgments
 References
 

  1. Parks F.R., Adams F., Longmire W.R., Jr Successful excision of a left ventricular hamartoma. Report of a case. Circulation 1962;26:1316-1320.[Abstract/Free Full Text]
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  7. Massin M.M., Bricteux G., Tebache M. Giant cardiac fibroma in an asymptomatic neonate. Clin Cardiol 1999;22:750.[Medline]
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  9. Stiller B., Hetzer R., Meyer R., et al. Primary cardiac tumours: when is surgery necessary?. Eur J Cardiothorac Surg 2001;20:1002-1006.[Abstract/Free Full Text]
  10. Beghetti M., Gow R.M., Haney I., Mawson J., Williams W.G., Freedom R.M. Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J 1997;134:1107-1114.[Medline]
  11. Cina S.J., Smialek J.E., Burke A.P., Virmani R., Hutchins G.M. Primary cardiac tumors causing sudden death: a review of the literature. Am J Forensic Med Pathol 1996;17:271-281.[Medline]
  12. Gonen R., Degani S., Samberg I., Sharf M., Grishkan A., Abend M. Congenital. cardiac fibroma associated with fetal arrhythmia. Int J Gynaecol Obstet 1983;21:39-43.
  13. Wong J.A., Fishbein M.C. Cardiac fibroma resulting in fatal ventricular arrhythmia. Circulation 2000;101:e168-170.[Free Full Text]
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  16. Ackerman J., McKeown P., Gunasekaran S., Spicer D. Pathologic case of the month. Cardiac fibroma. Arch Pediatr Adolesc Med 1995;149:199-200.
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