Surgical experience with 77 primary cardiac tumors
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《血管的通路杂志》
Department of Cardiac Surgery, Heart Center, University of Leipzig, Strümpellstr. 39, 04289 Leipzig, Germany
Presented at the 53rd International Congress of the European Society for Cardiovascular Surgery, Ljubljana, Slovenia, June 2–5, 2004.
Abstract
Background: To assess the prognosis and to develop management strategies for primary cardiac tumors all patients were included in an ongoing study. Method: From Oct. 1994 until December 2003 we prospectively evaluated all patients with cardiac tumors. Follow up examinations were performed every 12 months. Results: There were a total of 77 primary cardiac tumors. Seventy-three were benign: myxoma (n=59), papillary fibroelastoma (n=11), lipoma (n=2), fibroma (n=1) and four malignant sarcoma (n=4). The myxoma group consisted of 19 males and 40 females aged 12 to 88 years. Myxomas were located in the: left atrium in 50 (85%), left ventricle in 3 (5%), right atrium in 4 (7%) and on the mitral valve in 2 patients (3%). Papillary fibroelastoma was located on the aortic valve (n=4), mitral valve (n=3), right ventricle (n=2), left ventricle (n=1) and tricuspid valve (n=1). Both lipoma and the only fibroma were located in the right atrium. There were 4 primary cardiac sarcomas, located in the right ventricle (n=2), the pulmonary valve (n=1) and left atrium (n=1). Minimal invasive right thoracotomy was utilized in 19 of 73 patients all with benign tumor. There were two early deaths (3%): a myxoma patient with triple vessel disease and a LVEF less than 30% and one sarcoma patient. No recurrence or late death was observed in the group of benign tumors. However, two remaining patients with sarcoma had recurrent disease 10 and 15 month later, respectively. All patients were followed up with a total follow up of 203 patient years. Conclusion: Surgical excision of benign cardiac tumors is a safe and curative treatment, which is feasible using minimally invasive right thoracotomy approach and provides excellent results. However, therapy of malignant cardiac tumors continues to have a poor prognosis despite individualization of approach.
Key Words: Cardiac tumor; Myxoma; Papillary fibroelastoma; Cardiac sarcoma; Minimally invasive surgery
1. Introduction
Primary cardiac tumors are often reported to have a low incidence and represent only 0.3% of all open heart operations [1]. However, modern cardiac imaging transformed primary cardiac tumors from a condition rarely diagnosed before autopsy [2,3] to a curable form of heart disease.
In fact 50 years ago, Bahnson and Newman [4] performed the first surgical excision of a primary cardiac tumor using the inflow occlusion technique and in 1954 Crafoord reported the first successful myxoma excision using the extracorporal circulation [5].
The objective of the present study was, therefore, to evaluate our experience with all primary cardiac tumors seen at our institution with emphasis on new management strategies.
2. Material and methods
From October 1994 until December 2003 we evaluated prospectively all patients with a diagnosis of primary cardiac tumors presenting at our institution. Patient follow-up was complete with a total follow up of 203 patient years (mean 5.1 years). Patients and/or physicians were contacted by telephone interview to assess late functional status. We have also previously reported two patients from this series as case reports [6,7].
2.1. Patient evaluation
Patients had preoperative echocardiography evaluation (100%), chest X-ray (100%) and cardiac catheterization (70%). Intraoperative transesophageal echocardiography (TEE) was used in all patients to confirm, identify, tumor location and to monitor left ventricular function, filling status and valve function after tumor resection.
2.2. Surgical technique
Operation was undertaken on an urgent basis in all patients (within one week). Standard operative technique was a median sternotomy and cardiopulmonary bypass (CPB), using aortic and bicaval cannulation. In myxoma patients, en bloc excision of the septum around the base of the tumor pedicle was performed followed by primary ASD closure. In nonmyxoma tumors operative strategy was individualized but aimed towards curative resection.
Based on our experience in less invasive valve, congenital heart and CABG surgery we applied a less invasive technique through a right minithoracotomy for the resection of 19 intracardiac benign tumors (26%).
Pathological diagnosis was obtained for each tumor.
3. Results
There were a total of 77 patients with primary cardiac tumors (27 males and 50 females). Fifty-nine patients had myxoma, 14 patients had non-myxoma benign tumors and 4 sarcomas (Tables 1 and 2).
Routine chest X-ray performed in all cases, did not reveal any tumor calcification as classically described [1].
Concomittent surgery was performed in 10 patients: coronary artery bypass grafting (n=8), mitral valve repair (n=2), aortic valve replacement (n=1) and tricuspid valve repair (n=1).
The surgical approach to the myxoma was the left atrium in 44 patients, the right atrium in 4 patients, biatrial in 10 patients and a ventriculotomy in 1 patient. Primary closure of the surgically created defect was performed in 50 patients, whereas a pericardial patch closure was needed in 4 patients. The majority of the myxomas originated from the atrial septum or in the region of the foramen ovale.
Myxomas were located in the: left atrium in 50 (85%), left ventricle in 3 (5%), right atrium in 4 (7%) and on the mitral valve in 2 patients (3%).
Two of the myxoma patients (4%) were suspected to have a Carney complex. Cardiac myxomas are benign mesenchymal tumors that can present as components of the human autosomal dominant disorder Carney complex. Syndromic cardiac myxomas are associated with spotty pigmentation of the skin and endocrinopathy.
A 12-year-old boy and a 35-year-old woman. In the case of the 35-year-old woman we found a mutation of the tumor suppressor gene PRKAR1 at 17q22–24 locus. Her daughter was also found to have the same mutation with multiple extracardiac blue nevi and scin myxomas but no cardiac manifestation as of yet. In the other case, molecular investigation was negative.
There was one early death in the myxoma group: a patient with triple vessel coronary artery disease, a LVEF of less than 30% who suffered a perioperative myocardial infarction.
Less invasive technique was utilized in 19 patients with benign primary cardiac tumors resulting in good exposure and complete tumor resection (Fig. 2). In Table 3, we compare our myxoma patients who were conventionally operated to the patients with a right thoracotomy group (MIC group). None of the patients approached by MIC technique required conversion to hemisternotomy.
The surgical approach to the sarcoma was a median sternotomy in all four cases. In one case, a complete resection was not possible as the patient demonstrated evidence of tumor growth and embolization to the pulmonary arteries. This patient died on the 4th postoperative day secondary to intractable right heart failure. Autopsy was not performed. In the second patient (44 years, male), the tumor was excised with part of the right atrium which required reconstruction with autologous pericardium, tricuspid valve repair and aortocoronary bypass to RCA. A third patient with sarcoma on the pulmonary valve underwent curative resection and is still alive 20 month after surgery without signs of recurrence.
3.1. Pathologic findings
Forty-five patients (76%) of the myxoma group had a pedunculated tumor. The tumor size ranged from 10 mmx15 mm to 55x60 mm and the histological examination confirmed the diagnosis of myxoma in every instance. In one patient, the histological examination revealed a fibroma (myxoma suspected). The histological features of the papillary fibroelastomas, lipoma and fibroma were typical in all cases. The histological features of the malignant tumors (n=4) revealed a low-grade sarcoma, a high-grade angiosarcoma and two low-grade leiomyosarcoma [6].
3.2. Postoperative course and follow up
The 30 day mortality was 2/77 (3%) with none in the MIC group. Postoperative complications occurred in 9 patients: one acute abdomen on the 7th postoperative day due to bowel strangulation, 4 patients had episodes of supraventricular arrythmia controlled medically and two pericardial effusions requiring drainage. Two patients after resection of left atrial myxomas demonstrated persistent AV block 2 (already preoperatively diagnosed) requiring pacemaker insertion, without relation to the surgical procedure.
In follow up no recurrence, late complications or late death has been observed in the group of benign tumors. However, both patients with sarcoma had recurrence of disease and died 10 and 15 months after initial resection. The patient with leiomyosarcoma of the pulmonary valve is alive 20 month after resection without evidence of recurrrence.
4. Discussion
Myxomas are the most common primary cardiac tumors [1,2] and as reported by many others these constituted the majority of tumors in this consecutive series. We have shown that surgical results for myxomas can be excellent [8–11] but that follow up is necessary to identify rare recurrence [12,13], especially in cases with Carney complex [14]. Recently, a mutation in the PRKAR1 gene was identified in families with Carney complex [15] and we could find this mutation in one suspected familiy of our myxoma series.
Among all cardiac tumors, less than 10% are papillary fibroelastomas [1]. In our series we identified 11 patients with fibroelastomas (Fig. 1). Symptoms of these non-malignant tumors are rare and often misleading although severe complications such as cerebral embolization or coronary occlusion and sudden death may occur [16–18]. Therefore, any of these intracardiac papillary fibroelastomas should be resected by urgent indication. In contrast to myxoma, papillary fibroelastoma is typically attached to valves and preferentially found on the aortic valve [7,19]. Nevertheless, other locations such as the mitral valve [20], tricuspid valve [21], cordae [22] and the free wall of the ventricle have been reported [23] and demonstrated in our series.
Cardiac fibroma mostly occur in children. These tumors are solitary. Surgical resection is successful in most of the patients, particulary if the tumor is localized and does not involve vital structures.
Cardiac sarcoma can involve any and all chambers of the heart, with the right atrium involved most frequently as was the case in our series. An intracavitary growth may cause right ventricular inflow and outflow obstruction or can progressively obliterate the right or left ventricle or both ventricles. Heart transplantation is a potential option in selected primary sarcomas of the heart but has not been necessary as of yet.
Depending on the location of sarcoma, a variety of manifestations may suggest cardiac involvement: congestive heart failure; second- or third-degree atrioventricular block; atrial tachycardia and transient ischemic attack.
Augmentation of the right atrium and, if necessary, the inferior or superior caval vein, with a pericardial patch may prevent narrowing of these structures at the time of resection. For this purpose we prefer to use untreated autologous pericardium because this is pliable and adjusts optimally to pressure changes in the right atrium or venae cavae. Surgery in primary malignancies is often palliative to relieve obstruction, and adjuvant radio- and/or chemotherapy has been reported to prolong survival but rarely provide cure as was the case in 3 patients from our series with malignant sarcomas [24].
In summary, we have shown a modern series of primary cardiac tumors treated surgically with the added option of minimally invasive approaches via a right small thoracotomy (Fig. 2). We have demonstrated that less invasive cardiac tumor resection is technically feasible to a large proportion of patients and can be applied with good functional and favorable cosmetic results to benign tumors. This minimally invasive approach has also been suggested by other institutions and is gaining in popularity [25].
References
McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Hartmann WH, Cowan WR. eds. Atlas of Tumor Pathology. Second Series 1978;Washington, DC: Armed Forces Institute of Pathology 20–25. Fascicle 15.
Prichard RW. Tumors of the heart. Arch Pathol 1951;51:98–128.
Chiari H. Myxom des rechten Vorhofs: plotzlicher Tod durch Geschwulstembolie der Lungenschlagader. Centralbl Allg Pathol Pathol Anat 1931;52:241–246.
Bahnson HT, Spencer FC, Andrus EC. Diagnosis and treatment of intracavitary myxomas of the heart. Ann Surg 1957;145:915–925.
Chitwood Jr WR. Clarence Crafoord and the first successful resection of a cardiac myxoma. Ann Thorac Surgery 1992;54:997–998.
Fabricius AM, Autschbach R, Lochhaas L, Brose S, Mohr FW. Primary left-atrial leiomyosarcoma. Thorac Cardiov Surg 2000;48:306–308.
Bossert T, Diegeler A, Spyrantis N, Mohr FW. Papillary fibroelastoma of the aortic valve with obturation of the left coronary ostium causing repeated drop attacks. J Heart Valve Disease 2000;9:842–843.
Bortolotti U, Maraglino G, Rubino M, Santini F, Mazzucco A, Milano A, Fasoli G, Livi U, Thiene G, Gallucci V. Surgical excision of intracardiac myxomas: a 20-year follow-up. Ann Thorac Surg 1990;49:449–453.
Bhan A, Mehrotra R, Choudhary SK, Sharma R, Prabhakar D, Airan B, Kumar AS, Venugopal P. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thorac Surg 1998;66:810–813.
Bjessmo S, Ivert T. Cardiac myxoma: 40 years' experience in 63 patients. Ann Thorac Surg 1997;63:697–700.
Dapper F, Grlach G, Hoffmann C, Fitz H, Marck P, Scheld HH. Primary cardiac tumors – clinical experiences and late results in 48 patients. Thorac Cardiovasc Surgeon 1988;36:80–85.
Shinfeld A, Katsumata T, Westaby S. Recurrent cardiac myxoma: seeding or multifocal disease Ann Thorac Surg 1998;66:285–288.
Reber D, Birnbaum DE. Recurrent cardiac myxoma: why it occurs. J Cardiovasc Surg 2001;42:345–348.
Basson CT, MacRae CA, Korf B, Merliss A. Genetic Heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol 1997;79:994–995.
Casey M, Vaughan CJ, He J, Hatcher CJ, Winter JM, Weremowicz S, Montgomery K, Kucherlapati R, Morton CC, Basson CT. Mutations in the protein kinase A R1alpha regulatory subunit cause familial cardiac myxomas and Carney complex. J Clin Invest 2000;106:R31–R38.
Shelh M. Multiplane transesophageal echocardiography detection of papillary fibroelastomas of the aortic valve causing a stroke. Eur Heart J 1997;18:702–703.
Pasteuning HW, Zijnen P, van der Aa MAC, Peters JH, van Geldorp TR. Papillary fibroelastoma of the aortic valve in a patient with an acute myocardial infarction. J Am Soc Echocardiogr 1996;9:897–900.
Butterworth JS, Poindexter CA. Papilloma of cusp of the aortic valve. Report of a patient with sudden death. Circulation 1973;48:213–215.
Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59:538–541.
Bedi HS, Sharma VK, Mishra M, Kasliwal RR, Trehan N. Papillary Fibroelastoma of the mitral valve associated with rheumatic mitral stenosis. Eur J Cardiothorac Surg 1995;9:54–55.
Lee CC, Celik C, Lajos TZ. Excision of papillary fibroelastoma arising from the septal leaflet of the tricuspid valve. J Card Surg 1995;10:589–591.
Gallo R, Kumar N, Prabhakar G, Awada A, Maalouf Y, Duran CMG. Papillary fibroelastoma of mitral valve chorda. Ann Thorac Surg 1993;55:1576–1577.
Espada R, Talwalker NG, Wilcox G, Kleiman NS, Verani MS. Visualization of ventricular fibroelastoma with a video-assisted thoracoscope. Ann Thorac Surg 1997;63:221–223.
Pessoto R, Sivestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol 1997;60:91–94.
Ko PJ, Chang CH, Lin PJ, Chu JJ, Tsai FC, Hsueh C, Yang MW. Video-assisted minimal access in excision of left atrial myxoma. Ann Thorac Surg 1998;66:1301–1305.(Torsten Bossert, Jan F. G)
Presented at the 53rd International Congress of the European Society for Cardiovascular Surgery, Ljubljana, Slovenia, June 2–5, 2004.
Abstract
Background: To assess the prognosis and to develop management strategies for primary cardiac tumors all patients were included in an ongoing study. Method: From Oct. 1994 until December 2003 we prospectively evaluated all patients with cardiac tumors. Follow up examinations were performed every 12 months. Results: There were a total of 77 primary cardiac tumors. Seventy-three were benign: myxoma (n=59), papillary fibroelastoma (n=11), lipoma (n=2), fibroma (n=1) and four malignant sarcoma (n=4). The myxoma group consisted of 19 males and 40 females aged 12 to 88 years. Myxomas were located in the: left atrium in 50 (85%), left ventricle in 3 (5%), right atrium in 4 (7%) and on the mitral valve in 2 patients (3%). Papillary fibroelastoma was located on the aortic valve (n=4), mitral valve (n=3), right ventricle (n=2), left ventricle (n=1) and tricuspid valve (n=1). Both lipoma and the only fibroma were located in the right atrium. There were 4 primary cardiac sarcomas, located in the right ventricle (n=2), the pulmonary valve (n=1) and left atrium (n=1). Minimal invasive right thoracotomy was utilized in 19 of 73 patients all with benign tumor. There were two early deaths (3%): a myxoma patient with triple vessel disease and a LVEF less than 30% and one sarcoma patient. No recurrence or late death was observed in the group of benign tumors. However, two remaining patients with sarcoma had recurrent disease 10 and 15 month later, respectively. All patients were followed up with a total follow up of 203 patient years. Conclusion: Surgical excision of benign cardiac tumors is a safe and curative treatment, which is feasible using minimally invasive right thoracotomy approach and provides excellent results. However, therapy of malignant cardiac tumors continues to have a poor prognosis despite individualization of approach.
Key Words: Cardiac tumor; Myxoma; Papillary fibroelastoma; Cardiac sarcoma; Minimally invasive surgery
1. Introduction
Primary cardiac tumors are often reported to have a low incidence and represent only 0.3% of all open heart operations [1]. However, modern cardiac imaging transformed primary cardiac tumors from a condition rarely diagnosed before autopsy [2,3] to a curable form of heart disease.
In fact 50 years ago, Bahnson and Newman [4] performed the first surgical excision of a primary cardiac tumor using the inflow occlusion technique and in 1954 Crafoord reported the first successful myxoma excision using the extracorporal circulation [5].
The objective of the present study was, therefore, to evaluate our experience with all primary cardiac tumors seen at our institution with emphasis on new management strategies.
2. Material and methods
From October 1994 until December 2003 we evaluated prospectively all patients with a diagnosis of primary cardiac tumors presenting at our institution. Patient follow-up was complete with a total follow up of 203 patient years (mean 5.1 years). Patients and/or physicians were contacted by telephone interview to assess late functional status. We have also previously reported two patients from this series as case reports [6,7].
2.1. Patient evaluation
Patients had preoperative echocardiography evaluation (100%), chest X-ray (100%) and cardiac catheterization (70%). Intraoperative transesophageal echocardiography (TEE) was used in all patients to confirm, identify, tumor location and to monitor left ventricular function, filling status and valve function after tumor resection.
2.2. Surgical technique
Operation was undertaken on an urgent basis in all patients (within one week). Standard operative technique was a median sternotomy and cardiopulmonary bypass (CPB), using aortic and bicaval cannulation. In myxoma patients, en bloc excision of the septum around the base of the tumor pedicle was performed followed by primary ASD closure. In nonmyxoma tumors operative strategy was individualized but aimed towards curative resection.
Based on our experience in less invasive valve, congenital heart and CABG surgery we applied a less invasive technique through a right minithoracotomy for the resection of 19 intracardiac benign tumors (26%).
Pathological diagnosis was obtained for each tumor.
3. Results
There were a total of 77 patients with primary cardiac tumors (27 males and 50 females). Fifty-nine patients had myxoma, 14 patients had non-myxoma benign tumors and 4 sarcomas (Tables 1 and 2).
Routine chest X-ray performed in all cases, did not reveal any tumor calcification as classically described [1].
Concomittent surgery was performed in 10 patients: coronary artery bypass grafting (n=8), mitral valve repair (n=2), aortic valve replacement (n=1) and tricuspid valve repair (n=1).
The surgical approach to the myxoma was the left atrium in 44 patients, the right atrium in 4 patients, biatrial in 10 patients and a ventriculotomy in 1 patient. Primary closure of the surgically created defect was performed in 50 patients, whereas a pericardial patch closure was needed in 4 patients. The majority of the myxomas originated from the atrial septum or in the region of the foramen ovale.
Myxomas were located in the: left atrium in 50 (85%), left ventricle in 3 (5%), right atrium in 4 (7%) and on the mitral valve in 2 patients (3%).
Two of the myxoma patients (4%) were suspected to have a Carney complex. Cardiac myxomas are benign mesenchymal tumors that can present as components of the human autosomal dominant disorder Carney complex. Syndromic cardiac myxomas are associated with spotty pigmentation of the skin and endocrinopathy.
A 12-year-old boy and a 35-year-old woman. In the case of the 35-year-old woman we found a mutation of the tumor suppressor gene PRKAR1 at 17q22–24 locus. Her daughter was also found to have the same mutation with multiple extracardiac blue nevi and scin myxomas but no cardiac manifestation as of yet. In the other case, molecular investigation was negative.
There was one early death in the myxoma group: a patient with triple vessel coronary artery disease, a LVEF of less than 30% who suffered a perioperative myocardial infarction.
Less invasive technique was utilized in 19 patients with benign primary cardiac tumors resulting in good exposure and complete tumor resection (Fig. 2). In Table 3, we compare our myxoma patients who were conventionally operated to the patients with a right thoracotomy group (MIC group). None of the patients approached by MIC technique required conversion to hemisternotomy.
The surgical approach to the sarcoma was a median sternotomy in all four cases. In one case, a complete resection was not possible as the patient demonstrated evidence of tumor growth and embolization to the pulmonary arteries. This patient died on the 4th postoperative day secondary to intractable right heart failure. Autopsy was not performed. In the second patient (44 years, male), the tumor was excised with part of the right atrium which required reconstruction with autologous pericardium, tricuspid valve repair and aortocoronary bypass to RCA. A third patient with sarcoma on the pulmonary valve underwent curative resection and is still alive 20 month after surgery without signs of recurrence.
3.1. Pathologic findings
Forty-five patients (76%) of the myxoma group had a pedunculated tumor. The tumor size ranged from 10 mmx15 mm to 55x60 mm and the histological examination confirmed the diagnosis of myxoma in every instance. In one patient, the histological examination revealed a fibroma (myxoma suspected). The histological features of the papillary fibroelastomas, lipoma and fibroma were typical in all cases. The histological features of the malignant tumors (n=4) revealed a low-grade sarcoma, a high-grade angiosarcoma and two low-grade leiomyosarcoma [6].
3.2. Postoperative course and follow up
The 30 day mortality was 2/77 (3%) with none in the MIC group. Postoperative complications occurred in 9 patients: one acute abdomen on the 7th postoperative day due to bowel strangulation, 4 patients had episodes of supraventricular arrythmia controlled medically and two pericardial effusions requiring drainage. Two patients after resection of left atrial myxomas demonstrated persistent AV block 2 (already preoperatively diagnosed) requiring pacemaker insertion, without relation to the surgical procedure.
In follow up no recurrence, late complications or late death has been observed in the group of benign tumors. However, both patients with sarcoma had recurrence of disease and died 10 and 15 months after initial resection. The patient with leiomyosarcoma of the pulmonary valve is alive 20 month after resection without evidence of recurrrence.
4. Discussion
Myxomas are the most common primary cardiac tumors [1,2] and as reported by many others these constituted the majority of tumors in this consecutive series. We have shown that surgical results for myxomas can be excellent [8–11] but that follow up is necessary to identify rare recurrence [12,13], especially in cases with Carney complex [14]. Recently, a mutation in the PRKAR1 gene was identified in families with Carney complex [15] and we could find this mutation in one suspected familiy of our myxoma series.
Among all cardiac tumors, less than 10% are papillary fibroelastomas [1]. In our series we identified 11 patients with fibroelastomas (Fig. 1). Symptoms of these non-malignant tumors are rare and often misleading although severe complications such as cerebral embolization or coronary occlusion and sudden death may occur [16–18]. Therefore, any of these intracardiac papillary fibroelastomas should be resected by urgent indication. In contrast to myxoma, papillary fibroelastoma is typically attached to valves and preferentially found on the aortic valve [7,19]. Nevertheless, other locations such as the mitral valve [20], tricuspid valve [21], cordae [22] and the free wall of the ventricle have been reported [23] and demonstrated in our series.
Cardiac fibroma mostly occur in children. These tumors are solitary. Surgical resection is successful in most of the patients, particulary if the tumor is localized and does not involve vital structures.
Cardiac sarcoma can involve any and all chambers of the heart, with the right atrium involved most frequently as was the case in our series. An intracavitary growth may cause right ventricular inflow and outflow obstruction or can progressively obliterate the right or left ventricle or both ventricles. Heart transplantation is a potential option in selected primary sarcomas of the heart but has not been necessary as of yet.
Depending on the location of sarcoma, a variety of manifestations may suggest cardiac involvement: congestive heart failure; second- or third-degree atrioventricular block; atrial tachycardia and transient ischemic attack.
Augmentation of the right atrium and, if necessary, the inferior or superior caval vein, with a pericardial patch may prevent narrowing of these structures at the time of resection. For this purpose we prefer to use untreated autologous pericardium because this is pliable and adjusts optimally to pressure changes in the right atrium or venae cavae. Surgery in primary malignancies is often palliative to relieve obstruction, and adjuvant radio- and/or chemotherapy has been reported to prolong survival but rarely provide cure as was the case in 3 patients from our series with malignant sarcomas [24].
In summary, we have shown a modern series of primary cardiac tumors treated surgically with the added option of minimally invasive approaches via a right small thoracotomy (Fig. 2). We have demonstrated that less invasive cardiac tumor resection is technically feasible to a large proportion of patients and can be applied with good functional and favorable cosmetic results to benign tumors. This minimally invasive approach has also been suggested by other institutions and is gaining in popularity [25].
References
McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Hartmann WH, Cowan WR. eds. Atlas of Tumor Pathology. Second Series 1978;Washington, DC: Armed Forces Institute of Pathology 20–25. Fascicle 15.
Prichard RW. Tumors of the heart. Arch Pathol 1951;51:98–128.
Chiari H. Myxom des rechten Vorhofs: plotzlicher Tod durch Geschwulstembolie der Lungenschlagader. Centralbl Allg Pathol Pathol Anat 1931;52:241–246.
Bahnson HT, Spencer FC, Andrus EC. Diagnosis and treatment of intracavitary myxomas of the heart. Ann Surg 1957;145:915–925.
Chitwood Jr WR. Clarence Crafoord and the first successful resection of a cardiac myxoma. Ann Thorac Surgery 1992;54:997–998.
Fabricius AM, Autschbach R, Lochhaas L, Brose S, Mohr FW. Primary left-atrial leiomyosarcoma. Thorac Cardiov Surg 2000;48:306–308.
Bossert T, Diegeler A, Spyrantis N, Mohr FW. Papillary fibroelastoma of the aortic valve with obturation of the left coronary ostium causing repeated drop attacks. J Heart Valve Disease 2000;9:842–843.
Bortolotti U, Maraglino G, Rubino M, Santini F, Mazzucco A, Milano A, Fasoli G, Livi U, Thiene G, Gallucci V. Surgical excision of intracardiac myxomas: a 20-year follow-up. Ann Thorac Surg 1990;49:449–453.
Bhan A, Mehrotra R, Choudhary SK, Sharma R, Prabhakar D, Airan B, Kumar AS, Venugopal P. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thorac Surg 1998;66:810–813.
Bjessmo S, Ivert T. Cardiac myxoma: 40 years' experience in 63 patients. Ann Thorac Surg 1997;63:697–700.
Dapper F, Grlach G, Hoffmann C, Fitz H, Marck P, Scheld HH. Primary cardiac tumors – clinical experiences and late results in 48 patients. Thorac Cardiovasc Surgeon 1988;36:80–85.
Shinfeld A, Katsumata T, Westaby S. Recurrent cardiac myxoma: seeding or multifocal disease Ann Thorac Surg 1998;66:285–288.
Reber D, Birnbaum DE. Recurrent cardiac myxoma: why it occurs. J Cardiovasc Surg 2001;42:345–348.
Basson CT, MacRae CA, Korf B, Merliss A. Genetic Heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol 1997;79:994–995.
Casey M, Vaughan CJ, He J, Hatcher CJ, Winter JM, Weremowicz S, Montgomery K, Kucherlapati R, Morton CC, Basson CT. Mutations in the protein kinase A R1alpha regulatory subunit cause familial cardiac myxomas and Carney complex. J Clin Invest 2000;106:R31–R38.
Shelh M. Multiplane transesophageal echocardiography detection of papillary fibroelastomas of the aortic valve causing a stroke. Eur Heart J 1997;18:702–703.
Pasteuning HW, Zijnen P, van der Aa MAC, Peters JH, van Geldorp TR. Papillary fibroelastoma of the aortic valve in a patient with an acute myocardial infarction. J Am Soc Echocardiogr 1996;9:897–900.
Butterworth JS, Poindexter CA. Papilloma of cusp of the aortic valve. Report of a patient with sudden death. Circulation 1973;48:213–215.
Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59:538–541.
Bedi HS, Sharma VK, Mishra M, Kasliwal RR, Trehan N. Papillary Fibroelastoma of the mitral valve associated with rheumatic mitral stenosis. Eur J Cardiothorac Surg 1995;9:54–55.
Lee CC, Celik C, Lajos TZ. Excision of papillary fibroelastoma arising from the septal leaflet of the tricuspid valve. J Card Surg 1995;10:589–591.
Gallo R, Kumar N, Prabhakar G, Awada A, Maalouf Y, Duran CMG. Papillary fibroelastoma of mitral valve chorda. Ann Thorac Surg 1993;55:1576–1577.
Espada R, Talwalker NG, Wilcox G, Kleiman NS, Verani MS. Visualization of ventricular fibroelastoma with a video-assisted thoracoscope. Ann Thorac Surg 1997;63:221–223.
Pessoto R, Sivestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol 1997;60:91–94.
Ko PJ, Chang CH, Lin PJ, Chu JJ, Tsai FC, Hsueh C, Yang MW. Video-assisted minimal access in excision of left atrial myxoma. Ann Thorac Surg 1998;66:1301–1305.(Torsten Bossert, Jan F. G)