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Surgical treatment of cardiac myxomas
J
THoRAc CARDIOVASC SURG
1988;96:800-5
Surgical treatment of cardiac myxomas Infonnation of 40 patients operated on for cardiac myxoma was reviewed. The age ranges were as follows: nine patients, 20 to 30 years; seven patients, 31 to 40 years; 10 patients, 41 to 50 years; and 11 patients, over 50 years. One patient each was 14 months, 14 years, and 16 years old. Left atrial myxoma was diagnosed in 31 patients, left ventricular myxoma in one, right atrial myxoma in five, and right ventricular myxoma in two. Multiple calcified right atrial and ventricular myxomas were detected in one patient. Detailed clinical characteristics and diagnostic methods are presented. The hospital mortality rate was 7.5 %. At 15 years' foUow-up practically aU of the patients had good late results. There were no recurrent myxomas. Rare left ventricular myxomas and multiple calcified right atrial and ventricular myxomas involving a tricuspid valve are also presented.
V. I. Burakovsky, MD, G. I. Zuckerman, MD, G. A. Kossatch, MD, M. A. Golossovskaya, MD, and L. A. Javorskaya, MD, Moscow, U.S.S.R.
Benign cardiac myxoma is a rare disease with an unusual malignant clinical course. According to Fauvre, Johns, and Jutzy,' cardiac myxoma is seen in 1: 10,000 autopsies, and in a vast number of patients it is diagnosed during life. More than 75% of the tumors are located in the left atrium and 17% to 25% in the right atrium.i' Myxoma is rarely seen in the right ventricle4, 5 or left ventricle/-" Tumor calcification occurs in 8% of patients. I The first successful operation done by Crafoord" in 1954 stimulated detailed investigation into this disease. Even though 30 years have elapsed since the first diagnosis made during life and the first successful surgical treatment, interest in this problem has not waned. At present, interest has been heightened by the use of clinical echocardiography, which allows this tumor to be diagnosed at the preclinical stage. Patients and methods During the past 20 years, cardiac myxoma was diagnosed in 44 patients, This article analyzes the data obtained from 40 patients operated on with cardiopulmonary bypass, Their age ranges were as follows: nine patients, 20 to 30 years; seven
From the Bakulev Institute of Cardio-vascular Surgery of the U,S,S,R, A,M,S" Moscow, U,S,S,R, (Director, Y, I. Burakovsky, MD, Academician of the U,S,S,R, A.M,S,), Received for publication Dec, 10, 1987, Accepted for publication May 10, 1988. Address for reprints: Y, 1. Burakovsky, MD, Bakulev Institute of Cardio-vascular Surgery of the U.S.S,R, A.M,S" Moscow, U,S,S,R,
800
Table I. Location of cardiac myxomas No. of patients
%
Left atrium Left ventricle Right atrium Right ventricle Multiple myxomas of right atrium and ventricle
31
77.5
I
2.5 12.5 5.0 2.5
Total
40
Location
5 2 1
patients, 31 to 40 years; 10 patients, 41 to 50 years; and II patients, over 50 years, One patient each was operated on at the age of 14 months, 14 years and 16 years. There were 26 women and 14 men, The group with myxoma of the left side of the heart comprised mostly women (25/32, 78,1%) and the group with myxoma of the right side, predominantly men (7/8, 87.5%) (Table 1), In 21 patients with left atrial myxoma the tumor had a long pedicle originating from a foramen ovale; in 10 patients the pedicle was wide and originated from various interseptal areas, Left ventricular myxomas were located under a mitral septal cusp and obliterated an outflow tract. Right atrial myxomas with a wide pedicle originated from a foramen ovale in three patients and from an interseptal area in two, The right ventricular myxomas were located at the tricuspid valve in one patient and originated from a papillary muscle of the septal cusp in the other. In the patient with multiple myxomas of the right atrium and ventricle, the tumors were located at a foramen ovale, in the right atrial wall, at the tricuspid valve cusps, and in subvalvular structures, In 10% of the patients the lesions were asymptomatic, often being found during prophylactic examination, and in 30% the symptoms were minimal. Sixty percent of the patients had
Volume 96 Number 5 November 1988
Cardiac myxomas 8 0 1
Fig. 1. Left atrial myxoma prolapsing into left ventricle.
Table m. Clinical symptoms in relation to tumor location
Table II. Functional class in relation to myxoma location LA or LV Functional class I II III
IV
RA or RV
LA or LV
RA or RV
No. of patients
%
No. of patients
%
Total (%)
3 11 7 11
9.4 34.4 21.8 34.4
I 1 3 3
12.5 12.5 37.5 37.5
10 30 25 35
LA. Left atrium; LV. left ventricle; RA. right atrium; RV. right ventricle.
severe symptoms of circulatory disorders. In such patients the clinical course was specific for quickly developing circulatory decompensation resistant to drug therapy. In both groups of patients the prevailing symptoms were specific for blood flow obstruction in the heart. Several patients with a left atrial myxoma had a specific symptomdifficulty in finding a comfortable position-that could be explained by the location of the tumor (Tables II and III). Auscultatory and phonocardiographic symptoms of left atrial myxoma included a diastolic murmur along the mitral valve plane with a flopping first tone. In five patients murmurs were absent. In three of the five patients with a right atrial myxoma a systolic murmur was heard along the left side of the sternum, in one a diastolic murmur was audible, and in the fifth patient no murmur was heard. In one patient with a right ventricular myxoma a systolic murmur was heard over the pulmonary valve and in the other, along the tricuspid area. Electrocardiographic changes were also specific for blood flow obstruction in the left and right atrioventricular orifices.
Symptoms Dyspnea Cardiac pain Palpitation Syncope Hepatomegaly Edema Hemoptysis CNS embolism Temperature response
No. of patients
%
4 2 3 I 4 3
50 25 46.3 12.5 50 37.5
2
25
No. of patients
%
26 19 9 10 13 15 2 4 5
81.1 59.4 28.1 31.2 40.6 46.6 6.2 12.6 15.6
RA. Right atrium; RV. right ventricle; LA. left atrium; LV, left ventricle; eNS, central nervous system.
Diffuse myocardial changes, left atrial and right ventricular myocardial hypertrophy, and stable sinus rhythm were specific electrocardiographic features in the patients with left atrial myxoma. X-ray examination showed normal pulmonary vasculature in the patients with right atrial or right ventricular myxoma. In most patients with left atrial myxoma venous congestion prevailed. Cardiomegaly was not evident. Tumor calcification was diagnosed in five patients with right atrial or ventricular myxoma (62.5%) and in only two patients with left atrial or ventricular myxoma (6,2%). Cardiac catheterization was performed in four patients with right atrial or ventricular myxoma and in six with left atrial or
802
Burakovsky et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Calcified right atrial myxoma not prolapsing into right ventricle (RV). LV. Left ventricle; tv, tricuspid valve; T, tumor.
Fig. 3. Left ventricular myxoma located under aortic valve. LV. Left ventricle; RV, right ventricle; Ao, aorta; LA, left atrium.
ventricular myxoma. In six patients the correct diagnosis was established, in two the diagnosis was doubtful, and in two mitral stenosis was misdiagnosed. Pulmonary pressure was within 45 to 115 mm Hg in all patients. Echocardiography is the most valuable diagnostic tool used in patients with cardiac myxomas. Echocardiograms performed in 32 patients helped to make the exact diagnosis and
to define the size and location of the myxoma, the status of the valvular apparatus, and the contractile function of the myocardium (Figs. I to 3). Thirty-three operations were done with hypothermic cardiopulmonary bypass and pharmacologic myocardial protection and seven with normothermic cardiopulmonary bypass. The approach was through a median sternotomy. In the patients
Volume 96 Number 5 November 1988
Cardiac myxomas
jl:I/lllil"IlIHIJUllff'l'
I c,"-._
f
80 3
IIIII1
~~.• 30 4/84;
_
Fig. 4. Villose myxoma. 2.5 by 2 by 1.5 em, originating from left ventricular septum.
, tfAl·iz Fig. 5. Calcified right ventricular myxoma originating from papillary muscles and prolapsing into pulmonary artery.
with left atrial myxoma the left atrium was opened behind the interatrial groove. The left ventricular myxoma (Fig. 4) was excised through a transverse incision in the ascending aorta, since it was located in the left ventricular outflow tract under the aortic valve. In the patient with left atrial myxoma associated with severe mitral regurgitation, annuloplasty was successfully performed.
Right atrial myxomas were excised through the right atrium. In the patient with the calcified right ventricular myxoma originating from the papillary muscle and involving a tricuspid septal cusp, the tumor was excised with the valve, which was replaced with a semilunar valve inserted into the tricuspid position (Fig. 5). In the patient with multiple right atrial and ventricular myxomas. two of which originated from
8 04
Burakovsky et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 6. Multiple calcified myxomas of right atrium and ventricle and tricuspid valve.
papillary muscles and tricuspid valve cusps, the tumors were perforated with calcium at several sites. Because valve function had been disturbed, the valve was replaced with a semilunar prosthesis (Fig. 6). Myxomas with a wide pedicle were excised and the site was treated with iodine or a coagulant. If the tumor had a long pedicle and was well exposed, it usually was excised with a part of the septum and the septum was subsequently sutured. An ovoid dense myxoma was excised in 17 (42.5%) patients, a lobar one in eight (20.2%), and a villose one in 15 (37.5%) patients. In most patients myxomas on the right side of the heart were more dense than those on the left. The hospital mortality rate was 7.5% (three patients). Two patients with a myxoma on the right side died of embolization into a pulmonary artery branch, and one with a left atrial myxoma died of complicated mediastinitis. A review of the late results in all patients with left atrial or ventricular myxoma revealed good surgical results in all but one, who had mitral regurgitation and prolapse that had not been corrected intraoperatively. Good surgical results were achieved in all patients with a right atrial or ventricular myxoma. No recurrences developed during the I 5-year followup period in any patient with a pedicled tumor excised either with (21 patients) or without (16 patients) underlying tissue.
Discussion Cardiac myxoma is a rare disease. Clinical signs appear in the advanced stage of the disease, when the tumor has grown large. These signs are specific for circulatory disorders caused by obstruction of the inflow or outflow tract. Before the era of echocardiography, rheumatic mitral or tricuspid disease was a common
misdiagnosis."!' About 50% of patients examined before the era of
echocardiography were sent to the consultation department of the institute with a diagnosis of mitral or mitral-tricuspid disease. Angiocardiography" is reputed to be the most reliable diagnostic tool," but our experience showed that it was not always accurate. Among 10 examined patients, the diagnosis was correct in six patients, doubtful in two, and erroneous in two. Echocardiographic examination is practically inerrant. The two-dimensional echocardiographic method is highly effective, since it not only provides an exact diagnosis, but also pinpoints the exact location of the tumor and evaluates the valvular status and contractile function. We believe that, when the exact diagnosis has been established, urgent operation is indicated because of the constant danger of sudden death from thromboembolism or obstruction of the inflow or outflow tract by the tumor. A transsternal approach is optimal in patients with a cardiac tumor. It provides access for urgent physiologic operations done with pharmacologic cold cardioplegia, which is a prerequisite for total correction. This method has been successfully used since 1976. As our experience has shown, a left ventricular myxoma can be excised through a transaortic approach. If the valvular apparatus has been destroyed, it must be replaced; otherwise, the sustained regurgitation will impair late surgical results, as in one of our patients. In two patients with a calcified right atrial or ventricular myxoma, the tricuspid valve and subvalvular structures were extensively damaged. The valve was
Volume 96 Number 5 November 1988
incompetent and had to be replaced. Similar changes in the mitral valve were described by Nasser and associates." However, we have not found a report of tricuspid valve replacement in the available medical literature. The causes of multiple cardiac myxomas have been poorly studied. The medical literature contains descriptions of multicentric development of right ventricular myxomas" and of myxomas located in the left and right atria, I 5 but we have not found any description of multiple calcified right atrial or ventricular myxomas involving valvular structures. Cardiac myxoma is a benign tumor, but instances of invasive growth, recurrent development and malignancy, and of "benign" metastases have been published.r" 16-17 We may assume that, in our patients with multiple calcified myxomas of the right atrium or ventricle, a myxoma originating from the foramen ovale is a primary tumor. Its mobility may contribute to its becoming loosened from other structures, with subsequent implanation and local growth. Recurrent tumors and multicentric growth cast doubt on the "benign" nature of cardiac myxomas and dictate total excision of the primary tumor and of implants. Hypothermic heart-lung bypass and pharmacologic cold cardioplegia are prerequisites for radical excision. Excision of a pedicle together with the endocardium or interatrial septum is an optimal surgical method that prevents tumor recurrence.
Cardiac myxomas
REFERENCES I. Fauvre FG, Johns VJ Jr, Jutzy RV. Cardiac myxomas:
2. 3. 4. 5.
6.
7. 8.
9.
10.
11.
Conclusions 1. Cardiac myxoma is a rare benign cardiac tumor with an unusual malignant clinical course. 2. Echocardiography is a decisive diagnostic tool that pinpoints the exact location of the tumor and allows valvular and myocardial status to be evaluated. 3. When the exact diagnosis has been established, urgent operation is mandatory because of the constant danger of sudden death. 4. The "benign" nature of cardiac myxoma is doubtful. Surgical excision of the tumor is the only highly effective method of treatment, because total correction is mandatory. 5. In patients in whom cardiac myxoma is associated with destructive changes in the valvular apparatus and function, with consequent regurgitation, replacement of the valve is indicated. In patients with valve prolapse and regurgitation, it is wise to perform a reconstructive operation aimed at valve preservation.
805
12.
13.
14.
15.
16. 17.
report of eight cases with successful excision. West J Med 1977;127:284-91. Golossowskaya MA. Cardiac myxomas. Arkh Patol 1981;12:29-35. Greenwood WF. Profile of atrial myxoma. Am J Cardiol 1968;21:367-75. Heath D. Pathology of cardiac tumors. Am J Cardiol 1968;21:315-27. Martin CE, Hufnagel CA, de Leon AC Jr. Calcified atrial myxoma: diagnostic significance of the "systolic tumor sound" in a case presenting as tricuspid insufficiency. Am Heart J 1969;78:245-50. Thomas G1, Edmark KW, Jones TW, Eyer KM. Myxoma of the left ventricle: a case report. J THoRAc CARDlOVASC SCRG 1963;46:220-6. Dimitrov D, Dragoitchev T, Daskamov E, Georgiev T. Cardiac tumours. Khirurgia (Bulgaria) 1981;1:3-9. Crafoord CL. Discussion of Glover RP. Technique of mitral commissurotomy. In: Lam CR, ed. Proceedings of the Henry Ford Hospital International Symposium on Cardiovascular Surgery. Philadelphia: WB Saunders, 1955:202. Nasser WK, Davis RH, Dillon JC, et al. Atrial myxoma. I. Clinical and pathologic features in nine cases. Am Heart J 1972;83:694-704. Selzer A, Sakai FJ, Popper RW. Protean clinical manifestations of primary tumors of the heart. Am J Med 1972;52:9-18. Sung RJ, Ghahramany AR, Mallon SM, et al. Hemodynamic features of prolapsing and nonprolapsing left atrial myxoma. Circulation 1975;51:342-9. Goldberg HP, Glenn F, Dotter CT, Steinberg I. Myxoma of left atrium: diagnosis made during life with operative and postmortem findings. Circulation 1952;6:762-7. Bukharin VA, Petrosyan JS, Podzolkov VP, Ivanitsky AV, Golossovskaya MA. The right ventricular myxoma. Grudn Khir 1974;4:31-5. Kishimoto M, Sakaibori S, cited by Sakakibara S, Osawa M, Konno S, et al. Myxoma of the right ventricle of the heart. Am Heart J 1965;69:382-91. Zajtchuk R, Fitterer JD, Strevey TE, Nelson WP. Bilateral atrial myxomas: preoperative diagnosis and successful removal. J THoRAc CARDIOVASC SURG 1975;69:291-4. Castaneda AR, Varco RL. Tumors of the heart: surgical considerations. Am J Cardiol 1968;21:357-62. Reid RC, White HJ, Murphy ML, Williams D, Sun CN, Flanagan WHo The malignant potentiality of left atrial myxoma. J THoRAc CARDIOVASC SURG 1974;68:857-68.
THoRAc CARDIOVASC SURG
1988;96:800-5
Surgical treatment of cardiac myxomas Infonnation of 40 patients operated on for cardiac myxoma was reviewed. The age ranges were as follows: nine patients, 20 to 30 years; seven patients, 31 to 40 years; 10 patients, 41 to 50 years; and 11 patients, over 50 years. One patient each was 14 months, 14 years, and 16 years old. Left atrial myxoma was diagnosed in 31 patients, left ventricular myxoma in one, right atrial myxoma in five, and right ventricular myxoma in two. Multiple calcified right atrial and ventricular myxomas were detected in one patient. Detailed clinical characteristics and diagnostic methods are presented. The hospital mortality rate was 7.5 %. At 15 years' foUow-up practically aU of the patients had good late results. There were no recurrent myxomas. Rare left ventricular myxomas and multiple calcified right atrial and ventricular myxomas involving a tricuspid valve are also presented.
V. I. Burakovsky, MD, G. I. Zuckerman, MD, G. A. Kossatch, MD, M. A. Golossovskaya, MD, and L. A. Javorskaya, MD, Moscow, U.S.S.R.
Benign cardiac myxoma is a rare disease with an unusual malignant clinical course. According to Fauvre, Johns, and Jutzy,' cardiac myxoma is seen in 1: 10,000 autopsies, and in a vast number of patients it is diagnosed during life. More than 75% of the tumors are located in the left atrium and 17% to 25% in the right atrium.i' Myxoma is rarely seen in the right ventricle4, 5 or left ventricle/-" Tumor calcification occurs in 8% of patients. I The first successful operation done by Crafoord" in 1954 stimulated detailed investigation into this disease. Even though 30 years have elapsed since the first diagnosis made during life and the first successful surgical treatment, interest in this problem has not waned. At present, interest has been heightened by the use of clinical echocardiography, which allows this tumor to be diagnosed at the preclinical stage. Patients and methods During the past 20 years, cardiac myxoma was diagnosed in 44 patients, This article analyzes the data obtained from 40 patients operated on with cardiopulmonary bypass, Their age ranges were as follows: nine patients, 20 to 30 years; seven
From the Bakulev Institute of Cardio-vascular Surgery of the U,S,S,R, A,M,S" Moscow, U,S,S,R, (Director, Y, I. Burakovsky, MD, Academician of the U,S,S,R, A.M,S,), Received for publication Dec, 10, 1987, Accepted for publication May 10, 1988. Address for reprints: Y, 1. Burakovsky, MD, Bakulev Institute of Cardio-vascular Surgery of the U.S.S,R, A.M,S" Moscow, U,S,S,R,
800
Table I. Location of cardiac myxomas No. of patients
%
Left atrium Left ventricle Right atrium Right ventricle Multiple myxomas of right atrium and ventricle
31
77.5
I
2.5 12.5 5.0 2.5
Total
40
Location
5 2 1
patients, 31 to 40 years; 10 patients, 41 to 50 years; and II patients, over 50 years, One patient each was operated on at the age of 14 months, 14 years and 16 years. There were 26 women and 14 men, The group with myxoma of the left side of the heart comprised mostly women (25/32, 78,1%) and the group with myxoma of the right side, predominantly men (7/8, 87.5%) (Table 1), In 21 patients with left atrial myxoma the tumor had a long pedicle originating from a foramen ovale; in 10 patients the pedicle was wide and originated from various interseptal areas, Left ventricular myxomas were located under a mitral septal cusp and obliterated an outflow tract. Right atrial myxomas with a wide pedicle originated from a foramen ovale in three patients and from an interseptal area in two, The right ventricular myxomas were located at the tricuspid valve in one patient and originated from a papillary muscle of the septal cusp in the other. In the patient with multiple myxomas of the right atrium and ventricle, the tumors were located at a foramen ovale, in the right atrial wall, at the tricuspid valve cusps, and in subvalvular structures, In 10% of the patients the lesions were asymptomatic, often being found during prophylactic examination, and in 30% the symptoms were minimal. Sixty percent of the patients had
Volume 96 Number 5 November 1988
Cardiac myxomas 8 0 1
Fig. 1. Left atrial myxoma prolapsing into left ventricle.
Table m. Clinical symptoms in relation to tumor location
Table II. Functional class in relation to myxoma location LA or LV Functional class I II III
IV
RA or RV
LA or LV
RA or RV
No. of patients
%
No. of patients
%
Total (%)
3 11 7 11
9.4 34.4 21.8 34.4
I 1 3 3
12.5 12.5 37.5 37.5
10 30 25 35
LA. Left atrium; LV. left ventricle; RA. right atrium; RV. right ventricle.
severe symptoms of circulatory disorders. In such patients the clinical course was specific for quickly developing circulatory decompensation resistant to drug therapy. In both groups of patients the prevailing symptoms were specific for blood flow obstruction in the heart. Several patients with a left atrial myxoma had a specific symptomdifficulty in finding a comfortable position-that could be explained by the location of the tumor (Tables II and III). Auscultatory and phonocardiographic symptoms of left atrial myxoma included a diastolic murmur along the mitral valve plane with a flopping first tone. In five patients murmurs were absent. In three of the five patients with a right atrial myxoma a systolic murmur was heard along the left side of the sternum, in one a diastolic murmur was audible, and in the fifth patient no murmur was heard. In one patient with a right ventricular myxoma a systolic murmur was heard over the pulmonary valve and in the other, along the tricuspid area. Electrocardiographic changes were also specific for blood flow obstruction in the left and right atrioventricular orifices.
Symptoms Dyspnea Cardiac pain Palpitation Syncope Hepatomegaly Edema Hemoptysis CNS embolism Temperature response
No. of patients
%
4 2 3 I 4 3
50 25 46.3 12.5 50 37.5
2
25
No. of patients
%
26 19 9 10 13 15 2 4 5
81.1 59.4 28.1 31.2 40.6 46.6 6.2 12.6 15.6
RA. Right atrium; RV. right ventricle; LA. left atrium; LV, left ventricle; eNS, central nervous system.
Diffuse myocardial changes, left atrial and right ventricular myocardial hypertrophy, and stable sinus rhythm were specific electrocardiographic features in the patients with left atrial myxoma. X-ray examination showed normal pulmonary vasculature in the patients with right atrial or right ventricular myxoma. In most patients with left atrial myxoma venous congestion prevailed. Cardiomegaly was not evident. Tumor calcification was diagnosed in five patients with right atrial or ventricular myxoma (62.5%) and in only two patients with left atrial or ventricular myxoma (6,2%). Cardiac catheterization was performed in four patients with right atrial or ventricular myxoma and in six with left atrial or
802
Burakovsky et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Calcified right atrial myxoma not prolapsing into right ventricle (RV). LV. Left ventricle; tv, tricuspid valve; T, tumor.
Fig. 3. Left ventricular myxoma located under aortic valve. LV. Left ventricle; RV, right ventricle; Ao, aorta; LA, left atrium.
ventricular myxoma. In six patients the correct diagnosis was established, in two the diagnosis was doubtful, and in two mitral stenosis was misdiagnosed. Pulmonary pressure was within 45 to 115 mm Hg in all patients. Echocardiography is the most valuable diagnostic tool used in patients with cardiac myxomas. Echocardiograms performed in 32 patients helped to make the exact diagnosis and
to define the size and location of the myxoma, the status of the valvular apparatus, and the contractile function of the myocardium (Figs. I to 3). Thirty-three operations were done with hypothermic cardiopulmonary bypass and pharmacologic myocardial protection and seven with normothermic cardiopulmonary bypass. The approach was through a median sternotomy. In the patients
Volume 96 Number 5 November 1988
Cardiac myxomas
jl:I/lllil"IlIHIJUllff'l'
I c,"-._
f
80 3
IIIII1
~~.• 30 4/84;
_
Fig. 4. Villose myxoma. 2.5 by 2 by 1.5 em, originating from left ventricular septum.
, tfAl·iz Fig. 5. Calcified right ventricular myxoma originating from papillary muscles and prolapsing into pulmonary artery.
with left atrial myxoma the left atrium was opened behind the interatrial groove. The left ventricular myxoma (Fig. 4) was excised through a transverse incision in the ascending aorta, since it was located in the left ventricular outflow tract under the aortic valve. In the patient with left atrial myxoma associated with severe mitral regurgitation, annuloplasty was successfully performed.
Right atrial myxomas were excised through the right atrium. In the patient with the calcified right ventricular myxoma originating from the papillary muscle and involving a tricuspid septal cusp, the tumor was excised with the valve, which was replaced with a semilunar valve inserted into the tricuspid position (Fig. 5). In the patient with multiple right atrial and ventricular myxomas. two of which originated from
8 04
Burakovsky et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 6. Multiple calcified myxomas of right atrium and ventricle and tricuspid valve.
papillary muscles and tricuspid valve cusps, the tumors were perforated with calcium at several sites. Because valve function had been disturbed, the valve was replaced with a semilunar prosthesis (Fig. 6). Myxomas with a wide pedicle were excised and the site was treated with iodine or a coagulant. If the tumor had a long pedicle and was well exposed, it usually was excised with a part of the septum and the septum was subsequently sutured. An ovoid dense myxoma was excised in 17 (42.5%) patients, a lobar one in eight (20.2%), and a villose one in 15 (37.5%) patients. In most patients myxomas on the right side of the heart were more dense than those on the left. The hospital mortality rate was 7.5% (three patients). Two patients with a myxoma on the right side died of embolization into a pulmonary artery branch, and one with a left atrial myxoma died of complicated mediastinitis. A review of the late results in all patients with left atrial or ventricular myxoma revealed good surgical results in all but one, who had mitral regurgitation and prolapse that had not been corrected intraoperatively. Good surgical results were achieved in all patients with a right atrial or ventricular myxoma. No recurrences developed during the I 5-year followup period in any patient with a pedicled tumor excised either with (21 patients) or without (16 patients) underlying tissue.
Discussion Cardiac myxoma is a rare disease. Clinical signs appear in the advanced stage of the disease, when the tumor has grown large. These signs are specific for circulatory disorders caused by obstruction of the inflow or outflow tract. Before the era of echocardiography, rheumatic mitral or tricuspid disease was a common
misdiagnosis."!' About 50% of patients examined before the era of
echocardiography were sent to the consultation department of the institute with a diagnosis of mitral or mitral-tricuspid disease. Angiocardiography" is reputed to be the most reliable diagnostic tool," but our experience showed that it was not always accurate. Among 10 examined patients, the diagnosis was correct in six patients, doubtful in two, and erroneous in two. Echocardiographic examination is practically inerrant. The two-dimensional echocardiographic method is highly effective, since it not only provides an exact diagnosis, but also pinpoints the exact location of the tumor and evaluates the valvular status and contractile function. We believe that, when the exact diagnosis has been established, urgent operation is indicated because of the constant danger of sudden death from thromboembolism or obstruction of the inflow or outflow tract by the tumor. A transsternal approach is optimal in patients with a cardiac tumor. It provides access for urgent physiologic operations done with pharmacologic cold cardioplegia, which is a prerequisite for total correction. This method has been successfully used since 1976. As our experience has shown, a left ventricular myxoma can be excised through a transaortic approach. If the valvular apparatus has been destroyed, it must be replaced; otherwise, the sustained regurgitation will impair late surgical results, as in one of our patients. In two patients with a calcified right atrial or ventricular myxoma, the tricuspid valve and subvalvular structures were extensively damaged. The valve was
Volume 96 Number 5 November 1988
incompetent and had to be replaced. Similar changes in the mitral valve were described by Nasser and associates." However, we have not found a report of tricuspid valve replacement in the available medical literature. The causes of multiple cardiac myxomas have been poorly studied. The medical literature contains descriptions of multicentric development of right ventricular myxomas" and of myxomas located in the left and right atria, I 5 but we have not found any description of multiple calcified right atrial or ventricular myxomas involving valvular structures. Cardiac myxoma is a benign tumor, but instances of invasive growth, recurrent development and malignancy, and of "benign" metastases have been published.r" 16-17 We may assume that, in our patients with multiple calcified myxomas of the right atrium or ventricle, a myxoma originating from the foramen ovale is a primary tumor. Its mobility may contribute to its becoming loosened from other structures, with subsequent implanation and local growth. Recurrent tumors and multicentric growth cast doubt on the "benign" nature of cardiac myxomas and dictate total excision of the primary tumor and of implants. Hypothermic heart-lung bypass and pharmacologic cold cardioplegia are prerequisites for radical excision. Excision of a pedicle together with the endocardium or interatrial septum is an optimal surgical method that prevents tumor recurrence.
Cardiac myxomas
REFERENCES I. Fauvre FG, Johns VJ Jr, Jutzy RV. Cardiac myxomas:
2. 3. 4. 5.
6.
7. 8.
9.
10.
11.
Conclusions 1. Cardiac myxoma is a rare benign cardiac tumor with an unusual malignant clinical course. 2. Echocardiography is a decisive diagnostic tool that pinpoints the exact location of the tumor and allows valvular and myocardial status to be evaluated. 3. When the exact diagnosis has been established, urgent operation is mandatory because of the constant danger of sudden death. 4. The "benign" nature of cardiac myxoma is doubtful. Surgical excision of the tumor is the only highly effective method of treatment, because total correction is mandatory. 5. In patients in whom cardiac myxoma is associated with destructive changes in the valvular apparatus and function, with consequent regurgitation, replacement of the valve is indicated. In patients with valve prolapse and regurgitation, it is wise to perform a reconstructive operation aimed at valve preservation.
805
12.
13.
14.
15.
16. 17.
report of eight cases with successful excision. West J Med 1977;127:284-91. Golossowskaya MA. Cardiac myxomas. Arkh Patol 1981;12:29-35. Greenwood WF. Profile of atrial myxoma. Am J Cardiol 1968;21:367-75. Heath D. Pathology of cardiac tumors. Am J Cardiol 1968;21:315-27. Martin CE, Hufnagel CA, de Leon AC Jr. Calcified atrial myxoma: diagnostic significance of the "systolic tumor sound" in a case presenting as tricuspid insufficiency. Am Heart J 1969;78:245-50. Thomas G1, Edmark KW, Jones TW, Eyer KM. Myxoma of the left ventricle: a case report. J THoRAc CARDlOVASC SCRG 1963;46:220-6. Dimitrov D, Dragoitchev T, Daskamov E, Georgiev T. Cardiac tumours. Khirurgia (Bulgaria) 1981;1:3-9. Crafoord CL. Discussion of Glover RP. Technique of mitral commissurotomy. In: Lam CR, ed. Proceedings of the Henry Ford Hospital International Symposium on Cardiovascular Surgery. Philadelphia: WB Saunders, 1955:202. Nasser WK, Davis RH, Dillon JC, et al. Atrial myxoma. I. Clinical and pathologic features in nine cases. Am Heart J 1972;83:694-704. Selzer A, Sakai FJ, Popper RW. Protean clinical manifestations of primary tumors of the heart. Am J Med 1972;52:9-18. Sung RJ, Ghahramany AR, Mallon SM, et al. Hemodynamic features of prolapsing and nonprolapsing left atrial myxoma. Circulation 1975;51:342-9. Goldberg HP, Glenn F, Dotter CT, Steinberg I. Myxoma of left atrium: diagnosis made during life with operative and postmortem findings. Circulation 1952;6:762-7. Bukharin VA, Petrosyan JS, Podzolkov VP, Ivanitsky AV, Golossovskaya MA. The right ventricular myxoma. Grudn Khir 1974;4:31-5. Kishimoto M, Sakaibori S, cited by Sakakibara S, Osawa M, Konno S, et al. Myxoma of the right ventricle of the heart. Am Heart J 1965;69:382-91. Zajtchuk R, Fitterer JD, Strevey TE, Nelson WP. Bilateral atrial myxomas: preoperative diagnosis and successful removal. J THoRAc CARDIOVASC SURG 1975;69:291-4. Castaneda AR, Varco RL. Tumors of the heart: surgical considerations. Am J Cardiol 1968;21:357-62. Reid RC, White HJ, Murphy ML, Williams D, Sun CN, Flanagan WHo The malignant potentiality of left atrial myxoma. J THoRAc CARDIOVASC SURG 1974;68:857-68.