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ORIGINAL ARTICLE
Year : 2012  |  Volume : 1  |  Issue : 2  |  Page : 69-73

A frequently found rare tumor at high altitude: Atrial Myxoma; Kashmir experience


Department of Cardiovascular and Thoracic Surgery, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India

Date of Web Publication13-Sep-2012

Correspondence Address:
Mohd Lateef Wani
Senior Resident CVTS, SKIMS, Soura Srinagar 190010, Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0521.100947

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  Abstract 

Background: Myxomas are the most common type of cardiac tumors in all age groups, and symptoms are produced by mechanical interference with cardiac function or embolization. This study comprises the clinical profile, surgical approach, morbidity, mortality and recurrence rate of atrial myxomas in the Kashmir valley. Patients and Methods: The study was retrospective from January 1991 to July 2005 and prospective from August 2005 to December 2007. All patients admitted with the diagnosis of atrial myxomas were included in the study. Results: The study included 30 patients with a mean age of 52 ± 4.8 years. Females outnumbered males, with a ratio of 2.9:1. Dyspnea was the most common symptom on presentation, with majority of the patients belonging to New York Heart Association Class III (56.6%). Pre-operative diagnosis was established in all patients by echocardiography. Majority of myxomas, i.e. 22 (88%), arose from interatrial septum. Wide excision of tumor with polytetrafluorethylene (PTFE)/pericardial patch closure was the most common surgical procedure performed. Both early and late mortalities were 6.6%. In addition to clinical examination, periodic echocardiography was done on follow-up to detect recurrence, which was seen in one (3.3%) patient. Conclusions: Surgical management is curative with minimal early and late morbidity and mortality. Extensive resection of the myxoma attached to the atrial septum or atrial wall can reduce the likelihood of recurrence to a great extent, and long-term clinical and echocardiographic follow-up is mandatory.

Keywords: Atrial myxoma, cardiac tumor, pericardial patch


How to cite this article:
Lone RA, Ahangar AG, Wani ML, Dar AM, Bhat MA, Singh S, Hussain Z, Nu. A frequently found rare tumor at high altitude: Atrial Myxoma; Kashmir experience. Saudi J Health Sci 2012;1:69-73

How to cite this URL:
Lone RA, Ahangar AG, Wani ML, Dar AM, Bhat MA, Singh S, Hussain Z, Nu. A frequently found rare tumor at high altitude: Atrial Myxoma; Kashmir experience. Saudi J Health Sci [serial online] 2012 [cited 2020 Jul 7];1:69-73. Available from: http://www.saudijhealthsci.org/text.asp?2012/1/2/69/100947


  Introduction Top


Despite De-Senac's assertion in 1783 that "The heart is an organ too noble to be attacked by a primary tumor," the heart has no specific immunity from neoplasia, and cardiac tumors are being increasingly recognized. Myxomas are the most common type of cardiac tumors in all age groups, accounting for one-third to one-half of cases at postmortum and for about three-quarter of tumors treated surgically. [1] Most atrial myxomas arise from the atrial septum, usually from the region of the limbus of fossa ovalis. Symptoms are produced by mechanical interference with cardiac function or embolization. Laboratory studies are non-specific and non-diagnostic. Although transesophageal echocardiography is more sensitive, 2-dimensional echocardiography is usually adequate for diagnosis. Drug therapy is used only for complications such as congestive heart failure or cardiac arrhythmias. Surgical resection of the myxoma is the treatment of choice, with low morbidity and mortality. Recurrence is usually attributable to incomplete excision of tumor, growth from second focus or intracardiac implantation from primary tumor. As of now, no study on atrial myxoma has been published from the Kashmir Valley, although we frequently come across these patients; therefore, we conducted this study. The purpose of this study was:

  1. To study clinical profile of atrial myxoma.
  2. To study surgical approach, morbidity, mortality and recurrence rate following surgery at our institute over a period of 17 years.

  Patients and Methods Top


The study was a retrospective study was from January 1991 to July 2005 and prospective study from August 2005 to December 2007.

Case histories in the retrospective group were reviewed from the Department of Medical Records. Relevant information and follow-up of the patients was carried out by examining the relevant clinical notes available, by telephonic interview and personal contact wherever possible. Patients in the prospective group were subjected to detailed history, examination and investigations as per the proforma. Pre-operative diagnosis was established in all patients by echocardiography. Other investigations were carried out wherever indicated. All patients were subjected to surgery under cardiopulmonary bypass. The approach for left atrial myxomas was right atrial trans-septal, right atrial for right atrial myxomas and a biatrial approach in some patients. Care was taken not to manipulate the tumor before the aorta was cross-clamped. Complete excision of the tumor with a cuff of interatrial septum was the basic principle of excision. All four chambers were thoroughly explored for additional myxomas. Copious irrigation of the atria and ventricle with saline was done to eliminate any loose tumor fragments. The surgically created defect was repaired with a PTFE/pericardial patch. All the tumors were subjected to routine histopathological examination. Intraoperative complications, morbidity and mortality was recorded. Follow-up on the outpatient basis at regular intervals was carried out. Patients were subjected to clinical examination and echocardiography and were screened for recurrence. All the data was compiled and analyzed statistically.


  Results Top


The study included 30 patients, with 26 from retrospective and four from the prospective group, respectively, over 17 years. Majority of the cases were in the 5 th and 6 th decades of life, with a mean age of 52 ± 4.8 years. The youngest patient in our series was of the age of 17 years, and the oldest was 75 years old. There were 22 (73.2%) females and eight (26.6%) males, with a female to male ratio of 2.9:1. None of patients had a family history of the atrial myxoma. Patients presented with varied symptoms [Table 1] and physical findings on admission [Table 2]. Symptomatically, patients were graded according to the New York Heart Association (NYHA) classification for pre-operative functional status. Majority of the patients belonged to NYHA Class III (56.6%), followed by NYHA Class II (36.6%). X-ray chest and electrocardiograph (ECG) aided the diagnosis. Abnormal chest X-ray and ECG was seen in case of 16 (53.3%) and 14 (46.7%) patients, respectively. Pre-operative diagnosis was established in all patients by echocardiography (transthoracic). Two patients were received with magnetic resonance imaging (MRI) diagnosis and one with computed tomography (CT) diagnosis. Biatrial atriotomy was employed for resection of tumor in seven (23.3%), right atriotomy in 19 (63.3%) and left atriotomy in four (13.3%) patients. Distribution of atrial myxomas is shown in [Table 3].
Table 1: Initial clinical presentation of the patients

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Table 2: Physical findings on examination

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Table 3: Site of tumor (n = 30)

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Majority of our patients (93.3%) had tumor size of 3-10 cm. Wide excision of tumor with adjacent septal wall with PTFE/pericardial patch closure was the most common surgical procedure performed in 26 (87%) patients, followed by excision of tumor with surrounding myocardium with patch closure in four (13%) patients. Two patients were subjected to mitral valve repair in addition to excision of tumor. Wound infection and pneumonitis were the most common post-operative complications: one patient developed coagulopathy and another developed septicemia.

Early (<30 days post-operative) and late deaths (>30 days post-operative) were two (6.6%) in each group. All patients were followed on an Out Patient Department basis. Follow-up of patients ranged from 6 months to 15 years, with a mean duration of 5.7 years. In addition to clinical examination, periodic Echo was done to detect recurrence, which was seen in one (3.3%) patient.


  Discussion Top


Majority of the cases were in the 5 th and 6 th decades of life with a mean age of 52 ± 4.8 years. Majority of the patients belonged to NYHA Class III (56.6%), followed by NYHA Class II (36.6%). Biatrial atriotomy was employed for resection of tumor in seven (23.3%), right atriotomy in 19 (63.3%) and left atriotomy in four (13.3%) patients. Wound infection and pneumonitis were the most common post-operative complications: one patient developed coagulopathy and another septicemia.

The advent of modern diagnostic procedures and the cardiac operation has transformed primary cardiac neoplasms from a condition rarely diagnosed before autopsy [2] to a potentially curable form of heart disease. Thus, early diagnosis of cardiac tumors becomes important.

Of 30 patients, 22 (73.2%) were females, which is almost in accordance with other studies that suggest a 70-75% female preponderance. In a series of 24 patients conducted by Bulky et al., (1979) [3] the female sex incidence was 71%. The average age of our patients was 52 ± 4.8 years. In the study conducted by Pinede et al. [4] on 112 consecutive cases of left atrial myxomas, the median age was 53 years, ranging between 23 and 84 years. Acebo et al. [5] analyzed 37 cardiac myxoma cases, and the age of patients at diagnosis ranged from 12 to 73 years (mean 52 ± 15 years).

There are no pathogonamic symptoms and signs that suggest the presence of myxoma. [6] Dyspnea was the most frequent symptom at diagnosis in 60% patients in our study, which is slightly higher than 54% seen in the study by Aceb et al.[5] The higher incidence in our patients can be explained by the fact that most of our patients had a late presentation, which can be attributed to a number of factors like less education, lack of health consciousness and poor socio-economy and large size of tumors. Both angina and syncope were present in 13.3% of the patients in our study. This is more or less in compliance with the study by Elvira Acebo, [5] where he observed it as 14%. However, palpitations as a presenting symptom was present in 46% of our patients, which was higher as compared with the above study (35%). Bortolotti et al. [7] showed an almost similar incidence of this symptom in his study (44%). Arterial embolization occurred in five patients (16.6%), and 12 patients (39.6%) had symptoms of congestive cardiac failure (CCF)at the time of presentation. Constitutional symptoms like weight loss, fatigue and fever were present in 10%, 6.6% and 3.3% of the patients, respectively. One patient also presented with neurological symptoms. Arterial embolization and constitutional symptoms were more or less in accordance with the study by Centofanti et al. (1999), [8] where it was 23% and 13%, respectively. Bulkley and Hutchins (1979) [9] , in their study of 24 patients with atrial myxomas, reported CCF as the presenting feature in 54% patients, which is slightly higher than our study. However, Pinede, [4] in his series of 112 cases of left atrial myxomas, reported CCF in 43% of the patients, which complies with our study.

Of the 30 patients with atrial myxoma, 26 (86.6%) had abnormal auscultatory findings; the most common alteration was a systolic murmur, 12 (46.1%), followed by loud first heart sound in 10 (38.4%) and tumor plop in five (19.2%). All these auscultatory findings were in agreement with the study conducted by Alvera Acebo, [5] who, in his series of 34 patients, reported an abnormal auscultatory finding in 85%, with systolic murmur in 50%, a loud first heart sound in 32% and a tumor plop in 15% patients. Pinede et al. [4] also reported a tumor plop in 15% patients in their study.

The most frequent laboratory finding was raised erythrocyte sedimentation rate (ESR) (Westergren method >20 mm/h) found in nine patients (30%), followed by anemia (Hb < 12 gm/dL in men and <10 gm/dL in women), which was seen in seven patients (23.3%). These findings were consistent with the findings stated by Pinede et al. [4]

In our series of 30 patients, 11 (33.3%) were in NYHA Class II, 17 patients (56.6%) were in NYHA Class III and two patients (6.6%) were in NYHA Class IV. Kumar et al., [10] in their study of 61 intracavitory cardiac myxomas, showed 34.43% patients in NYHA Class II (which is in agreement with our study), 44.27% in NYHA Class III (which is lower as compared with our study) and 3.28% in NYHA Class IV.

The higher number of patients in NYHA Class III and Class IV in our study may be again attributed to a late presentation of our patients due to the reasons cited above and a larger size of tumors.

Echocardiography has evolved as a highly reliable method for diagnosing atrial myxomas. [11],[12] Many reports now advocate basing the pre-operative diagnosis of a myxoma solely on echocardiography. [13] Currently, two-dimensional (2D) echocardiography is being used with great facility in the pre-operative evaluation of atrial myxoma patients and remains the standard diagnostic method. [9] In our study, pre-operative diagnosis of cardiac myxoma was established in all 30 patients by echocardiography and a 100% reliability of this modality was subsequently proved per-operatively. Twenty-five patients (82.5%) had Left atrium (LA) myxoma and five patients (17.5%) had RA myxomas. Two patients were received in our department with an MRI diagnosis and one with a CT diagnosis of atrial myxoma, which was subsequently confirmed by echocardiography.

Surgery immediately following the diagnosis of a myxoma is imperative. Because local recurrence is common, extensive invasion have been described and surgery must be directed toward total tumor removal. [14] Generally, patients do very well during and following surgery, with a less than 3% operative mortality. [12] As multiple, especially biatrial myxomas occur, the surgical technique may vary, but always should be directed toward total extirpation. Traditional operative approaches have included bulk tumor removal and stalk cauterization. However, this is no longer recommended. [15] Modern cardiopulmonary bypass methods, preferably using hypothermic, pharmacological cardioplagia, are preferred to assure the adequate visualization necessary for complete tumor excision. [16]

Of 30 patients operated, a median sternotomy with bicaval venous cannulation has been used in 22 patients (73.2%). Although exposure is somewhat more difficult, a submammary incision with a right 4 th intercostals space, anterio-lateral thoacotomy has been used in eight patients (26.8%) for better cosmetic results. [17]

A right and left atriotomy (biatrial) was employed for resection of tumor in seven patients (23.3%); a right atriotomy in 19 patients (63.3%) and left atriotomy in four patients (13.3%).

Although a biatrial approach not only gives good exposure but also facilitates easy extraction of tumor with minimal handling, [18] we, at our institute, excised the majority (63.3%) of our myxomas successfully through a right atrial trans-septal approach. A trans-septal approach through right atriotomy suggested by Chitwood [19] is more practicable and conservative, gives good access to the myxoma with minimum handling and allows inspection of all cardiac chambers. [19]

In our study, 82.5% myxomas were located in the left atrium. The same findings have been reported by Centofanti et al. [8] -84.6%, Bortolotti et al. [7] -85%, Kabani et al. [20] -91.30% and Bjessmo and Ivert [21] -88%.

Of 25 left atrial myxomas, 22 (88%) arose from the interatrial septum, from (8%) from the post left atrial wall and one (4%) from the anterior left atrial wall. Among those arising from the interatrial septum, two patients (8%) had mitral regurgitation and were subjected to valve repair in addition to excision of the tumor. Of five right atrial myxomas, four (80%) arose from the right interatrial septum and one (20%) from the posterior part of the right atrial wall.

The most common location of myxomas was interatrial septum (87%). Other studies also show the interatrial septum to be the most common location of the tumor, but the percentage in our study is slightly higher than in the rest, viz. Centofauti et al. [8] 74.69%, Bortolothi et al. [7] 74.07% and Kabani et al. [20] 79.16%.

The aim of the operation was to remove not only the whole mass but also its base of attachment. In our study, all the interatrial septal myxomas, i.e. 26 (87%), were of medium to large size; hence, a wide excision of the tumor with patch closure (pericardial/PTFE) of the resulting septal defect was performed. The tumors attached elsewhere, four (13%), were removed by excision of the surrounding endocardium and underlying myocardium followed by patch closure. This method of wide surgical excision followed by patch closure was employed by Kabbani et al., [20] Cenntofanti et al., [8] Castells et al. [22] and Bjessmo et al., [21] with excellent outcome. When handling the mass, great care was always taken to avoid fragmentation and detachment and, therefore, potential systemic embolisation. At the end of the procedure, all cardiac chambers were routinely explored to rule out coexistent tumor or a detached tumor, but, when a myxoma was accidently fragmented during removal, the heart was generously irrigated with saline solution. In two patients with associated mitral regurgitation, mitral valve repair was performed after tumor excision from the left atrium.

The results of our surgical excision have been comparable to other centers with an operative mortality of about 6.6%. These results coincide with other studies, which show an overall average reported operative mortality of about 5%. [18],[19] Of the two patients who died post-operatively in our study, one died in the immediate post-operative period due to coagulopathy and another died on the 15 th post-operative day due to septicemia.

Wound infection and pneumonitis were the most common post-operative complications, which were managed by supportive treatment and specific antibiotics directed by culture sensitivity.


  Follow-Up Top


All surviving patients underwent physical examination, electrocardiography and echocardiography periodically during follow-up. Follow-up of patients ranged from 6 months to 15 years, with a mean duration of 5.7 years.

Of the 28 surviving patients, one died of bronchogenic carcinoma at the age of 65 years (5 years post-operatively) and another patient died at the age of 60 years (8 years post-operatively) because of carcinoma stomach.

Of the 26 surviving patients, eight were lost to follow-up, 14 had NYHA Class I symptoms and four had NYHA Class II symptoms.

One patient (3.3%) showed recurrence after 10 years of excision of right atrial myxoma. The recurrent site was left atrial septum. Recurrence can be due to inadequate resection, intraoperative implantation, embolization or multicentric growth. Most recurrences occur in LA. [23] Garbode was the first to report recurrence. Sporadic myxomas rarely recur. However, recurrence in familial cases is 30-75%. [24] The average recurrence occurs about 30 months after removal of the first myxoma. [25] In our series, only one (3.3%) of the atrial myxomas recurred within 10 years. This rate of recurrence has been reported by several authors like Castells et al., [22] who reported a recurrence of 3.7%.


  Conclusions Top


Myxomas are the most common tumor of the heart. They are frequently located in the left atrium and produce symptoms when they fragment and cause systemic emboli or when they interfere with cardiac valvular function and cause pulmonary congestion. Careful surgical management of these lesions should be curative with minimal early and late morbidity and mortality. Optimal operative technique emphasized minimal manipulation of the heart before institution of cardiopulmonary bypass and aortic cross-clamping and careful examination of the intracardiac chambers with meticulous removal of myxomatous debris. Recurrence of atrial myxomas can occur most likely in about 3% of the patients. However, extensive resection of the myxoma attached to the atrial septum or atrial wall can reduce the likelihood of recurrence to a greater extent. Thus, long-term clinical and echocardiographic follow-up is mandatory.

 
  References Top

1.Braunwalde, Kasper DL, Hauser SL, Fauci AS, Longo DL. Disorders of cardiovascular system: Cardiac tumours, cardiac manifestations of systemic disease. 15 th edition. Harrison's Principles of Internal Medicine. McGraw-Hill Professional, New York, 2001. p. 1372-4.  Back to cited text no. 1
    
2.Perichard RW. Tumors of the heart; a review of the subject and report of 150 cases. AMA Arch Pathol 1951;51:98-128.  Back to cited text no. 2
    
3.Bulklay BH, Hutchins GM. Atrial myxomas: A fifty year review. Am Heart J 1979;97:639-43.  Back to cited text no. 3
    
4.Pinede L, Duhant P, Loire R. Clinical presentation of left atrial cardiac myxoma: A series of 112 consecutive cases. Medicine (Baltimore) 2001;80:159-72.  Back to cited text no. 4
    
5.Acebo E, Val-Bernal JF, Gomez-Roman JJ. Revuelta JM. Clinicopathologic study and DNA analysis of 37 cardiac myxomas: A 28-year experience. Chest 2003;123:1379-85.  Back to cited text no. 5
    
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7.Bortolotti U, Maraglino G, Rubino M, Santini F, Mazzucco A, Milano A, et al. Surgical excision of intracardiac myxoma: A 20 year follow-up. Ann Thorac Surg 1990;49:449-53.  Back to cited text no. 7
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8.Centofanti P, Rosa ED, Deorsola L. Primary cardiac tumours: Early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999;68:1236-41.  Back to cited text no. 8
    
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22.Castells E, Ferran V, Octavio de Toledo MC, Calbet JM, Benito M, Fontanillas C, et al. Cardiac myxomas: Surgical treatment, long term results and recurrence. J Cardiovasc Surg (Torino) 1993;34:49-53.  Back to cited text no. 22
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24.McCarthy PM, Piehler JM, Schaff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr, et al. The significance of multiple, recurrent and "complex" cardiac myxomas. J Thorac Cardiovasc Surg 1986;91:389-96.  Back to cited text no. 24
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