Highlights & Basics
- Atrial myxoma is the most common of the rare benign primary cardiac tumors.
- Usually found in the left atrium and typically attached to the septum.
- Most common presentation is with symptoms of mitral valve obstruction, followed by embolic manifestations. Constitutional symptoms include fever, fatigue, and weight loss.
- The only definitive treatment is surgical.
Quick Reference
History & Exam
Key Factors
dyspnea
syncope
dizziness
CHF/pulmonary edema
embolic manifestations
systolic or diastolic murmur
tumor plop
Other Factors
weight loss
fatigue
fever
pallor
arthralgia
Raynaud phenomenon
loud first heart sound
opening snap
Carney complex
intracerebral aneurysm
Diagnostics Tests
1st Tests to Order
echocardiogram
ECG
CBC
CXR
Other Tests to consider
erythrocyte sedimentation rate
C-reactive protein
protein electrophoresis
CT scan (chest)
MRI scan (chest)
biopsy
Treatment Options
acute
surgical candidate
myxoma resection (atriotomy)
valve repair/replacement and/or coronary artery bypass graft
postoperative aspirin or anticoagulation
treatment for dysrhythmia, embolization, and/or heart failure
non-surgical candidate
treatment for dysrhythmia, embolization, and/or heart failure
Definition
Classifications
Benign primary cardiac tumors
- Myxoma
- Papillary fibroelastoma
- Fibroma
- Lipoma
- Rhabdomyoma.
Vignette
Common Vignette 1
Common Vignette 2
Other Presentations
Epidemiology
Etiology
Pathophysiology
Images
Large left atrial myxoma
Macroscopic view of a left atrial myxoma
Two-dimensional echocardiogram showing a right atrial mass suggestive of a myxoma
Atrial myxoma identified on echocardiography (A) and following surgical resection (B)
Chest CT demonstrating a mass in the right atrium (RA) subsequently confirmed to be an atrial myxoma. RV = right ventricle, LV = left ventricle
Two-dimensional echocardiogram of a right atrial thrombus (note not attached to septum)
Transesophageal echocardiogram showing a catheter in the superior vena cava and a right atrial thrombus (note not attached to septum)
Diagnostic Approach
History and exam
Imaging
Blood tests
- Anemia (49% of patients)
- Elevated serum gamma globulin (45% of patients)
- Elevated erythrocyte sedimentation rate (55% of patients)
- Elevated serum C-reactive protein (75% of patients).[8]
Biopsy
Risk Factors
History & Exam
Tests
Differential Diagnosis
Mitral stenosis
Differentiating Signs/Symptoms
- There may be no differences in symptoms and signs.
Differentiating Tests
- ECG will most frequently reveal left atrial hypertrophy. Echocardiogram will show stenosis of the mitral valve.
Infective endocarditis
Differentiating Signs/Symptoms
- Syncope is unusual as a presenting symptom in infective endocarditis. Patients with endocarditis may have a fever, petechiae, splenomegaly, history of drug abuse, previous endocarditis, or prosthetic valves. Otherwise there may be no major differentiating symptoms and signs.
Differentiating Tests
- Echocardiogram will reveal the valvular vegetation. Blood culture indicates the causative organism.
Differentiating Signs/Symptoms
- There may be no differences in symptoms and signs.Images
Differentiating Tests
- Echocardiogram or MRI/CT scan will reveal the thrombus. Echocardiogram will also identify the catheter that is commonly associated with the formation of the thrombus. Rarely a transvenous biopsy may be indicated.
Differentiating Signs/Symptoms
- Symptoms of arthralgia and fatigue, and peripheral signs of vasculitis. Syncope and acute heart failure are very unusual presenting features.
Differentiating Tests
- Specific ANAs in the blood may identify the disease process. ECG findings in connective tissue disease are nonspecific with no differentiating features.
Differentiating Signs/Symptoms
- There may be no differences in symptoms and signs.
Differentiating Tests
- Imaging studies (e.g., CT scan and MRI) will reveal cardiac tumor.
Criteria
Screening
Treatment Approach
Treatment
- Arrhythmias are treated with the appropriate antiarrhythmic drug until surgery can be performed.
- If there is any evidence of residual heart failure, then appropriate long-term therapies (e.g., beta-blockers, ACE inhibitors, and furosemide) may be needed in individual cases.
- No guidelines or recommendations exist regarding treatment of embolic phenomena in cases of myxoma. The definitive treatment is surgical removal of the cardiac tumor.
- The treatment of embolic phenomena depends on the vascular territory involved. In cases where large vessels are involved, anticoagulation is started based on an initial diagnosis of vascular occlusion, but the definitive treatment is surgical removal of the myxomatous tissue.[12]
Treatment Options
surgical candidate
myxoma resection (atriotomy)
Comments
- Generally, after median sternotomy, the myxoma is surgically excised using cardiopulmonary bypass and cardioplegic arrest. The tumor is removed by either right or left atriotomy or combined atriotomy, depending on the site and extent of the tumor.
- The choice of technique also depends on associated conditions that need surgical intervention, such as valve repair or replacement, and coronary artery bypass graft.
valve repair/replacement and/or coronary artery bypass graft
Comments
- The choice of surgical technique depends on associated conditions that need surgical intervention, such as valve repair or replacement, and coronary artery bypass graft.
postoperative aspirin or anticoagulation
Comments
- Postoperatively, there is no definitive recommendation for anticoagulation if the myxoma is removed. However, postoperative aspirin or anticoagulation should be considered if an embolic event has occurred or if there are other indications for such therapy, though the evidence base for such an approach is lacking. Consult your local protocols for guidance on choice of agent and dose.
treatment for dysrhythmia, embolization, and/or heart failure
Comments
- If there is any associated condition (e.g., arrhythmias), then medications may be needed to control the arrhythmias until surgery. If there is any evidence of residual heart failure, then appropriate long-term therapies (e.g., beta-blockers, ACE inhibitors, and furosemide) may be needed in individual cases.
non-surgical candidate
treatment for dysrhythmia, embolization, and/or heart failure
Comments
- Those who have extensive comorbidity with poor prognosis and likely to succumb to cardiac surgery are not candidates for surgery.
- If there is any associated condition (e.g., arrhythmias), then medications may be needed to control the arrhythmias. If there is any evidence of residual heart failure, then appropriate long-term therapies (e.g., beta-blockers, ACE inhibitors, and furosemide) may be needed in individual cases.
- The role of anticoagulation in these patients is not established, hence this should be individualized. Aspirin is not routinely recommended for these patients.
Emerging Tx
Cardiac autotransplantation
Follow-Up Overview
Prognosis
Recurrence
Monitoring
Complications
Citations
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001 May;80(3):159-72.[Abstract]
Edwards A, Bermudez C, Piwonka G, et al. Carney's syndrome: complex myxomas. Report of four cases and review of the literature. Cardiovasc Surg. 2002 Jun;10(3):264-75.[Abstract]
Grossniklaus HE. McLean IW, Gillespie JJ. Bilateral eyelid myxomas in Carney's complex. Br J Ophthalmol. 1991 Apr;75(4):251-2.[Abstract][Full Text]
Ohara N, Komiya I, Yamauchi K, et al. Carney's complex with primary pigmented nodular adrenocortical disease and spotty pigmentations. Intern Med. 1993 Jan;32(1):60-2.[Abstract][Full Text]
1. Zhang R, Tang Z, Qiao Q, et al. Anesthesia management of atrial myxoma resection with multiple cerebral aneurysms: a case report and review of the literature. BMC Anesthesiol. 2020 Jul 4;20(1):164.[Abstract][Full Text]
2. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996 Jan 1;77(1):107.[Abstract]
3. Bussani R, Castrichini M, Restivo L, et al. Cardiac tumors: diagnosis, prognosis, and treatment. Curr Cardiol Rep. 2020 Oct 10;22(12):169.[Abstract][Full Text]
4. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001 May;80(3):159-72.[Abstract]
5. Edwards A, Bermudez C, Piwonka G, et al. Carney's syndrome: complex myxomas. Report of four cases and review of the literature. Cardiovasc Surg. 2002 Jun;10(3):264-75.[Abstract]
6. Grossniklaus HE. McLean IW, Gillespie JJ. Bilateral eyelid myxomas in Carney's complex. Br J Ophthalmol. 1991 Apr;75(4):251-2.[Abstract][Full Text]
7. Ohara N, Komiya I, Yamauchi K, et al. Carney's complex with primary pigmented nodular adrenocortical disease and spotty pigmentations. Intern Med. 1993 Jan;32(1):60-2.[Abstract][Full Text]
8. Acebo E, Val-Bernal JF, Gomez-Roman JJ, et al. Clinicopathologic study and DNA analysis of 37 cardiac myxomas: a 28-year experience. Chest. 2003 May;123(5):1379-85.[Abstract]
9. Cotran RS, Kumar V, Robbins SL. Robbins pathologic basis of disease. 5th ed. Philadelphia, PA: Saunders; 1994.
10. Griborio-Guzman AG, Aseyev OI, Shah H, et al. Cardiac myxomas: clinical presentation, diagnosis and management. Heart. 2022 May 12;108(11):827-33.[Abstract]
11. Boyacıoğlu K, Kalender M, Dönmez AA, et al. Outcomes following embolization in patients with cardiac myxoma. J Card Surg. 2017 Oct;32(10):621-6.[Abstract]
12. Shavit L, Appelbaum L, Grenader T. Atrial myxoma presenting with total occlusion of the abdominal aorta and multiple peripheral embolism. Eur J Intern Med. 2007 Jan;18(1):74-5.[Abstract]
13. Panos A, Kalangos A, Sztajzel J. Left atrial myxoma presenting with myocardial infarction. Case report and review of the literature. Int J Cardiol. 1997 Oct 31;62(1):73-5.[Abstract]
14. Rongjing D, Kun X. Myocardial infarction in a woman with no apparent risk factors. Lancet. 2007 Jul 28;370(9584):360.[Abstract]
15. Reardon MJ, Malaisrie SC, Walkes JC, et al. Cardiac autotransplantation for primary cardiac tumors. Ann Thorac Surg. 2006 Aug;82(2):645-50.[Abstract]
16. Karabinis A, Samanidis G, Khoury M, et al. Clinical presentation and treatment of cardiac myxoma in 153 patients. Medicine (Baltimore). 2018 Sep;97(37):e12397.[Abstract][Full Text]
17. Jiang CX, Wang JG, Qi RD, et al. Long-term outcome of patients with atrial myxoma after surgical intervention: analysis of 403 cases. J Geriatr Cardiol. 2019 Apr;16(4):338-43.[Abstract][Full Text]
18. Bjessmo S, Ivert T. Cardiac myxoma: 40 years' experience in 63 patients. Ann Thorac Surg. 1997 Mar;63(3):697-700.[Abstract]
19. McAllister SM, Bornstein AM, Callen JP. Painful acral purpura. Arch Dermatol. 1998 Jul;134(7):789-91.[Abstract]
20. Kajihara N, Tanoue Y, Eto M, et al. Surgical experience of cardiac tumors: early and late results. Surg Today. 2006;36(7):602-7.[Abstract]
21. Sugimoto T, Ogawa K, Asada T, et al. The problems of surgical treatment for cardiac myxoma and associated lesions. Surg Today. 1994;24(8):673-80.[Abstract]
22. Keeling IM, Oberwalder P, Anelli-Monti M, et al. Cardiac myxomas: 24 years of experience in 49 patients. Eur J Cardiothorac Surg. 2002 Dec;22(6):971-7.[Abstract]
23. McCarthy PM, Piehler JM, Schaff HV, et al. The significance of multiple, recurrent, and "complex" cardiac myxomas. J Thorac Cardiovasc Surg. 1986 Mar;91(3):389-96.[Abstract]