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Atrial Myxoma
Introduction
Myxomas are the most common primary cardiac tumor.[1][2] More than 75% of myxomas originate in the left atrium, either at the mitral annulus or the fossa ovalis border of the interatrial septum; 20% arise from the right atrium, while 5% stem from both atria.[3][4] Atrial myxomas are associated with a triad of complications, including obstruction, emboli, and constitutional symptoms (eg, fever and weight loss).[3][5][6]
Etiology
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Etiology
Immunohistochemical studies suggest that myxoma cells originate from multipotent mesenchymal cells capable of neural and endothelial differentiation.[7] Myxomas associated with the inherited autosomal-dominant disorder Carney complex account for approximately 10% of all myomas, whereas the remainder appear to be sporadic.[8] The exact etiology of atrial myxoma is still under investigation.
Epidemiology
Atrial myxomas occur predominantly in women, with the peak between the fourth and sixth decades of life.[9][10] Some studies' results suggest that the female-to-male ratio is 2.05:1 for left atrial myxomas and 0.75:1 for right atrial myxomas.[4] Very few cases of atrial myxoma have been reported in pediatric individuals.[9][11] Most of these tumors, about 75%, are located in the left atrium, while right atrial myxomas make up 10% to 20%.[12]
Pathophysiology
Macroscopically, atrial myxomas are often pedunculated and soft. The myxoma diameter ranges from 1 to 15 cm, and the weight ranges from 15 to 180 g. The tumor can present with a smooth, villous, or friable surface. The villous and friable myxomas tend to be associated with embolic events, while the smooth myxomas are usually large and present more with an obstructive picture.[5]
Atrial myxomas are increasingly recognized as tumors that actively secrete cytokines, with interleukin-6 (IL-6) playing a central role in the systemic inflammatory and constitutional manifestations. The neoplastic myxoma cells continuously produce IL-6, and circulating IL-6 concentrations tend to parallel tumor burden, typically returning to baseline after surgical removal of the mass. Excess IL-6 is thought to underlie the characteristic “tumor syndrome” of fever, unintentional weight loss, malaise, diffuse myalgias or arthralgias, and fatigue, with these symptoms usually resolving once the myxoma is excised and IL-6 levels decrease.[13][14]
History and Physical
Atrial myxoma can present with multiple manifestations through various mechanisms.[15] These include:
- Obstructive symptoms
- Left-sided atrial myxomas
- Patients with left-sided myxomas usually develop signs and symptoms associated with mitral valve obstruction or regurgitation, left-sided heart failure, and secondary pulmonary hypertension. The most common symptoms are dyspnea with exertion, followed by orthopnea, paroxysmal nocturnal dyspnea, and pulmonary edema.[16] On physical examination, a "tumor plop" may be heard early in diastole.[17]
- Right-sided atrial myxomas
- Similarly, cardiovascular manifestations of right-sided myxomas are those of tricuspid stenosis and right heart failure. Common symptoms include exertional dyspnea, pedal edema, hepatomegaly, and ascites. On physical exam, a diastolic murmur, similar to the "tumor flop," can sometimes be appreciated at the tricuspid region; in addition, a prominent "a wave" in the jugular veins can also be observed occasionally.
- Both left-sided and right-sided atrial myxomas penetrate the myocardium and cause cardiac arrhythmias.[18]
- Left-sided atrial myxomas
- Thromboembolic symptoms
- Due to their high systolic pressure and location, left atrial myxomas were strongly associated with an increased risk of systemic embolization, particularly to the central nervous system, retinal arteries, viscera, spleen, kidneys, adrenals, abdominal aorta, iliac arteries, and femoropopliteal arteries.[19] Hence, patients can have a variety of presentations, such as transient ischemic attack, hemiplegia, loss of vision, chest pain, and dyspnea. Neurologic defects are likely the most serious complications of embolizations associated with left-sided atrial myxomas.[20]
- Right atrial myxomas can result in pulmonary arterial embolism or systemic embolism if either an atrial septal defect or a patent foramen ovale also coexist.[21] Pulmonary embolism can present as hypoxia, tachycardia, or sudden death. The manifestations of systemic embolization associated with atrial myxomas are outlined above.
- Constitutional symptoms
- Constitutional symptoms of atrial myxoma include fever, malaise, anorexia, arthralgia, and weight loss. The release of the cytokine IL-6, which plays a significant role in myxoma cell proliferation and the induction of acute-phase reactants, is strongly associated with systemic manifestations.[22]
- Other symptoms
- Atrial myxoma can present as part of the Carney complex. Patients with the Carney complex have unusual skin pigmentation and tend to develop tumors in endocrine tissues, including adrenal glands, thyroid, testes, and ovaries. Patients with a pituitary adenoma can also develop acromegaly. Patients with the Carney complex tend to develop recurrent atrial myxomas despite resections.[23][24][25]
Evaluation
Basic laboratory studies may reveal anemia, leukocytosis, thrombocytopenia, a high erythrocyte sedimentation rate, and elevated gamma globulin levels. Echocardiography, cardiac magnetic resonance imaging (MRI), cardiac computed tomography (CT), positron emission tomography (PET) imaging, and coronary angiography are valuable diagnostic tools. Generally, data obtained from these noninvasive imaging tools can provide sufficient information on tumor type, location, and the need for surgery.[15]
Echocardiography
Echocardiography is usually the diagnostic modality of choice. This modality can characterize the size, location, attachment, and mobility of the atrial mass, as well as the extent to which the tumor obstructs circulation and acts as a source of emboli. Transesophageal echocardiography (TEE) is superior to transthoracic echocardiography (TTE) for characterizing atrial masses, although TTE is simpler and is usually obtained first when clinical suspicion is present.[26] The former is more sensitive, detecting tumors up to 2 mm in size.
Cardiac MRI and CT
Cardiac MRI is another diagnostic test of choice. Cardiac MRI not only characterizes anatomic detail but also provides insight into the tumor microenvironment on T1- and T2-weighted sequences.[27][28] When cardiac MRI is unavailable or contraindicated, cardiac CT is a suitable alternative.
PET Scan
Although infrequently used, PET scanning can also characterize atrial myxomas.[29]
Coronary Angiography
Coronary angiography is essential in assessing the tumor's blood supply. If patients need to undergo surgical resection of the atrial tumor, coronary angiography is part of the routine preoperative evaluation.
Transvenous Biopsy
The need for a transvenous biopsy is determined on a case-by-case basis. Generally, noninvasive imaging is sufficient to identify and characterize atrial myxoma. Transvenous biopsy carries the risk of mobilizing tumor-associated emboli.
Treatment / Management
Considering the risk of cardiovascular complications and embolization, prompt surgical excision is the treatment of choice for atrial myxomas, irrespective of the size. Pericardial patch placement can help repair defects not amenable to primary closure. The resected tumor is sent to pathology to rule out other malignant tumors or metastasis.[30] Cardiac transplantation is proposed as a potential treatment option for recurrent atrial myxoma.[31](B2)
The surgical steps are as follows:
- Approach
- The chest is typically opened via a full median sternotomy. Less common alternatives include anterolateral thoracotomy at the fourth intercostal space, submammary approach in females, or a limited sternotomy with J or T extensions.
- Establishing cardiopulmonary bypass
- Aortic cannulation is performed via a single distal ascending aortic cannula. Venous drainage is achieved by separately cannulating the superior and inferior venae cavae, typically with 24 F and 28 F cannulae, respectively.
- Initiation of cardiac arrest
- Once cardiopulmonary bypass is initiated, the aorta is cross-clamped, and a combination of antegrade and retrograde cardioplegia is delivered per surgeon preference.
- Exposure of the left atrium
- The Sondergaard groove between the atria is dissected to obtain optimal left atrial exposure. Left atriotomy is made, and the tumor is examined, giving attention to the interatrial septum.
- Tumor excision
- The tumor is excised with a cuff of surrounding tissue (0.5–1 cm) to ensure complete removal, while avoiding damage to critical structures such as the mitral valve annulus or conduction pathways. If the tumor is large or complex to access through the left atrium alone, a right atriotomy may be performed, and the entire septum secundum can be removed to facilitate excision. Septal reconstruction might be required, and any resulting septal defect is repaired using either an autologous pericardial patch or a synthetic Gore-Tex patch, depending on the case.
- Verification and irrigation
- After tumor removal, the chamber is thoroughly examined for any residual fragments. The atrial cavity is then thoroughly irrigated with saline to remove debris before closure.
- Weaning and other considerations
- The patient is weaned off bypass and decannulated in the usual fashion. A "no-touch" approach is critical before cross-clamping the aorta to reduce the risk of embolization. In patients with left atrial tumors, left ventricular ventricularization via the right superior pulmonary vein should be avoided until the mass is removed. In cases with inferior vena cava extension beyond the pericardium, deep hypothermic circulatory arrest may be necessary.
Differential Diagnosis
Mural thrombi with myxoid stroma are a major differential diagnosis for atrial myxoma. The 2 conditions share a similar histopathological appearance, rendering immunohistochemistry unhelpful. The calretinin marker, which is specific for myxomas, can help differentiate them from mural myxoid thrombi.[32] Other malignant tumors, such as primary sarcoma, primary cardiac lymphoma, and large B-cell lymphoma, can also mimic atrial myxomas.[33][34][35][36]
Prognosis
The prognosis for patients undergoing surgical resection of atrial myxomas is excellent, as the operative mortality rate does not exceed 5% with rapid postoperative recovery.[3][5][37] Recurrence rates are 1% to 3% in sporadic cases, 12% in familial cases, and 22% in complex atrial myxomas.[5] Results from one study propose that minimal tumor manipulation, excision with adequate margins, and careful inspection of all heart chambers are important measures to prevent recurrence of tumors.[38]
Complications
Congestive Heart Failure Symptoms
Atrial myxomas can lead to the presentation of congested heart failure, and diuretics can provide symptomatic relief. Guideline-directed medical therapy for heart failure with beta-blockers, angiotensin-converting enzyme inhibitors, and spironolactone is usually unnecessary, as the left ventricular function is often intact.[16]
Cardiac Arrhythmia and Valvular Defect
Either local invasion of the tumor or surgical resection can precipitate cardiac arrhythmias. Mitral and tricuspid regurgitation or stenosis are also complications of atrial myxomas.
Thromboembolic Events
Patients with left atrial myxomas often present with ischemic stroke secondary to systemic embolization, in which case anticoagulation and antiplatelet therapies are warranted for secondary prevention.[39] The development of cerebral aneurysm and myxomatous metastasis is a delayed neurological complication of atrial myxomas; the presentation can resemble infectious endocarditis or vasculitis.[19] Right atrial myxomas, on the other hand, can cause pulmonary embolization that also requires anticoagulation treatment or embolectomy in severe cases. Pulmonary artery aneurysm is one of the delayed complications of atrial myxomas.
Infection
Atrial myxomas can also be a nidus for infection. Patients can present with signs and symptoms of infectious endocarditis, sepsis, or disseminated intravascular coagulation. The risk factors include dental procedures, invasive procedures, and immunosuppression. Streptococcus viridans, Staphylococcus, and Enterococcus faecalis are common pathogens. Extensive antibiotic therapy and surgical resection are mandatory for survival.[40]
Deterrence and Patient Education
Patients diagnosed with atrial myxoma should receive an overview of the disease's pathophysiology and complications. Patients should be educated to watch out for signs and symptoms that need immediate medical care, such as:
- Weakness or numbness, especially on one side of the body, is concerning for stroke (systemic embolization)
- Chest pain, shortness of breath, leg swelling (congestive heart failure, pulmonary embolism)
- Fever, chills, night sweats, unusual skin change (endocarditis)
Patients need to be aware that surgical resection is the treatment of choice with a good prognosis. The primary team needs to help patients set up a coordinated interdisciplinary team to treat the disease.
Enhancing Healthcare Team Outcomes
Effective management of atrial myxoma requires a coordinated, interprofessional strategy that integrates timely recognition, accurate diagnosis, and definitive surgical treatment. Physicians and advanced practitioners must maintain a high index of suspicion in patients presenting with unexplained embolic events, positional cardiac symptoms, or constitutional findings, and promptly initiate diagnostic evaluation with transthoracic or transesophageal echocardiography. Cardiologists and cardiothoracic surgeons collaborate to interpret imaging, assess surgical risk, and determine the urgency of resection to minimize embolic and obstructive complications. Clear communication of diagnostic findings, operative indications, and perioperative risks is essential to align the care plan with patient goals and ensure informed decision-making.
Nurses, pharmacists, and allied health professionals play a critical role in optimizing perioperative safety and longitudinal outcomes. Nursing staff coordinate preoperative assessments, monitor for hemodynamic instability or embolic sequelae, and provide postoperative surveillance for arrhythmias and recurrence. Pharmacists support medication reconciliation, anticoagulation management when indicated, and infection prevention strategies. Ongoing care coordination among the interprofessional team ensures structured follow-up, surveillance imaging, patient education on symptom recurrence, and adherence to postoperative plans. This collaborative approach enhances patient-centered care, improves team performance, and reduces the risk of preventable complications associated with atrial myxoma.
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