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Atrial Myxoma

Updated : April 22, 2024





Background

The most prevalent primary heart tumor is a myxoma.  It is estimated that more than 75% of myxomas develop in the left atrium, either at the mitral annulus or the fossa ovalis boundary of the interatrial septum, about 20% originate from the right atrium, and 5% originate from both atria and ventricles. Common issues associated with atrial myxomas include emboli, cardiovascular obstructions and symptoms such as weight loss and fever.

Epidemiology

Atrial myxomas are most common in females between the age of 40 and 60. Some research indicates that men are around 25% more like to develop right atrial myxomas and women are more than twice likely to develop left atrial myxoma. There are very few instances of children developing atrial myxomas.

Anatomy

Pathophysiology

Macroscopically, atrial myxomas are typically pedunculated and are of a soft consistency. Myxomas range in size from 1cm-15cm in circumference and 15g-180 g in weight. The surface of the tumor may be smooth, villous, or fragile.

Myxomas which are connected with embolic events typically have a villous or fragile surface, but myxomas with smooth surface are typically large and more obstructive. Atrial myxomas frequently produce vascular endothelial growth factor, which induces angiogenesis, as well as diverse cytokines and growth factors.

These factors lead to physical symptoms such as:

  • Weight loss
  • Fever
  • Malaise
  • Anorexia
  • High sedimentation rate

Etiology

Immunohistochemical investigations reveal that myxoma cells derive from neural and endothelial-differentiable mesenchymal cells. Carney’s complex, a hereditary autosomal dominant condition, accounts for 10% of myxomas, whereas the rest appear to be sporadic. The precise cause of atrial myxoma is still being investigated.

Genetics

Prognostic Factors

Patients who undergo a surgical route for atrial myxomas generally have a favorable prognosis. Mortality rate following surgical resection is below 5% and postoperative recovery is quick.

Given below are the recurrence rates following surgery:

  • Sporadic cases: 1%-3%
  • Familial cases: 12%
  • Complex cases: 24%

One study suggests that limited treatment of tumors, excision with proper margins, and meticulous evaluation of all heart chambers are crucial factors for preventing tumor recurrence.

Clinical History

Age Group:  

Atrial myxoma is typically occurs in adults, most commonly in individuals aged 30 to 60 years old. 

Physical Examination

  • Cardiac Auscultation: Listening to the heart sounds can provide important clues. Specific findings may include: 
  • Peripheral Signs of Embolization: Examination of the peripheral vascular system may reveal signs suggestive of embolization. 
  • Neurological Examination: Given the risk of embolic events to the brain, a thorough neurological examination should be performed to assess for signs of stroke or transient ischemic attack (TIA). 
  • Signs of Heart Failure: Patients with atrial myxoma may present with signs and symptoms of heart failure. 

Age group

Associated comorbidity

Palpitations and an irregular heartbeat are two symptoms of atrial fibrillation that can be brought on by atrial myxoma.  

The possibility of embolic events is a major risk factor for atrial myxoma. Tumor fragments have the potential to break off and enter the circulation, where they might damage many organs. 

Atrial myxoma may result in symptoms including weariness, dizziness, shortness of breath, and chest discomfort that are indicative of heart failure. 

Heart valve dysfunction symptoms, such as murmurs or heart failure symptoms, can result from a tumor obstructing blood flow within the heart. 

Associated activity

Acuity of presentation

The presentation of atrial myxoma can vary widely depending on several factors, including the size and location of the tumor, as well as whether it causes obstruction of blood flow.  

Differential Diagnoses

  • Valvular Heart Disease: Conditions such as mitral stenosis or mitral regurgitation can present with similar symptoms and signs, including dyspnea, fatigue, and murmurs. 
  • Infective Endocarditis: This is an infection of the endocardium, which may involve heart valves or other cardiac structures. Symptoms can include fever, new murmurs, and embolic events, which can overlap with atrial myxoma. 
  • Atrial Septal Defect (ASD): Congenital defects in the atrial or ventricular septum can present with similar symptoms, including heart murmurs, palpitations, and signs of heart failure. 
  • Pulmonary Embolism: This occurs when a blood clot travels to the lungs, causing symptoms such as dyspnea, chest pain, and hemoptysis, which can overlap with atrial myxoma. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

  • Surgical Resection: The primary treatment for atrial myxoma is surgical removal of the tumor. This is typically performed via open-heart surgery with cardiopulmonary bypass. 
  • Long-term Follow-up: After discharge from the hospital, patients require long-term follow-up with serial imaging studies to monitor for recurrence of the tumor or development of complications.  
  • Genetic Counseling: Atrial myxoma can be associated with familial syndromes, such as Carney complex or familial cardiac myxoma syndrome.  
  • Symptomatic Management: In cases where surgery is not feasible or if the patient is not a surgical candidate, symptomatic management may include medical therapy to control symptoms. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Use of non-pharmacological approach for Atrial Myxoma

  • Cardiovascular Risk Factor Management: Managing cardiovascular risk factors such as hypertension, hyperlipidemia, and diabetes mellitus is essential to reduce the risk of complications such as atherosclerosis and thromboembolism, which may exacerbate the risk associated with atrial myxoma. 
  • Arrhythmia Management: Atrial myxoma can predispose patients to arrhythmias such as atrial fibrillation. Therefore, appropriate management of arrhythmias through pharmacological therapy, electrical cardioversion, or catheter ablation may be necessary to maintain sinus rhythm and reduce the risk of thromboembolic events. 
  • Regular Cardiac Surveillance: Long-term follow-up with regular cardiac imaging studies, such as echocardiography, is essential to monitor for recurrence of the tumor, changes in tumor size or morphology, and associated cardiac abnormalities. 
  • Healthy Lifestyle Modifications: Encouraging patients to adopt a healthy lifestyle, including regular exercise, smoking cessation, and a balanced diet, can help mitigate cardiovascular risk factors and promote overall cardiovascular health. 

Use of Anticoagulants

  • Warfarin: Warfarin is a vitamin K antagonist that interferes with the synthesis of clotting factors in the liver. It has been traditionally used for anticoagulation therapy in patients with atrial myxoma to reduce the risk of thromboembolic events.  
  • Apixaban: Apixaban is a direct oral anticoagulant that inhibits factor Xa, an essential component of the coagulation cascade. Apixaban is used for stroke prevention in patients with non-valvular atrial fibrillation and for the treatment and prevention of DVT and PE. 

Use of Intervention with a procedure in treating Atrial Myxoma

  • Cardiopulmonary Bypass: The surgery is typically performed under general anesthesia. The patient is connected to a cardiopulmonary bypass machine, which temporarily takes over the function of the heart and lungs during the surgery. 

The patient’s blood is diverted through the cardiopulmonary bypass machine, allowing the heart to be stopped temporarily while the surgery is performed. 

Use of phases in managing Atrial Myxoma

  • Diagnostic Phase: This phase involves the initial evaluation and diagnosis of atrial myxoma. It may include a thorough medical history, physical examination, imaging studies, and possibly biopsy for confirmation of the diagnosis. 
  • Preoperative Phase: Once the diagnosis of atrial myxoma is established, patients undergo a preoperative assessment to evaluate their overall health and surgical candidacy. 
  • Surgical Intervention Phase: The primary intervention for atrial myxoma is surgical resection of the tumor. This phase involves the actual surgical procedure, which typically includes median sternotomy, cardiopulmonary bypass, tumor resection, and closure of the atrial incision. 
  • Postoperative Phase: Following surgical resection, patients enter the postoperative phase, which involves intensive monitoring in the immediate postoperative period, typically in the intensive care unit (ICU). 
  • Rehabilitation and Recovery Phase: As patients recover from surgery, they transition to the rehabilitation and recovery phase, which involves gradual mobilization, physical therapy, and supportive care to regain strength and function. 
  • Long-term Follow-up Phase: Atrial myxoma requires long-term surveillance to monitor for recurrence of the tumor, development of complications, and overall cardiac health.  

Medication

Media Gallary

Atrial Myxoma

Updated : April 22, 2024




The most prevalent primary heart tumor is a myxoma.  It is estimated that more than 75% of myxomas develop in the left atrium, either at the mitral annulus or the fossa ovalis boundary of the interatrial septum, about 20% originate from the right atrium, and 5% originate from both atria and ventricles. Common issues associated with atrial myxomas include emboli, cardiovascular obstructions and symptoms such as weight loss and fever.

Atrial myxomas are most common in females between the age of 40 and 60. Some research indicates that men are around 25% more like to develop right atrial myxomas and women are more than twice likely to develop left atrial myxoma. There are very few instances of children developing atrial myxomas.

Macroscopically, atrial myxomas are typically pedunculated and are of a soft consistency. Myxomas range in size from 1cm-15cm in circumference and 15g-180 g in weight. The surface of the tumor may be smooth, villous, or fragile.

Myxomas which are connected with embolic events typically have a villous or fragile surface, but myxomas with smooth surface are typically large and more obstructive. Atrial myxomas frequently produce vascular endothelial growth factor, which induces angiogenesis, as well as diverse cytokines and growth factors.

These factors lead to physical symptoms such as:

  • Weight loss
  • Fever
  • Malaise
  • Anorexia
  • High sedimentation rate

Immunohistochemical investigations reveal that myxoma cells derive from neural and endothelial-differentiable mesenchymal cells. Carney’s complex, a hereditary autosomal dominant condition, accounts for 10% of myxomas, whereas the rest appear to be sporadic. The precise cause of atrial myxoma is still being investigated.

Patients who undergo a surgical route for atrial myxomas generally have a favorable prognosis. Mortality rate following surgical resection is below 5% and postoperative recovery is quick.

Given below are the recurrence rates following surgery:

  • Sporadic cases: 1%-3%
  • Familial cases: 12%
  • Complex cases: 24%

One study suggests that limited treatment of tumors, excision with proper margins, and meticulous evaluation of all heart chambers are crucial factors for preventing tumor recurrence.

Age Group:  

Atrial myxoma is typically occurs in adults, most commonly in individuals aged 30 to 60 years old. 

  • Cardiac Auscultation: Listening to the heart sounds can provide important clues. Specific findings may include: 
  • Peripheral Signs of Embolization: Examination of the peripheral vascular system may reveal signs suggestive of embolization. 
  • Neurological Examination: Given the risk of embolic events to the brain, a thorough neurological examination should be performed to assess for signs of stroke or transient ischemic attack (TIA). 
  • Signs of Heart Failure: Patients with atrial myxoma may present with signs and symptoms of heart failure. 

Palpitations and an irregular heartbeat are two symptoms of atrial fibrillation that can be brought on by atrial myxoma.  

The possibility of embolic events is a major risk factor for atrial myxoma. Tumor fragments have the potential to break off and enter the circulation, where they might damage many organs. 

Atrial myxoma may result in symptoms including weariness, dizziness, shortness of breath, and chest discomfort that are indicative of heart failure. 

Heart valve dysfunction symptoms, such as murmurs or heart failure symptoms, can result from a tumor obstructing blood flow within the heart. 

The presentation of atrial myxoma can vary widely depending on several factors, including the size and location of the tumor, as well as whether it causes obstruction of blood flow.  

  • Valvular Heart Disease: Conditions such as mitral stenosis or mitral regurgitation can present with similar symptoms and signs, including dyspnea, fatigue, and murmurs. 
  • Infective Endocarditis: This is an infection of the endocardium, which may involve heart valves or other cardiac structures. Symptoms can include fever, new murmurs, and embolic events, which can overlap with atrial myxoma. 
  • Atrial Septal Defect (ASD): Congenital defects in the atrial or ventricular septum can present with similar symptoms, including heart murmurs, palpitations, and signs of heart failure. 
  • Pulmonary Embolism: This occurs when a blood clot travels to the lungs, causing symptoms such as dyspnea, chest pain, and hemoptysis, which can overlap with atrial myxoma. 
  • Surgical Resection: The primary treatment for atrial myxoma is surgical removal of the tumor. This is typically performed via open-heart surgery with cardiopulmonary bypass. 
  • Long-term Follow-up: After discharge from the hospital, patients require long-term follow-up with serial imaging studies to monitor for recurrence of the tumor or development of complications.  
  • Genetic Counseling: Atrial myxoma can be associated with familial syndromes, such as Carney complex or familial cardiac myxoma syndrome.  
  • Symptomatic Management: In cases where surgery is not feasible or if the patient is not a surgical candidate, symptomatic management may include medical therapy to control symptoms. 

  • Cardiovascular Risk Factor Management: Managing cardiovascular risk factors such as hypertension, hyperlipidemia, and diabetes mellitus is essential to reduce the risk of complications such as atherosclerosis and thromboembolism, which may exacerbate the risk associated with atrial myxoma. 
  • Arrhythmia Management: Atrial myxoma can predispose patients to arrhythmias such as atrial fibrillation. Therefore, appropriate management of arrhythmias through pharmacological therapy, electrical cardioversion, or catheter ablation may be necessary to maintain sinus rhythm and reduce the risk of thromboembolic events. 
  • Regular Cardiac Surveillance: Long-term follow-up with regular cardiac imaging studies, such as echocardiography, is essential to monitor for recurrence of the tumor, changes in tumor size or morphology, and associated cardiac abnormalities. 
  • Healthy Lifestyle Modifications: Encouraging patients to adopt a healthy lifestyle, including regular exercise, smoking cessation, and a balanced diet, can help mitigate cardiovascular risk factors and promote overall cardiovascular health. 

  • Warfarin: Warfarin is a vitamin K antagonist that interferes with the synthesis of clotting factors in the liver. It has been traditionally used for anticoagulation therapy in patients with atrial myxoma to reduce the risk of thromboembolic events.  
  • Apixaban: Apixaban is a direct oral anticoagulant that inhibits factor Xa, an essential component of the coagulation cascade. Apixaban is used for stroke prevention in patients with non-valvular atrial fibrillation and for the treatment and prevention of DVT and PE. 

  • Cardiopulmonary Bypass: The surgery is typically performed under general anesthesia. The patient is connected to a cardiopulmonary bypass machine, which temporarily takes over the function of the heart and lungs during the surgery. 

The patient’s blood is diverted through the cardiopulmonary bypass machine, allowing the heart to be stopped temporarily while the surgery is performed. 

  • Diagnostic Phase: This phase involves the initial evaluation and diagnosis of atrial myxoma. It may include a thorough medical history, physical examination, imaging studies, and possibly biopsy for confirmation of the diagnosis. 
  • Preoperative Phase: Once the diagnosis of atrial myxoma is established, patients undergo a preoperative assessment to evaluate their overall health and surgical candidacy. 
  • Surgical Intervention Phase: The primary intervention for atrial myxoma is surgical resection of the tumor. This phase involves the actual surgical procedure, which typically includes median sternotomy, cardiopulmonary bypass, tumor resection, and closure of the atrial incision. 
  • Postoperative Phase: Following surgical resection, patients enter the postoperative phase, which involves intensive monitoring in the immediate postoperative period, typically in the intensive care unit (ICU). 
  • Rehabilitation and Recovery Phase: As patients recover from surgery, they transition to the rehabilitation and recovery phase, which involves gradual mobilization, physical therapy, and supportive care to regain strength and function. 
  • Long-term Follow-up Phase: Atrial myxoma requires long-term surveillance to monitor for recurrence of the tumor, development of complications, and overall cardiac health.