Cardiac myxomas represent prevalent primary neoplasms of the heart. Despite their biological benignity, they are considered “functionally malignant” due to their capacity for embolization. While their most frequent origin is the left atrium, no heart chamber is exempt from their occurrence.
Although predominantly sporadic, cardiac myxomas can also manifest as part of familial cases, particularly in the context of the Carney complex. Cardiac myxomas are often found in the left atrium of the heart, attached to the wall that separates the left and right sides of the heart (interatrial septum). However, they can also occur in the right atrium.
These tumors are composed of connective tissue cells and mucous-like material. The mucous material gives them a gelatinous appearance. While cardiac myxomas are often considered sporadic and not linked to specific risk factors, there may be a connection with certain genetic conditions in some cases.
Epidemiology
Cardiac myxomas can occur at any age, but they are most commonly diagnosed in adults between the ages of 30 and 60. They are rare in children. The majority of cardiac myxomas are found in the left atrium, typically attached to the interatrial septum. About 75-80% of myxomas occur in the left atrium. Right atrial myxomas are less common but can also occur.
Recurrence of cardiac myxomas after surgical removal is uncommon but can occur, particularly in cases associated with genetic syndromes. In some cases, cardiac myxomas may be associated with genetic syndromes such as Carney complex, a rare, autosomal dominant disorder characterized by cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity. However, the majority of cardiac myxomas are sporadic.
Anatomy
Pathophysiology
The pathophysiology of cardiac myxoma involves the development of a benign tumor within the heart, most commonly located in the left atrium. These tumors are characterized by a gelatinous and mucoid consistency, composed of stellate-shaped cells embedded in a matrix of myxoid material. While the exact etiology remains unclear, some cases of cardiac myxomas are associated with genetic syndromes such as Carney complex.
The tumors may originate from multipotent mesenchymal cells, and their growth within the heart can lead to various clinical manifestations. The myxomas often attach to the interatrial septum and can obstruct blood flow, causing symptoms such as dyspnea, chest pain, and palpitations. Additionally, fragments of the tumor can break off, leading to embolism in distant organs.
The tumor’s presence can interfere with normal cardiac function, causing hemodynamic disturbances. Overall, while cardiac myxomas are rare, understanding their pathophysiology is crucial for timely diagnosis and appropriate management, especially considering the potential for serious complications related to their presence in the heart.
Etiology
Hormonal Influence: There is some evidence suggesting a potential hormonal influence on the development of cardiac myxomas, particularly in cases associated with Carney complex. However, the precise mechanisms by which hormones may contribute to myxoma formation still need to be fully elucidated.
Genetic Factors: While most cardiac myxomas are sporadic, a small percentage is associated with genetic syndromes, the most notable being Carney complex. Carney complex is a rare, autosomal dominant disorder characterized by multiple cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity.
Multipotent Mesenchymal Cells: It is believed that cardiac myxomas may originate from multipotent mesenchymal cells, which have the potential to differentiate into various cell types. The transformation of these cells into myxomas is thought to contribute to the development of the tumor.
Cellular Abnormalities: Molecular studies have indicated that certain cellular abnormalities, including mutations in genes such as PRKAR1A, may play a role in the pathogenesis of cardiac myxomas. However, the significance of these genetic alterations in sporadic cases remains an area of ongoing research.
Genetics
Prognostic Factors
Clinical History
The clinical manifestation of cardiac myxomas is contingent upon factors such as their location, size, and mobility, often characterized by the triad of embolization, intracardiac obstruction, and constitutional symptoms. These tumors are frequently linked to constitutional symptoms that mimic those seen in inflammatory or connective tissue disorders.
The prevalence of these symptoms tends to be higher in women compared to men, in cases of right-sided myxomas as opposed to left-sided ones, and in instances where the myxomas are large or multicentric. Common constitutional symptoms include malaise, anorexia, fever, arthralgia, and weight loss.
This range of clinical presentations underscores the variable nature of cardiac myxomas. It emphasizes the importance of considering factors such as tumor characteristics and patient demographics in understanding their clinical impact.
Physical Examination
A triad of embolic manifestations, intracardiac obstruction, and constitutional symptoms characterize cardiac myxomas. However, due to variations in size, location, histopathology, and morphology, they may stay completely asymptomatic, present with classical indications, or give rise to life-threatening emergencies such as systemic embolization or sudden cardiac death.
During a general examination, observable features may include fever, cachexia, clubbing, cyanosis, or rash. Engorged neck veins and a prominent A wave in the jugular venous pulse may be evident. Precordial results may mimic mitral stenosis, with a loud and widely split first heart sound (S1) due to delayed mitral valve closure caused by tumor prolapse into the mitral valve orifice.
The pulmonary element of the second heart sound (P2) may vary in intensity based on the presence of pulmonary hypertension. A distinctive “tumor plop,” a low-pitched premature diastolic sound following S2, may be discerned, resembling the opening snap of rheumatic mitral stenosis, albeit at a different pitch.
Additionally, audible sounds may include a third heart sound (S3), a fourth heart sound (S4), tricuspid stenosis, or a diastolic murmur indicative of functional mitral. In some cases, a systolic murmur associated with mitral or tricuspid regurgitation may be present. Notably, the auscultatory results of cardiac myxomas exhibit characteristic changes with alterations in the patient’s position.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Fibroelastoma
Papillary Fibroelastoma
Infective Endocarditis
Cardiac Thrombus
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Peri-operative management
Diagnosis is typically made using imaging studies, with echocardiography being the primary diagnostic tool. Other imaging modalities, such as cardiac MRI or CT scans, may be employed for further evaluation. The goal is to accurately determine the location, size, and characteristics of the myxoma.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
A careful excision of the tumor is performed to ensure complete removal while minimizing the risk of embolization or damage to surrounding structures. In some cases, minimally invasive approaches may be considered, especially for small, accessible myxomas. After surgical removal, close monitoring is essential to assess cardiac function and detect any potential complications.
Anticoagulation may be initiated in some cases to prevent thromboembolic events, especially if there is evidence of embolization before surgery. Symptomatic relief measures may be initiated as needed, especially for constitutional symptoms such as fever, malaise, or weight loss. Regular follow-up visits are important to monitor for any signs of recurrence or the development of new myxomas.
Genetic Counseling
In cases where cardiac myxomas are associated with genetic syndromes, genetic counseling may be recommended for the patient and their family members to assess the risk of recurrence and associated conditions.
Cardiac myxomas represent prevalent primary neoplasms of the heart. Despite their biological benignity, they are considered “functionally malignant” due to their capacity for embolization. While their most frequent origin is the left atrium, no heart chamber is exempt from their occurrence.
Although predominantly sporadic, cardiac myxomas can also manifest as part of familial cases, particularly in the context of the Carney complex. Cardiac myxomas are often found in the left atrium of the heart, attached to the wall that separates the left and right sides of the heart (interatrial septum). However, they can also occur in the right atrium.
These tumors are composed of connective tissue cells and mucous-like material. The mucous material gives them a gelatinous appearance. While cardiac myxomas are often considered sporadic and not linked to specific risk factors, there may be a connection with certain genetic conditions in some cases.
Cardiac myxomas can occur at any age, but they are most commonly diagnosed in adults between the ages of 30 and 60. They are rare in children. The majority of cardiac myxomas are found in the left atrium, typically attached to the interatrial septum. About 75-80% of myxomas occur in the left atrium. Right atrial myxomas are less common but can also occur.
Recurrence of cardiac myxomas after surgical removal is uncommon but can occur, particularly in cases associated with genetic syndromes. In some cases, cardiac myxomas may be associated with genetic syndromes such as Carney complex, a rare, autosomal dominant disorder characterized by cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity. However, the majority of cardiac myxomas are sporadic.
The pathophysiology of cardiac myxoma involves the development of a benign tumor within the heart, most commonly located in the left atrium. These tumors are characterized by a gelatinous and mucoid consistency, composed of stellate-shaped cells embedded in a matrix of myxoid material. While the exact etiology remains unclear, some cases of cardiac myxomas are associated with genetic syndromes such as Carney complex.
The tumors may originate from multipotent mesenchymal cells, and their growth within the heart can lead to various clinical manifestations. The myxomas often attach to the interatrial septum and can obstruct blood flow, causing symptoms such as dyspnea, chest pain, and palpitations. Additionally, fragments of the tumor can break off, leading to embolism in distant organs.
The tumor’s presence can interfere with normal cardiac function, causing hemodynamic disturbances. Overall, while cardiac myxomas are rare, understanding their pathophysiology is crucial for timely diagnosis and appropriate management, especially considering the potential for serious complications related to their presence in the heart.
Hormonal Influence: There is some evidence suggesting a potential hormonal influence on the development of cardiac myxomas, particularly in cases associated with Carney complex. However, the precise mechanisms by which hormones may contribute to myxoma formation still need to be fully elucidated.
Genetic Factors: While most cardiac myxomas are sporadic, a small percentage is associated with genetic syndromes, the most notable being Carney complex. Carney complex is a rare, autosomal dominant disorder characterized by multiple cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity.
Multipotent Mesenchymal Cells: It is believed that cardiac myxomas may originate from multipotent mesenchymal cells, which have the potential to differentiate into various cell types. The transformation of these cells into myxomas is thought to contribute to the development of the tumor.
Cellular Abnormalities: Molecular studies have indicated that certain cellular abnormalities, including mutations in genes such as PRKAR1A, may play a role in the pathogenesis of cardiac myxomas. However, the significance of these genetic alterations in sporadic cases remains an area of ongoing research.
The clinical manifestation of cardiac myxomas is contingent upon factors such as their location, size, and mobility, often characterized by the triad of embolization, intracardiac obstruction, and constitutional symptoms. These tumors are frequently linked to constitutional symptoms that mimic those seen in inflammatory or connective tissue disorders.
The prevalence of these symptoms tends to be higher in women compared to men, in cases of right-sided myxomas as opposed to left-sided ones, and in instances where the myxomas are large or multicentric. Common constitutional symptoms include malaise, anorexia, fever, arthralgia, and weight loss.
This range of clinical presentations underscores the variable nature of cardiac myxomas. It emphasizes the importance of considering factors such as tumor characteristics and patient demographics in understanding their clinical impact.
A triad of embolic manifestations, intracardiac obstruction, and constitutional symptoms characterize cardiac myxomas. However, due to variations in size, location, histopathology, and morphology, they may stay completely asymptomatic, present with classical indications, or give rise to life-threatening emergencies such as systemic embolization or sudden cardiac death.
During a general examination, observable features may include fever, cachexia, clubbing, cyanosis, or rash. Engorged neck veins and a prominent A wave in the jugular venous pulse may be evident. Precordial results may mimic mitral stenosis, with a loud and widely split first heart sound (S1) due to delayed mitral valve closure caused by tumor prolapse into the mitral valve orifice.
The pulmonary element of the second heart sound (P2) may vary in intensity based on the presence of pulmonary hypertension. A distinctive “tumor plop,” a low-pitched premature diastolic sound following S2, may be discerned, resembling the opening snap of rheumatic mitral stenosis, albeit at a different pitch.
Additionally, audible sounds may include a third heart sound (S3), a fourth heart sound (S4), tricuspid stenosis, or a diastolic murmur indicative of functional mitral. In some cases, a systolic murmur associated with mitral or tricuspid regurgitation may be present. Notably, the auscultatory results of cardiac myxomas exhibit characteristic changes with alterations in the patient’s position.
Fibroelastoma
Papillary Fibroelastoma
Infective Endocarditis
Cardiac Thrombus
Peri-operative management
Diagnosis is typically made using imaging studies, with echocardiography being the primary diagnostic tool. Other imaging modalities, such as cardiac MRI or CT scans, may be employed for further evaluation. The goal is to accurately determine the location, size, and characteristics of the myxoma.
A careful excision of the tumor is performed to ensure complete removal while minimizing the risk of embolization or damage to surrounding structures. In some cases, minimally invasive approaches may be considered, especially for small, accessible myxomas. After surgical removal, close monitoring is essential to assess cardiac function and detect any potential complications.
Anticoagulation may be initiated in some cases to prevent thromboembolic events, especially if there is evidence of embolization before surgery. Symptomatic relief measures may be initiated as needed, especially for constitutional symptoms such as fever, malaise, or weight loss. Regular follow-up visits are important to monitor for any signs of recurrence or the development of new myxomas.
Genetic Counseling
In cases where cardiac myxomas are associated with genetic syndromes, genetic counseling may be recommended for the patient and their family members to assess the risk of recurrence and associated conditions.
medtigo
Cardiac myxoma
Updated :
June 20, 2024
Cardiac myxomas represent prevalent primary neoplasms of the heart. Despite their biological benignity, they are considered “functionally malignant” due to their capacity for embolization. While their most frequent origin is the left atrium, no heart chamber is exempt from their occurrence.
Although predominantly sporadic, cardiac myxomas can also manifest as part of familial cases, particularly in the context of the Carney complex. Cardiac myxomas are often found in the left atrium of the heart, attached to the wall that separates the left and right sides of the heart (interatrial septum). However, they can also occur in the right atrium.
These tumors are composed of connective tissue cells and mucous-like material. The mucous material gives them a gelatinous appearance. While cardiac myxomas are often considered sporadic and not linked to specific risk factors, there may be a connection with certain genetic conditions in some cases.
Cardiac myxomas can occur at any age, but they are most commonly diagnosed in adults between the ages of 30 and 60. They are rare in children. The majority of cardiac myxomas are found in the left atrium, typically attached to the interatrial septum. About 75-80% of myxomas occur in the left atrium. Right atrial myxomas are less common but can also occur.
Recurrence of cardiac myxomas after surgical removal is uncommon but can occur, particularly in cases associated with genetic syndromes. In some cases, cardiac myxomas may be associated with genetic syndromes such as Carney complex, a rare, autosomal dominant disorder characterized by cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity. However, the majority of cardiac myxomas are sporadic.
The pathophysiology of cardiac myxoma involves the development of a benign tumor within the heart, most commonly located in the left atrium. These tumors are characterized by a gelatinous and mucoid consistency, composed of stellate-shaped cells embedded in a matrix of myxoid material. While the exact etiology remains unclear, some cases of cardiac myxomas are associated with genetic syndromes such as Carney complex.
The tumors may originate from multipotent mesenchymal cells, and their growth within the heart can lead to various clinical manifestations. The myxomas often attach to the interatrial septum and can obstruct blood flow, causing symptoms such as dyspnea, chest pain, and palpitations. Additionally, fragments of the tumor can break off, leading to embolism in distant organs.
The tumor’s presence can interfere with normal cardiac function, causing hemodynamic disturbances. Overall, while cardiac myxomas are rare, understanding their pathophysiology is crucial for timely diagnosis and appropriate management, especially considering the potential for serious complications related to their presence in the heart.
Hormonal Influence: There is some evidence suggesting a potential hormonal influence on the development of cardiac myxomas, particularly in cases associated with Carney complex. However, the precise mechanisms by which hormones may contribute to myxoma formation still need to be fully elucidated.
Genetic Factors: While most cardiac myxomas are sporadic, a small percentage is associated with genetic syndromes, the most notable being Carney complex. Carney complex is a rare, autosomal dominant disorder characterized by multiple cardiac and extracardiac myxomas, spotty skin pigmentation, and endocrine overactivity.
Multipotent Mesenchymal Cells: It is believed that cardiac myxomas may originate from multipotent mesenchymal cells, which have the potential to differentiate into various cell types. The transformation of these cells into myxomas is thought to contribute to the development of the tumor.
Cellular Abnormalities: Molecular studies have indicated that certain cellular abnormalities, including mutations in genes such as PRKAR1A, may play a role in the pathogenesis of cardiac myxomas. However, the significance of these genetic alterations in sporadic cases remains an area of ongoing research.
The clinical manifestation of cardiac myxomas is contingent upon factors such as their location, size, and mobility, often characterized by the triad of embolization, intracardiac obstruction, and constitutional symptoms. These tumors are frequently linked to constitutional symptoms that mimic those seen in inflammatory or connective tissue disorders.
The prevalence of these symptoms tends to be higher in women compared to men, in cases of right-sided myxomas as opposed to left-sided ones, and in instances where the myxomas are large or multicentric. Common constitutional symptoms include malaise, anorexia, fever, arthralgia, and weight loss.
This range of clinical presentations underscores the variable nature of cardiac myxomas. It emphasizes the importance of considering factors such as tumor characteristics and patient demographics in understanding their clinical impact.
A triad of embolic manifestations, intracardiac obstruction, and constitutional symptoms characterize cardiac myxomas. However, due to variations in size, location, histopathology, and morphology, they may stay completely asymptomatic, present with classical indications, or give rise to life-threatening emergencies such as systemic embolization or sudden cardiac death.
During a general examination, observable features may include fever, cachexia, clubbing, cyanosis, or rash. Engorged neck veins and a prominent A wave in the jugular venous pulse may be evident. Precordial results may mimic mitral stenosis, with a loud and widely split first heart sound (S1) due to delayed mitral valve closure caused by tumor prolapse into the mitral valve orifice.
The pulmonary element of the second heart sound (P2) may vary in intensity based on the presence of pulmonary hypertension. A distinctive “tumor plop,” a low-pitched premature diastolic sound following S2, may be discerned, resembling the opening snap of rheumatic mitral stenosis, albeit at a different pitch.
Additionally, audible sounds may include a third heart sound (S3), a fourth heart sound (S4), tricuspid stenosis, or a diastolic murmur indicative of functional mitral. In some cases, a systolic murmur associated with mitral or tricuspid regurgitation may be present. Notably, the auscultatory results of cardiac myxomas exhibit characteristic changes with alterations in the patient’s position.
Fibroelastoma
Papillary Fibroelastoma
Infective Endocarditis
Cardiac Thrombus
Peri-operative management
Diagnosis is typically made using imaging studies, with echocardiography being the primary diagnostic tool. Other imaging modalities, such as cardiac MRI or CT scans, may be employed for further evaluation. The goal is to accurately determine the location, size, and characteristics of the myxoma.
A careful excision of the tumor is performed to ensure complete removal while minimizing the risk of embolization or damage to surrounding structures. In some cases, minimally invasive approaches may be considered, especially for small, accessible myxomas. After surgical removal, close monitoring is essential to assess cardiac function and detect any potential complications.
Anticoagulation may be initiated in some cases to prevent thromboembolic events, especially if there is evidence of embolization before surgery. Symptomatic relief measures may be initiated as needed, especially for constitutional symptoms such as fever, malaise, or weight loss. Regular follow-up visits are important to monitor for any signs of recurrence or the development of new myxomas.
Genetic Counseling
In cases where cardiac myxomas are associated with genetic syndromes, genetic counseling may be recommended for the patient and their family members to assess the risk of recurrence and associated conditions.
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