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Right Atrial Myxoma

Myxoma is a primary tumor of the heart that develops in the right atrium in about 20% of cases, with a strong predilection toward females and a mean onset of symptoms around 50 years of age. Both sporadic and familial forms are recognized and the clinical presentation ranges from asymptomatic to pulmonary embolism and heart failure. Echocardiography is necessary for diagnosis, whereas surgical excision is the method of treatment.


Presentation

In early stages, an asymptomatic course is frequently noted, but as the tumor grows and obstructs normal blood flow through the chambers, various symptoms can appear. Malaise, chronic low-grade fever, arthralgia and weight loss are signs of reduced flow, whereas tricuspid valve obstruction caused by an right atrial myxoma can lead to hepatic congestion, ascites, peripheral edema and even heart failure [1]. In the setting of thromboembolic events, the clinical presentation can be identical to pulmonary embolism - chest pain, dyspnea, syncope, hemoptysis and pulmonary hypertension [4].

Fever of Unknown Origin
  • For a 68-year-old patient with recurrent fever of unknown origin, weight loss, exertional dyspnea, and tachycardia, the authors further describe epidemiology, clinical symptoms, prognosis, and the specific diagnostic and therapeutic challenges involved[ncbi.nlm.nih.gov]
Low Fever
  • The obstruction of blood flow leads to intermittent heart failure, and, similar to systemic non-specific flu-like malaise symptoms, there is usually a low fever of long duration, arthralgia, anorexia, and thromboembolic events [ 7 ].[jmedicalcasereports.biomedcentral.com]
Pleuritic Pain
  • Abstract A 47-year-old man was admitted to the hospital with a pleuritic pain, dyspnea, nonproductive cough and low-grade fever.[ncbi.nlm.nih.gov]
Jaundice
  • On further examination, it was revealed that he had mild tender hepatomegaly with jaundice. His blood analysis for HBsAg was positive.[ncbi.nlm.nih.gov]
Heart Disease
  • We are presenting a case of the right atrial myxoma found in a case of rheumatic heart disease.[ncbi.nlm.nih.gov]
  • Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 95. Mckenna WJ, Elliott P. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed.[medlineplus.gov]
  • Neoplastic heart diseases. En: Alexander RW, Schalnt RC, Fuster V, editores. Hurst's the heart. McGraw-Hill Inc., 1998;p. 2295-318. 2. Fitzpatrick A, Lanham J, Doyle D. Cardiac tumors simulating collagen vascular diseases. Br Heart J 1986;55:592-5.[revespcardiol.org]
  • Another 55 year old male, known case of diabetes, hypertension, ischemic heart disease and pulmonary tuberculosis was admitted with TEE showing a myxoma in the right atrium associated with moderate tricuspid and severe mitral regurgitation, and acute[apicareonline.com]
Tachycardia
  • An ECG documented a sinus tachycardia with S1Q3T3 pattern and incomplete right bundle branch block, and lung scintigraphy showed multiple perfusion defects. The initial diagnosis was pulmonary embolism.[ncbi.nlm.nih.gov]
  • She presented with supraventricular tachycardia during the operation. She was placed in the intensive care unit and her condition improved after the use of amiodarone. The diagnosis of myxoma was confirmed by histopathological study.[jmedicalcasereports.biomedcentral.com]
Diastolic Murmur
  • Cardiac auscultation revealed mid diastolic murmur at tricuspid area. Beside this other systemic examination were normal. His twelve lead electro cardiogram showed normal sinus rhythm.[austinpublishinggroup.com]
  • Physical examination can reveal a diastolic murmur and in some cases, a typical "tumor plop" caused by presence of the tumor in the atria may be heard, but to confirm the diagnosis, echocardiography, either transthoracic or transesophageal, is necessary[symptoma.com]
  • In cases where the tumour remains in the atrium throughout the cardiac cycle, a diastolic murmur and pressure tracings practically indistinguishable from those of mitral stenosis will likely be present.[atlasgeneticsoncology.org]
Left Ventricular Dysfunction
  • It is known that surgical management of such tumours is difficult regarding venous cannulation and embolic risk, but in our patient, the surgery was more challenging because of the severe left ventricular dysfunction.[ncbi.nlm.nih.gov]

Workup

Physical examination can reveal a diastolic murmur and in some cases, a typical "tumor plop" caused by presence of the tumor in the atria may be heard, but to confirm the diagnosis, echocardiography, either transthoracic or transesophageal, is necessary to perform [5]. A pedunculated, solitary lesion is identified in most cases, but rare description of multiple tumors have been documented, especially in familial forms [2]. Additional imaging studies, including computed tomography or magnetic resonance imaging, should be used for preoperative assessment [3].

Multiple Pulmonary Nodules
  • A chest x-ray image from a 48-year-old woman with shortness of breath revealed multiple pulmonary nodules, which suggested lung metastases. An FDG PET/CT was performed for further evaluation.[ncbi.nlm.nih.gov]
Decreased Platelet Count
  • Echocardiography, undertaken before application of the anticoagulant therapy because of hematological disturbances reflecting possible coagulopathy (elevated erythrocyte sedimentation rate, increased leukocyte count, decreased platelet count), revealed[ncbi.nlm.nih.gov]
Incomplete Right Bundle Branch Block
  • An ECG documented a sinus tachycardia with S1Q3T3 pattern and incomplete right bundle branch block, and lung scintigraphy showed multiple perfusion defects. The initial diagnosis was pulmonary embolism.[ncbi.nlm.nih.gov]

Treatment

Surgical excision of the tumor is the method of choice and various approaches have been tested with equal efficacy [5] [6]. Tumor removal results in cure and minimal rates of recurrence have been observed.

Prognosis

The prognosis is good with an early diagnosis and appropriate therapy, but because thromboembolic events may occur and lead to cardiac or respiratory failure, recognition of the disease is detrimental in reducing the risk of adverse events created by loose tumor fragments [4]. Current surgical methods show curative effects, but tumor recurrence ranges from 1-2% in sporadic forms to 12-22% in familial forms [3].

Etiology

The exact cause of atrial myxomas remain unknown, but it is known that the tumor arises from multipotent mesenchymal cells in the subendocardium [2]. Myxomas are sporadic benign tumors that appear as solitary lesions in the vast majority of cases, but in approximately 7% of individuals, a familial autosomal dominant disease called Carney complex (multiple myxomas, pigmented nevi and various endocrine abnormalities) may be the disorder responsible for their development [3]. Mutations of the tumor-suppressor gene PRKAR1A have been identified in familial cases, but the cause of sporadic forms remains unknown [3].

Epidemiology

Myxomas are very rarely encountered in medical practice, as an incidence of 0.0017-0.19% has been established, but they are termed to be the most common primary tumors of the heart [4]. The right atrium is the location of approximately 20% of myxomas and a significant gender predilection toward female gender is observed [2]. Patients of all ages may be affected, but the diagnosis is most commonly made between ages 30 and 60 [2].

Sex distribution
Age distribution

Pathophysiology

The pathogenesis model remains unclear, but studies have confirmed that secretion of interleukin-6 (IL-6, a pro-inflammatory cytokine) by the tumor triggers the appearance of symptoms such as fever, arthralgias and elevations of erythrocyte sedimentation rate (ESR) [4].

Prevention

Current prevention strategies do not exist as the mechanism of tumor development remains unknown.

Summary

Myxoma is a primary benign tumors of the heart that is extremely rare in clinical practice, with an estimated incidence of 0.0017% in the general population, but it is the most common primary tumor of the heart [1]. Derived from multipotent subendocardial mesenchymal cells, myxoma is a solitary tumor embedded in a glycosaminoglycan-rich stroma and its most frequent site of occurrence is the left atrium (75%), whereas 20% of tumors are found in the right atrium [2]. In most cases, myxomas arise sporadically and individuals of all ages may be affected, but they are most frequently diagnosed between 30-60 years of age [1] [2]. Some tumors, however, can be associated with a familial form of the disease called Carney complex, in which autosomal dominant pattern of inheritance is observed [1] [3]. A significant gender predilection toward women is seen in sporadic forms [4]. The clinical presentation depends on the size and position of the tumor and it is not uncommon for patients to be asymptomatic for a prolonged period of time. At some point, constitutional symptoms such as fever, weight loss, anemia and arthralgias appear, as well as dyspnea, chest pain, syncope and other symptoms mimicking pulmonary embolism due to obstruction by loose fragments of the tumor [4]. Auscultation can reveal a diastolic heart murmur, but to confirm the diagnosis, transthoracic or transesophageal echocardiography must be performed [5]. Surgical excision of the tumor is curative, but tumor recurrence is seen in approximately 1-2% of sporadic and 12-22% of familial cases [3]. Moreover, larger tumors may cause pulmonary hypertension, right heart failure or even death [4], which is why an early diagnosis is detrimental.

Patient Information

A myxoma is a very rare benign primary tumor of the heart, with its most common location being the left atrium (75%), whereas the tumor is found in the right atrium in about 20% of cases. Its cause remains unknown and the vast majority of individuals develop the tumor in a sporadic fashion, meaning that no preexisting inherited conditions have caused. In 7% of patients, however, a disorder called Carney complex is the underlying cause, with myxomas being a constitutive feature of this inherited disease. Right atrial myxoma is more frequently encountered in females and the diagnosis is most often made between 30-60 years of age. The tumor may be asymptomatic in its initial stages, but inevitable obstruction of blood flow through the heart chambers and detachment of tumor fragments that can cause pulmonary embolism occurs, giving rise to symptoms such as chest pain, dyspnea, brief loss of consciousness (known as syncope), swelling of the extremities, fever, profound fatigue and weight loss. The diagnosis can be suspected during physical examination, when a heart murmur can be heard during auscultation. To confirm atrial myxoma, however, it is necessary to perform an ultrasound of the heart (known as echocardiography) in order to visualize the tumor and exclude other diagnoses, primarily endocarditis. Surgical removal of the tumor is curative and recurrence rates are very small. Because atrial myxomas can lead to life-threatening heart failure and pulmonary hypertension, early recognition of the disease is necessary in ensuring adequate treatment and prevention of complications.

References

Article

  1. Nina VJ, Silva NA, Gaspar SF, et al. Atypical size and location of a right atrial myxoma open link: a case report. J Med Case Reports. 2012;6:26.
  2. Vroomen M, Houthuizen P, Khamooshian A, Soliman Hamad MA, van Straten AH. Long-term follow-up of 82 patients after surgical excision of atrial myxomas. Interact Cardiovasc Thorac Surg. 2015;21(2):183-188.
  3. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.
  4. Azevedo O, Almeida J, Nolasco T, et al. Massive right atrial myxoma presenting as syncope and exertional dyspnea: case report. Cardiovasc Ultrasound. 2010;8:23.
  5. Oliveira R, Branco L, Galrinho A, et al. Cardiac myxoma: a 13-year experience in echocardiographic diagnosis [in English and Portuguese]. Rev Port Cardiol. 2010;29:1087-1100.
  6. Kesavuori R, Raivio P, Jokinen JJ, Sahlman A, Vento A. Quality of life after robotically assisted atrial myxoma excision. J Robot Surg. 2015;9(3):235-241.

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Last updated: 2019-07-11 21:18