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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].

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]
Hiccup
  • The patient presented with a history of chronic hiccups and weight loss.[ncbi.nlm.nih.gov]
Heart Disease
  • Associated cardiac lesions included moderate and severe tricuspid regurgitation in four, coronary atherosclerotic heart disease in five, and pulmonary embolism in one.[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.[medlineplus.gov]
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]

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].

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: 2018-06-22 04:26