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].

• Leg Edema

  A 52-year-old male presented with progressive dyspnea, bilateral leg edema, and elevated central venous pressure due to a large right atrial myxoma that caused vascular obstruction and pulmonary emboli. The myxoma contained gastric heterotopia. [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]

  Angiography was performed which revealed coronary artery disease. [apicareonline.com]

• Tachycardia

  Abstract A 65-year-old woman presented with shortness of breath, stenocardia, and tachycardia. She underwent several steps of examination. Echocardiography showed a suspicious formation in the right atrium extending into the inferior caval vein. [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]

  Auscultation can reveal a diastolic heart murmur, but to confirm the diagnosis, transthoracic or transesophageal echocardiography must be performed. [symptoma.com]

  A pulmonary ejection murmur with a delayed and accentuated pulmonic second sound may be heard. There may also be an early, late or prolonged diastolic murmur heard. [atlasgeneticsoncology.org]

• Cardiomegaly

  Abnormalities, if present, are non-specific and include: cardiomegaly : left atrial enlargement intracardiac tumoral calcification evidence of mitral valve obstruction evidence of elevated left atrial pressure, e.g. pulmonary edema Ultrasound Echocardiography [radiopaedia.org]

  Cardiomegaly due to RA and right ventricular enlargement was observed. During an anoxic arrest for 17 minutes with single aortic cross-clamping, the tumor was completely excised through a right longitudinal atriotomy. [jmedicalcasereports.biomedcentral.com]

  Chest Radiography Radiographic findings are non-specific in cases of cardiac myxoma, generally showing signs of pulmonary hypertension and congestion, with possible cardiomegaly. [atlasgeneticsoncology.org]

• Myalgia

  Case Report The patient, a 30 year old female presented to the out patient department with myalgia, arthralgia and low back ache. [ispub.com]

  Constitutional manifestations Constitutional manifestations occur in approximately 30% of cases of cardiac myxoma, and commonly appear as; - Fatigue - Fever - An erythematous rash - Arthralgia - Myalgia - Weight loss, and - Raynaud's phenomenon. [atlasgeneticsoncology.org]

• Prolapse

  Echocardiography revealed a large mass arising from the inferior vena cava and prolapsing into the right atrium and ventricle. Histopathologic examination revealed the classic features of a myxoma. [ncbi.nlm.nih.gov]

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].

• Pericardial Effusion

  It was visualized by transthoracic echocardiography and small and insignificant pericardial effusion was also seen. After surgical removal of the tumor, the patient remained without any symptoms and pericardial effusion. [ncbi.nlm.nih.gov]

  Postoperative complications occurred in 6 patients, including pericardial effusion requiring drainage and AKI managed conservatively with IV fluids and medications. [apicareonline.com]

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.

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].

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].

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].

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].

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

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.

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.

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.
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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.