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Karkout, Amrani, and Jamil: Atrial myxoma – the first case report in Emirati patients


Myxomas are round or oval tumours with a smooth or lobulated surface that tend to be on short stalks and mobile in nature. They predominantly obstruct blood flow, causing congestive cardiac failure, with possible cranial or peripheral emboli dislodgement. Other signs and symptoms, such as fever, weight loss, shortness of breath, atypical chest pain and palpitations, indicate the presence of a myxoma. They represent almost half of all benign heart tumours, occur mainly in middle-aged men and rarely recur after excision, except in cases of familial inheritance.

History of atrial myxomas at a glance

  • 1559 Realdo Colombo became the first physician to describe a benign heart tumour.1,2

  • 1845 King described six cases of benign tumours, which are now referred to as myxomas.3,4

  • 1953 James Gibbon introduced the idea of cardiopulmonary bypass (CPB).5

  • 1954 Crafoord performed the first removal of an atrial myxoma (AM) using CPB.6

  • 1960 Sixty cases of myxoma removal were reported.7

Case 1

A 56-year-old Emirati woman with obstructive sleep apnoea, Graves’ disease and vitamin D deficiency presented to the Tawam Hospital cardiology clinic in 2014 with nocturnal choking spells, daytime somnolence, shortness of breath and chest tightness. A Persantine nuclear stress test was performed as part of the investigations and showed mild to moderate and fixed hypoperfusion involving the septal and apical regions (Figure 1). At rest the computed left ventricular ejection fraction (LVEF) was 40%, that is, abnormal. The patient was referred for transthoracic echocardiography, which showed a 2.3 × 2.0 cm solitary mass in the left atrium, attached to the intra-atrial septum. Transoesophageal echocardiography (TEE) was performed and showed an isolated left atrial mass of 2.3 × 1.9 cm; the left and right ventricles were of normal size and systolic function was also normal (Figure 2). A cardiac myxoma was, therefore, suspected and cardiac magnetic resonance imaging (MRI) undertaken, confirming our suspicions. The patient underwent cardiac catheterization prior to surgery, which was completely normal, after which surgical resection of the left atrial myxoma was performed. Post-surgery echocardiography showed mild mitral regurgitation with improved LVEF (55%), moderate tricuspid regurgitation and no residual mass. One year later the patient was doing well and complained only of mild on-and-off palpitations; she was referred for cardiac rehabilitation.


Myocardial perfusion scan.


Transoesophageal echocardiogram.


Case 2

A 61-year-old Emirati man with fibromyalgia, diabetes mellitus type 2 on treatment and previous nephrectomy presented to our emergency department (ED) with pleuritic chest pain and shortness of breath that had been present for several months. He was investigated for a suspected pulmonary embolism, but a ventilation–perfusion scan ruled this out. Radiography showed bilateral infiltration and the patient was admitted to the intensive care unit with acute respiratory distress, and was started on antibiotics, non-invasive mechanical ventilation and inotropes. Perfusion lung scintigraphy was performed and showed non-conclusive diffuse hypoperfusion; therefore, a ventilation study was not performed. The cardiology team was consulted after finding an abnormal murmur suggestive of mitral stenosis. Echocardiography was performed and showed a left atrial mass floating inside the mitral valve and obliterating it; however, systolic function was preserved. TEE was then performed and confirmed the presence of a large left atrial mass (Figure 3) (4 × 4 cm, Figure 4) obstructing the mitral valve and leading to a severe functional stenosis. Severe pulmonary hypertension was also noted (120 mmHg). The patient requested transfer to the military hospital for further workup and treatment. It was recommended that coronary angiography be performed before attempting surgical excision of the left myxoma.


Transoesophageal echocardiogram.


The excised tumour.



Myxomas are the most common benign cardiac tumours, mostly occurring between the ages of 30 and 60 years.8,40 They arise from the endocardium and are derived from a subendocardial multipotent cell. Only 15% of myxomas occur in the right atrium, whereas 75% occur in the left atrium and are attached to fossa ovalis.9 The remainder occur in the left and right ventricles (< 2%).10 About 90% of myxomas are solitary, and approximately 5% of cases show autosomal dominant (AD) inheritance. They are usually found in a young population and have a recurrence rate of about 20–65%.1113 Carney complex is characterized by myxomas that can occur in the heart, skin and breast, as well as other sites, with associated primary pigmented nodular adrenocortical disease, psammomatous melanotic schwannomas, growth hormone-producing pituitary adenomas, testicular Sertoli cell tumours and other tumours. It shows AD inheritance and is an example of familial cardiac myxomas.14

Cardiac MRI is a useful tool to distinguish myxomas from other intracavitary masses in the heart. On T1-weighted imaging they appear isointense and heterogeneous, whereas on T2 they appear hyperintense and heterogeneous. Post contrast they show heterogeneous enhancement. However, cardiac MRI15,16 is not used as a definitive tool to determine therapy and is always preceded by echocardiography. Tissue biopsy is the best modality for diagnosis and TEE remains helpful in ruling out other intracardiac tumours intraoperatively, as reported by the DeBakey Centre case series.8

Symptomatic myxomas are normally > 5 cm in diameter, weigh > 70 g and are usually located in the left atrium, whereas right atrial myxomas need to grow to almost twice this size before they become symptomatic. Around 30–40% of patients have an embolic event at different sites, depending on the location of the myxoma, particularly those with polypoid tumours.1720 Up to 19% of patients with large myxomas (> 5 cm) have atrial fibrillation (AF).

Lijoi et al.21 reported a case series of operated myxomas, with different results, as shown in Table 1. Myxomas are vascular and can become neovascularized through coronary branches, and therefore they have the potential to bleed within the heart chambers.22 This phenomenon is produced by angiogenesis as a result of the production of vascular endothelial growth factor and expression of interleukin 6.2325 Preoperative cardiac angiography may be helpful to identify neovascularization; however, since the invention of echocardiography, cardiac angiography has seldom been used as a diagnostic tool.26 Histologically, the excised cells appear polygonal with eosinophilic cytoplasm, associated with calcification in up to 20% of cases. Tumour necrosis is found in 8% of cases. Cells are usually lipidic in a vascular stroma and mitosis is normally absent.27 Interleukin 6 expression was reported in 74% of 37 cases presented by Acebo et al.28


Case series by Lijoi et al. 199321

Outcome n Comments
 6 days 1 Left ventricular myxoma, multiple cerebral emboli 6 days post surgery
 3 years 4 Colon cancer/lung cancer/traffic accident/myocardial infarction
 Atrial fibrillation 9 Eight converted with medical therapy, one did not
 First degree heart block 5 One had pacemaker inserted 6 months post-operatively
 Hemiplegia 1
 Heart failure 4 New York Heart Association functional class II

Total excised: 26; total survived after 3 years: 20.

Surgery is the usual treatment,29 as non-surgical treatment is reserved for possible pre- or post-excision complications. Post-operative mortality is < 7%30 and post-operative morbidity is variable. The most common side-effect from surgery is AF, occurring in up to 33% of cases.31 Pinede et al.32 reported < 4% mortality in case series of 112 patients over 3 years’ follow-up. Recurrence of the tumour predominantly occurs in familial cases, in cases of incomplete excision and when the location of the primary myxoma is atypical. The rate is estimated to be up to 5%, being lower after the fourth year following surgery.33 Wide surgical resection is rarely required except for large myxomas, and is usually avoided by destruction of pre-tumorous cells around the stalk using photocoagulation. In 2007, Deshpande et al.34 reported 23 cases of endoscopic cardiac resection of myxomas using port access. This procedure had a better cosmetic effect and minimal complications at follow-up. In 2011, Patil et al.35 published a single-centre study of 62 patients who underwent excision of a cardiac myxoma. The survival rate was reported to be 96.8%, with no reoperation needed in 98% of patients at 5 years and 96.8% at 10 years.

Atrial myxoma in the United Arab Emirates

We have found only one case report36 of AM in the United Arab Emirates (UAE); however, this was in a non-local patient. A 47-year-old man with an insignificant medical history was reported to have a syncopal attack for a few minutes followed by unilateral numbness and weakness. Echocardiography showed a 6.9 × 2.4 cm mobile mass in the left arterial septum, sparing the anterior mitral leaflet. TEE was performed and confirmed the findings; surgical excision was then carried out to remove the 20-g mass.

Atrial myxoma in the Gulf region

In the Gulf region, as elsewhere, AM is usually diagnosed after the patient has developed a complication. However, as a result of the development of a health-care system, diagnosis is now made earlier and surgery is successful in most cases. Surgeons are now performing successful resections without the need to send the patients abroad, as they had done previously. While reviewing the literature, most local case reports came from Saudi Arabia and Kuwait, with fewer cases reported from the UAE, Qatar, Bahrain and Oman. Post-operative complication rates have been identical to global rates, and patients were comfortable to proceed with treatment locally. There are no clear published data about the rates of inheritance of the disease in this area. All reported cases were sporadic without a family history of diagnosed AM.

Recently, a case of myxomatous embolic involvement of the coronary arteries leading to a myocardial infarction was reported from Saudi Arabia.37 The English-language literature was reviewed and 16 cases were reported about myocardial infarctions associated with myxomas between 2003 and 2013;37 however, the paper concluded that coronary arteries are usually normal in patients who have an AM and presenting with myocardial infarction, and it should therefore always be suspected and ruled out to avoid misdiagnosis.

Another study from Saudi Arabia looked at the link between cardiac myxomas and immunosuppression.38 A small number of cases of myxomas in patients receiving immunosuppressive therapy have been reported worldwide. Lymphoproliferative and squamous cell malignancies are more common, but myxomas do occur and should be considered in the differential diagnosis. The paper reviewed four case reports about this link; in all cases the patient had been treated with cyclosporine. However, the authors recommended further studies to confirm any correlation between cyclosporine, interleukins 6 and 8 and AM.

In a third case, from King Faisal Specialist Hospital and Research Centre, Saudi Arabia,39 an AM was removed from a 3-month pregnant woman presenting with a transient ischaemic attack and who was found to have multiple emboli in the middle cerebral artery. Histological examination confirmed the type of the tumour, surgery was successful and the patient’s recovery was smooth. No adverse effects on the fetus were reported.

In the same hospital, it was found that 30% of patients with cardiac myxomas have neurological symptoms secondary to embolic phenomena.40 In one case,41 a 67-year-old woman received emergency cardiac surgery after developing aphasia and right-side hemiparesis. She was known to have a myxoma after confirmation of multiple emboli on magnetic resonance angiography, hence the decision for the urgent surgery. The operation was successful and the patient recovered well.

The final case report, from King Fahed Medical City, Saudi Arabia,42 described a 51-year-old woman presenting with blindness and unilateral body weakness. Transthoracic echocardiography and MRI showed a mobile mass, consistent with left atrial myxoma.42

Many cases have been well reported from Kuwait. In one case series completed by Kuwait University over 14 years,43 12 patients were diagnosed with AM. The majority were initially misdiagnosed as having valvular heart disease; however, echocardiography confirmed the diagnosis of myxoma in all. Surgical excision of the tumour was carried out in 10 patients. One patient underwent mitral valve repair surgery but died post-operatively as a result of a coronary embolism and reoperation. One patient refused surgery. Of the 10 surviving patients who had operations, nine remain asymptomatic and in sinus rhythm, whereas one has progressive mitral regurgitation and cardiac failure. The conclusion was that tumour excision under TEE guidance should be carried out shortly after diagnosis to reduce mortality and morbidity rates. It has been shown that even ED bedside echocardiography is useful in ruling out cardiac masses in patients with cardiac or neurological symptoms and, hence, ED physicians should have a high index of suspicion for this easily treated tumour soon after patient arrives in the ED.44

In a final reported case from Oman,45 a 54-year-old asymptomatic man, electrocardiographic changes suggestive of an old inferior infarct were identified during pre-operative investigations for eye surgery. Chest X-ray radiography revealed cardiomegaly and TEE confirmed a left atrial mass, consistent with myxoma. Surgical excision was performed and a 47-g mass was removed.

All cases described above are summarised in Table 2.


Summary of cases reported in the Gulf

Case Patient information Presentation Diagnosis Treatment
UAE Dubai (2015)36 Male, aged 41 years Syncope, numbness and weakness Chest radiography (cardiomegaly), echocardiography, TEE, mass 6.9 × 2.4 cm Surgical, 20-g mass removed
King Fahd University, Saudi Arabia (2014)37 Male, aged 22 years Chest pain, palpitations, inferior MI Electrocardiogram (Q waves), echocardiography, mass 25 × 1.8 cm Surgical, unknown weight
King Saud University, Saudi Arabia (2012)38 Male, aged 71 years, renal transplant (17 years previously), immunosuppressed (cyclosporine/azathioprine/prednisone) Recurrent TIAs, right MCA emboli, palpitations Head computerized tomography, echocardiography, mass 3.58 × 1.95 cm Conservative
Prince Salman Centre, Saudi Arabia (2010)39 Female, aged 35 years, pregnant (3 months) TIA, right-side weakness, MCA emboli Head MRI, echocardiography, unknown size Surgical, unknown weight
King Faisal Centre, Saudi Arabia (2013)41 Female, aged 67 years, known myxoma Aphasia, right-side weakness Magnetic resonance angiography (emboli), echocardiography, unknown size Emergency surgery, unknown weight
King Fahd University, Saudi Arabia (2014)42 Female, aged 51 years, hypertension and diabetes mellitus Left eye central retinal artery occlusion, right-side weakness MRI (ischaemia LMCA and ACA), echocardiography, TEE, cardiac MRI (T1 and T2), unknown size Surgical, unknown weight
Muscat, Oman (2011)45 Male, aged 54 years, diabetes mellitus and DLP Asymptomatic, pre-operative assessment Echocardiogram (LAD, LVH, old inferior MI), chest radiography (cardiomegaly), TEE, mass 5 × 3.5 cm Surgical, 47-g mass removed
Kuwait (1996)43 12 patients Cardiac murmurs Echocardiography Surgical in 10 patients, one patient mitral valve repair (died of embolism), one patient refused surgery, one patient had post-surgical mitral regurgitation

ACA, anterior cerebral artery; DLP, dyslipidaemia; IMI, myocardial infarction; LAD, left anterior descending artery; LMCA, left main coronary artery; LVH, left ventricular hypertrophy; MCA, middle cerebral artery; TIA, transient ischaemic attack.

Conclusion and recommendations

  • Primary cardiac tumours are very rare and AM is the most common benign cardiac tumour, easily picked up by conventional two-dimensional echocardiography. Cardiac MRI is a helpful tool to detect the size and mobility of the tumour, whereas tissue diagnosis is the best tool to differentiate between different cardiac tumours.

  • Surgery is the only treatment and should be carried out once diagnosis is made to prevent fatal events, such as cardioembolic phenomenon.

  • Familial myxoma shows AD inheritance and familial cases are approximately 10–15 times more common than sporadic ones, necessitating screening of family members when an index case is identified.

  • Complications, most commonly AF and congestive heart failure, are rarer than in the past owing to advances in diagnosis and treatment modalities.

  • Medical therapy is successful in treatment of most complications. The prognosis is excellent and most patients have a healthy, complication-free life post excision.

  • AMs are increasingly being diagnosed in the Gulf region; however, better training to diagnose and treat this type of tumour earlier is required and should extend to ED physicians, internal medicine specialists, cardiologists and cardiothoracic surgeons.



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