Giant right atrial myxoma presenting as right heart failure: a rare manifestation

Abstract

A 37-year-old man was presented in outpatient clinic of cardiology department with symptoms of easy fatigability and progressive increasing generalised anasarca since 5 months. Echocardiogram showed large mass of 9.8×7.8 cm in size in right atrium, attached to interatrial septum. Urgent opinion of thoracic surgeon was taken and surgical excision of mass under cardiopulmonary bypass was done. The tumour was large, fragile and histology confirmed it as myxoma. The patient made a good recovery and his symptoms resolved completely on follow-up.

Background

Primary cardiac tumours are very rare and cardiac myxoma is the most common primary benign tumour of heart. The most common site of cardiac myxoma in decreasing order is left atrium, right atrium, right ventricle, left ventricle and rarely from mitral or tricuspid valves.^{1 2} We are presenting a case of giant right atrial myxoma manifesting as right heart failure secondary to right ventricular inflow tract obstruction wherein symptoms improved completely after surgical resection. We have also discussed the various presentation of right atrial myxoma described in literature so far.

Case presentation

A fit-and-well 37-year-old labourer, with no significant previous medical history presented to outpatient clinic with easy fatigability associated with gradually increasing pedal oedema and abdominal distension since 5 months. There was no history of cardiac disease in family.

Vital signs were stable with a blood pressure of 110/80 mm Hg, pulse rate of 110 bpm, irregular rhythm with heart rate of 120 bpm, all peripheral pulses were present, respiratory rate of 22 breaths/min and temperature of 37.60°C. On physical examination, jugular venous pressure was raised 5 cm above angle of Louis with prominent v wave and Y descent. Bilateral pitting oedema up to mid thigh was noted. Auscultation of lungs revealed crepitations on right side. Cardiovascular examination was notable for a grade 2/6, pansystolic murmer at fourth left parasternal space suggestive of tricuspid regurgitation. On tilting towards right, tricuspid tumour plop was appreciated.

On abdominal examination, shifting dullness was there, however fluid thrill was absent. Liver and spleen were not enlarged.

Investigations

Laboratory findings revealed normocytic, normochromic anaemia (haemoglobin 10.2 g/dl; reference range 13–15 g/dL), raised erythrocyte sedimentation rate (ESR) (52 mm; reference range 0–10 mm), mildly raised aminotransferases (alanine aminotransferase (ALT) 80 U/L; reference range 7–56 U/L; aspartate aminotransferase (AST) 48 U/L; reference range 10–40 U/L) with normal electrolytes and kidney function tests.

ECG showed atrial fibrillation with fast ventricular rate of 120 bpm. Two-dimensional-transthoracic echocardiography (2D TTE) showed large 9.8×7.8 cm mass in the right atrium, attached to interatrial septum (IAS), filling the whole chamber with slight protrusion into septal tricuspid leaflet and right ventricle during diastole (figure 1A–C). Mild tricuspid valve regurgitation was noted. A diagnosis of right atrial myxoma was kept and urgent surgical consultation was taken.

Figure 1.

(A, B) Transthoracic echocardiography (apical four chamber and subcostal view) revealed a large right atrium myxoma (9.80×7.82 cm) attached to the interatrial septum of the right atrium and completely obliterating the right atrium. (C) Parasternal short axis view showing right atrial myxoma and relationship to left atrium (LA), aorta (Ao) and pulmonary artery (PA).

Differential diagnosis

The differential diagnosis of right atrial mass is thrombus, vegetation and tumour. Myxoma and fibroma are common right atrial tumour. In index patient, echocardiogram showed polypoid mass with smooth surface and a rough core including lucencies and cystic areas due to haemorrhage and necrosis favoured myxoma. Histopathology confirmed it further as myxoma (figure 2).

Figure 2.

H&E stained section (40× magnification) shows tumour cells arranged in loose clusters and scattered singly in a myxoid matrix.

Treatment

Following discussion with the thoracic surgeon and anaesthesiologist, excision of large mass obstructing the right ventricle outflow tract was deemed to be the treatment priority. The excision was performed through midline sternotomy under cardiopulmonary bypass using standard ascending aorta and bicaval cannulation. Access was through right atrium and after right atriotomy, complete excision of myxoma with excision of part of IAS was done.

Reconstruction of IAS was done using autologous untreated pericardial patch. The tumour was large, lobulated, having irregular surface with multiple polypoid areas, and spots of loose and friable tissue.

As planned, the patient was admitted to the intensive care unit following surgery and had an uneventful postoperative recovery. Atrial fibrillation was controlled with amiodarone infusion postoperatively. Prior to discharge, patient was started on oral beta blockers and anticoagulation with warfarin for atrial fibrillation.

Outcome and follow-up

At 3-month postsurgical resection, patient’s right heart failure symptoms of easy fatigability, pedal oedema and abdominal distension were resolved completely and he was able to return to work. His international normalised ratio) was kept in between 2 and 3.

Discussion

Primary cardiac neoplasms are rare than secondary cardiac neoplasms. Cardiac myxoma is the most common primary heart tumour where 75%–80% of cardiac myxomas are located in left atrium and 15%–20% of cardiac myxomas are present in right atrium.^{1 2} The right atrium myxomas usually originate in the fossa ovalis or base of the IAS. Myxomas are usually polypoid and pedunculated tumours.

Most of the myxomas are asymptomatic and discovered incidentally on routine 2D echocardiography. The right atrial myxomas sometimes present with obstructive features like dyspnoea, syncope, right side heart failure symptoms like ascites, pedal oedema. Patients may present with non-specific signs and symptoms like fever, weight loss, arthralgias, raynaud phenomenon, anaemia, hypergammaglobulinemia and an increase in ESR. In this report, patient presented with signs and symptoms of right heart failure with fever as constitutional symptom. Giant right atrial myxomas are unusual and there are very few case reports of large right atrium tumours (table 1).

Table 1.

Various presentations and diagnosis of right atrial myxoma

Author	Size of right atrial myxoma	Clinical presentation	Diagnosis
Frishman et al7	Described atypical right atrium myxoma of size 9×8×4 cm in a 57-year-old black woman.	Anasarca	Echocardiogram and histopathology confirmed it as atypical glandular variant of myxoma and patient made complete recovery after surgical excision.
Willey et al8	Described right atrial myxoma of size 9×8×6 cm in a 28-year-old woman.	Light headedness, sweating, breathlessness, tiredness	Histology confirmation of myxoma and patient made complete recovery after surgical excision.
Yazici et al9	Described right atrial myxoma of size 6.3×5.2 cm in a 65-year-old woman.	Syncope	Echocardiogram and histopathology confirmed it as myxoma and patient made good recovery after surgical excision.
Nina et al10	Described large right atrium myxoma of size 10 cm (lobulated mass) in a 45-year-old Caucasian.	Dyspnoea and weight loss	Echocardiogram followed by histopathological confirmation of myxoma and patient made complete recovery after surgical excision.
Strecker et al11	Described right atrial myxoma of size 41×46 mm in a 62-year-old patient.	Asymptomatic	Incidentally diagnosed in echocardiogram followed by histopathological confirmation of myxoma and patient made complete recovery after surgical excision.
Muthiah et al12	Described large RA myxoma in 12-year-old man of size 69.5×38.1 mm in 12-year-old patient.	Low grade fever, joint pains and weight loss	Echocardiogram and patient died due to syncope.
Wang et al13	Described three cases of myxoma, myxoma originating from annulus of tricuspid valve in one of the cases of size 3×2 cm in a 50-year-old woman.	Chills and fever	Echocardiogram followed by histopathological confirmation of myxoma.
Choi et al14	Described right atrial myxoma in 72-year-old woman of size 7×4 cm in a 72-year-old man.	Shortness of breath	Echocardiogram followed by histopathological confirmation of myxoma and patient made slow recovery in postoperative follow-up.

ALT, alanine aminotransferase; AST, aspartate aminotransferase; IAS, Interatrial septum; RA, right atrium.

2D TTE remains the best modality of choice for screening cardiac masses. A 2D echocardiogram usually demonstrates mass in atrium, with the stalk attached to the IAS. A transoesophageal echocardiography provides better delineation of tumour. Cardiac MRI and CT provide better delineation of intracardiac mass and its extent to surrounding structures which is helpful in preoperative planning.^{3 4}

The treatment of choice for myxomas is surgical removal. The results of surgical resection are very good with operative mortality of less than 5%. For the most part, surgical resection is completely curative but there are chances of recurrence of tumour especially in familial cases of myxomas. In some series, 5% of patients developed recurrent myxomas.^{5 6} Life-long follow-up is needed for postsurgery patients. Regular echocardiogram is required to look for recurrence. Prompt diagnosis and resection of these tumours is important to prevent complications secondary to embolism and sudden cardiac death.

Learning points.

• Right heart failure is commonly caused by volume overload conditions, pressure overload conditions, right ventricle infarction and sometimes intrinsic disease of tricuspid valve.

• Right atrial tumour, although are rare but treatable cause of right heart failure.

• Postoperative surveillance by echocardiography is required to look for recurrence of tumour.