Massive right atrial myxoma causing exertional dyspnoea
1 Department of Cardiology, University Hospitals Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry CV2 2DX, UK
2 Department of Cardiothoracic Surgery, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
Received 23 March 2007; accepted after revision 15 April 2007; online publish-ahead-of-print 25 June 2007.
* Corresponding author. Tel: +44 2476964000; fax: +44 2476965657. E-mail address: rajbilku{at}hotmail.com
 |
Abstract |
|---|
 Top Abstract DiscussionSupplementary materialReferences |
|---|
Metastatic tumours are the commonest cardiac tumours being found in 1–3% of patients dying of cancer while primary tumours are unusual and have an incidence of 0.02–0.5%. The majority (80%) of all primary cardiac tumours are benign with myxomas accounting for 50%. Myxomas arising from the right atrium are uncommon.
We present the case of a 39-year-old female with a 4-month history of progressive exertional dyspnoea accompanied by symptoms of palpitations and presyncope. Transthoracic echocardiography showed an extremely large right atrial myxoma prolapsing into the right ventricle and obstructing the tricuspid valve. We demonstrate how intraoperative transoesophageal echocardiography, prior to sternotomy, was useful in providing information about the myxoma which clearly displayed its attachment and anatomical relationship in the planning of the ‘safe’ surgical excision.
Keywords: Right atrial myxomas; Transoesophageal echocardiography; Transthoracic; Cardiac mass
A 39-year-old lady presented with a 4-month history of progressive exertional breathlessness with gradual reduction in exercise tolerance accompanied by palpitations, pre-syncope and symptoms of NYHA class II/III heart failure with no orthopnoea or chest pains. Clinical examination revealed hypertension with BP 160/110, a resting tachycardia of 110 beats per minute, raised JVP, audible first and second heart sounds with an opening snap and a rumbling mid-diastolic murmur heard loudest in the tricuspid region. Her chest was clear. She had 2 cm hepatomegaly and mild bilateral pedal oedema. Interestingly, her heart rate varied dramatically as she moved from a supine to upright posture, accompanied by sudden breathlessness. An ECG showed tall P-waves in lead II with T wave inversion in leads V1–V3. Urgent transthoracic echocardiograhy (see Figure 1) showed a massive intra-cardiac mass occupying the entire right atrium and prolapsing through the tricuspid valve into a non-dilated right ventricle causing ‘functional’ tricuspid stenosis. The IVC was dilated with minimal collapse on inspiration suggesting raised right atrial pressure. The left atrium, left ventricle, aortic valve, mitral valve and pulmonary valve all appeared normal. She was admitted by the cardiologists and referred for urgent surgery which was undertaken the following day. Following induction of anaesthesia, transoesophageal echocardiography (TOE) was undertaken. This revealed an extensive myxoma occupying the entirety of the right ventricle and prolapsing out into the pulmonary artery (see Figure 2). There were no other intra-cardiac abnormalities. At surgery, examination of the heart revealed an extensively dilated right atrium. Cardiopulmonary bypass was instituted with bicaval cannulation returning blood to the ascending aorta. Both of the cavae were snared, a longitudinal right atriotomy performed and the myxoma delivered into the right atrium. The stalk appeared to originate from the septum between the coronary sinus and the tricuspid annulus (Figure 3). This was at the inferior limbus of the fossa ovalis. The myxoma was excised along with the stalk. Tricuspid valve annuloplasty was performed for annular dilatation prior to closure of the right atriotomy. The patient was then weaned off cardiopulmonary bypass and the chest closed routinely.
Pathologically, the macroscopic specimen showed a nodular outer surface measuring 80 mm by 50 mm by 34 mm and contained haemorrhagic areas. The histology confirmed this to be a benign myxoma with no residual tumour at the base of the stalk. Clinically, at 6 weeks follow-up, her exertional capacity was much improved compared to her preoperative state and examination revealed she was in sinus rhythm with no audible murmurs.
|
|
|
|
Discussion |
|---|
|
TopAbstractDiscussionSupplementary materialReferences |
|---|
There are two types of cardiac myxomas, the friable polypoid type and the smooth-surface rounded type.1 The polypoidal type is associated with a higher incidence of embolism because of its obvious fragility,2,3 and tumour size also appears to be a major risk factor.4,5 Our patient had no evidence to suggest that she had suffered from a pulmonary embolism pre-operatively but recurrent pulmonary embolism secondary to right atrial myxoma has been described previously.6 Her massive right atrial myxoma by prolapsing through and obstructing the tricuspid valve caused a ‘functional’ tricuspid stenosis as evidenced by auscultation of the opening snap and mid diastolic murmur in the tricuspid region. Also, by occupying a significant volume of the right ventricle during diastole, the myxoma caused significant reduction in right ventricular inflow with consequent reduction in the stroke volume ejected into the RVOT. This explained our patient's resting tachycardia, palpitations and pre-syncopal symptoms that varied with posture. Catastrophic complications are known to occur during induction of anaesthesia and sternotomy.3,7 Echocardiography established the diagnosis and urgency of surgical treatment. In particular, TOE was extremely important and valuable in providing information about the myxoma by clearly displaying its attachment and anatomical relationship. TOE should be performed prior to sternotomy as part of the planning in how to safely perform the surgical excision.
|
Supplementary material |
|---|
|
TopAbstractDiscussionSupplementary materialReferences |
|---|
Supplementary material associated with this article can be found in the online version.
|
References |
|---|
|
TopAbstractDiscussionSupplementary materialReferences |
|---|
- McAllister HA Jr, Fenoglio JJ Jr. Myxoma. In: Tumors of the Cardiovascular System (1977) 2nd ed. Washington, DC: Armed Forces Institute of Pathology. 5–20.
- Burke AP, Virmani R. Tumors and tumor like conditions of the heart. In. In: Cardiovascular Pathology—Silver MD, Gotlieb AI, Schoen FJ, eds. (2001) 3rd ed. Philadelphia: Churchill Livingstone. 583–605.
- Panwar S, Banerjee A, Mohan JC, Tomar AS. Aortic leaflet injury caused by left ventricular myxoma: a hitherto unreported association. Indian Heart J (2000) 52:328–30.[Medline]
- Endo A, Ohtahara A, Kinugawa T, Mori M, Fujimoto Y, Yoshida A, et al. Characteristics of 161 patients with cardiac tumors diagnosed during 1993 and 1994 in Japan. Am J Cardiol (1997) 79:1708–11.[CrossRef][Web of Science][Medline]
- Chakfe N, Kretz JG, Valentin P, Geny B, Petit H, Popescu S, et al. Clinical presentation and treatment options for mitral valve myxoma. Ann Thorac Surg (1997) 64:872–77.
[Abstract/Free Full Text] - Jardine DL, Lamont DL. Right atrial myxoma mistaken for recurrent pulmonary thromboembolism. Heart (1997) 78:512–4.
[Abstract/Free Full Text] - Mukadam ME, Kulkarni HL, Kumar CJ, Tendolkar AG. Right ventricular myxoma presenting as right-ventricular outflow-tract obstruction: case report and review of the literature. Thorac Cardiovasc Surg (1994) 42.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  M. A.R. Chamsi-Pasha, A. M. Anwar, Y. F.M. Nosir, W. Abukhudair, A. Ashmeg, and H. Chamsi-Pasha Right atrial myxoma associated with an atrial septal defect by real-time three-dimensional echocardiography Eur J Echocardiogr, March 1, 2009; 10(2): 362 - 364. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||