Download Massive right atrial myxoma causing exertional dyspnoea

European Journal of Echocardiography (2008) 9, 130–132
doi:10.1016/j.euje.2007.04.009
Massive right atrial myxoma causing exertional dyspnoea
R.S. Bilku1*, M. Loubani2, M. Been1, and R.L. Patel2
1
Department of Cardiology, University Hospitals Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry
CV2 2DX, UK; and 2Department 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
KEYWORDS
Right atrial myxomas;
Transoesophageal
echocardiography;
Transthoracic;
Cardiac mass
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.
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
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E-mail address: [email protected]
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
There are two types of cardiac myxomas, the friable polypoid type and the smooth-surface rounded type.1 The
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2007.
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Right atrial myxoma causing exertional dyspnoea
131
Figure 1 Transthoracic echocardiogram showing extremely large right atrial myxoma. (A) Parasternal long axis view in systole when the
mitral valve is closed showing no visible mass in the right ventricle. When the atrioventricular valves are open in diastole, there is prolapse
of the right atrial myxoma into the right ventricle as seen in the parasternal long axis view (B) and parasternal LV short axis view (C). Apical
four-chamber view in (D) shows the extent of the size of the myxoma occupying almost the entire volume of a dilated right atrium. AMVL,
anterior mitral valve leaflet; Asc Ao, ascending aorta; AV, aortic valve; IVS, interventricular septum; LA, left atrium; LV, left ventricle; MV,
mitral valve; Myx, myxoma; RA, right atrium; RV, right ventricle.
Figure 2 Intraoperative transoesophageal echocardiogram showing the large right atrial myxoma prolapsing into the right ventricle and
RVOT in the mid-oesophageal AV short axis views (A) and (B), respectively. Transgastric LV short axis view (C) and transgastric RV inflow
view (D) show the myxoma occupying an extensive volume of the RV in diastole and prolapsing into the RVOT, thus impairing RV inflow
and hence reducing stroke volume ejected into the RVOT . The stalk of the myxoma appears to originate from an area of the interatrial
septum between the coronary sinus and tricuspid valve annulus (E). Ant, anterior LV wall; AV, aortic valve; CS, coronary sinus; Inf, inferior
LV wall; IVS, interventricular septum; LA, left atrium; LV, left ventricle; Myx, myxoma; RA, right atrium; RCC, right coronary cusp; RV,
right ventricle; RVOT, right ventricular outflow tract; TV, tricuspid valve.
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R.S. Bilku et al.
Figure 3 Intraoperative pictures of the right atrial myxoma demonstrating its attachment to the atrial septum near the coronary sinus
(A) with the dilated tricuspid valve annulus (B) and (C) the size of the myxoma following resection.
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
Supplementary material associated with this article can be
found in the online version.
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