European Journal of Echocardiography 2008 9(1):184-185; doi:10.1016/j.euje.2007.07.009
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Lymphoma presenting as an ejection systolic murmur
D.B. McKenzie*,
V. Khanna,
R.D. Proctor and
J.P. Boullin
Wessex Cardiothoracic Centre, Southampton General Hospital, Southampton University Hospitals NHS Trust, East Wing, Level E, Tremona Road, Southampton, SO16 5YA, United Kingdom
Received 24 June 2007; accepted after revision 29 July 2007; online publish-ahead-of-print 14 September 2007.
* Corresponding author: Tel: +44 7801 562183; fax: +44 1983 862245. E-mail address: dan{at}mckenzie0512.freeserve.co.uk
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Abstract
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We report the case of a 22-year-old gentleman with non-Hodgkin's
lymphoma who presented with a 3-week history of non-specific
symptoms and an ejection systolic murmur. Urgent transthoracic
echocardiography demonstrated a large anterior mediastinal mass
compressing the right ventricular outflow tract that was confirmed
on subsequent computerised tomography.
Keywords: Mediastinal mass; Lymphoma; Right ventricular outflow tract; Echocardiography
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Case report
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A 22-year-old university student presented with a 3-week history
of malaise, productive cough and exertional breathlessness.
His general practitioner had treated him with antibiotics for
a presumed chest infection. He then developed central pleuritic
chest pain, associated with sweating and feeling light-headed,
prompting his presentation to the Accident and Emergency Department.
On examination he was thin, pale and afebrile. His pulse was
110 bpm with a blood pressure of 110/55 mmHg. He had normal
heart sounds with an ejection systolic murmur, loudest at the
upper left sternal edge, with no radiation to the carotids.
He had clear lung fields with no lymphadenopathy or testicular
masses. A 12-lead ECG demonstrated sinus tachycardia, but was
otherwise normal.
An urgent transthoracic echocardiogram (Philips IE33) was performed for suspected infective endocarditis. This demonstrated a large extra-cardiac mass compressing the right ventricular outflow tract (RVOT). The peak gradient using continuous wave Doppler in the RVOT was 27 mmHg (Figures 1 and 2). There was a small pericardial effusion of no haemodynamic significance. A posterior—anterior (PA) and lateral chest radiograph confirmed a large anterior mediastinal mass (Figure 3). A spiral CT angiogram with contrast demonstrated that the mass filled the mediastinal space, displacing the heart and great vessels posteriorly (Figure 4). It also confirmed compression of the pulmonary trunk and the small pericardial effusion. Subsequent biopsy confirmed non-Hodgkin's lymphoma (mediastinal large B-cell), for which the patient was initially treated with chemotherapy and is currently receiving radiotherapy.
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Figure 4 A spiral thoracic computerised tomography angiogram with contrast demonstrating the large mediastinal mass displacing the heart and great vessels posteriorly, and compressing the pulmonary trunk. LPA = left pulmonary artery. RPA = right pulmonary artery.
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Discussion
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Clinically detectable pulmonic stenosis resulting from compression
of the pulmonary artery or right ventricular outflow tract is
rare.
1,2 It is uncommon for mediastinal masses to compress the
heart or pulmonary artery sufficiently to produce murmurs or
haemodynamically important obstruction.
3,4 This is thought to
be due to the tendency of mediastinal tumours to enlarge laterally
and produce superior vena cava obstruction instead of extrinsic
compression of the heart.
Non-Hodgkin's lymphoma is a very rare cause of right ventricular outflow tract obstruction confined to a few case reports over the past forty years.3–7 Some authors advocate the use of echocardiography for subsequent follow-up of these patients,2,6 though many institutions, including ours, tend to use other imaging modalities, such as CT or magnetic resonance imaging (MRI) for disease follow-up.
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Supplementary material
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Supplementary data associated with this article can be found in the online version.
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References
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- Marshall ME, Trump DL. Acquired extrinsic pulmonic stenosis caused by mediastinal tumors. Cancer (1982) 49:1496–99.[Medline]
- Visseur P, Unger P. Doppler echocardiographic diagnosis and follow-up of acquired pulmonary stenosis due to external cardiac compression. Cardiology (1995) 86:80–82.[Web of Science][Medline]
- Shaver VC, Bailey WR, Marragoni AG. Acquired pulmonary stenosis due to external cardiac compression. Am J Cardiol (1965) 16:256–61.[CrossRef][Web of Science][Medline]
- Gough JH, Gold RG, Gibson RV. Acquired pulmonary stenosis and pulmonary artery compression. Thorax (1967) 22:358–67.[Abstract/Free Full Text]
- Shields JJ, Cho KJ, Geisurger KR. Pulmonary artery constriction by mediastinal lymphoma simulating pulmonary embolus. Am J Roentgenol (1980) 135:147–50.[Abstract]
- Putterman C, Gilon D, Uretzki G, Bar-Ziv J, Polliack A. Right ventricular outflow tract obstruction due to extrinsic compression by non-Hodgkin's lymphoma: Importance of echocardiographic diagnosis and follow up. Leuk Lymphoma (1992) 7:211–15.[CrossRef][Web of Science][Medline]
- Mandysova E, Neuzil P, Niederle P, Belohlavek O, Kozak T, Mandys V. Pulmonary stenosis caused by external compression of non-Hodgkin lymphoma. Echocardiography (2004) 21:565–67.[CrossRef][Web of Science][Medline]
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