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European Journal of Echocardiography Advance Access originally published online on February 7, 2008
European Journal of Echocardiography 2008 9(4):577-578; doi:10.1093/ejechocard/jen069
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Non-Hodgkin's lymphoma causing extrinsic pulmonary artery compression

Timothy Robinson*, Jane Lynch and Ever Grech

Department of Cardiology, Northern General Hospital, Sheffield Teaching Hospitals NHS Trust, Herries Road, Sheffield, South Yorkshire S5 7AU, UK

Received 17 September 2007; accepted after revision 13 January 2008; online publish-ahead-of-print 7 February 2008.

* Corresponding author. Tel: +44 114 2434343; fax: +44 114 2714084. E-mail address: tim.robinson{at}sth.nhs.uk


   Abstract
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Acquired pulmonary stenosis in adults is rare and is usually caused by extrinsic compression from a mediastinal tumour. We present the case of a patient with high grade non-Hodgkin's lymphoma who presented with progressive exertional dyspnoea. Compression of the right pulmonary artery was diagnosed by transthoracic echocardiography.

Keywords: Lymphoma; Non-Hodgkin; Pulmonary artery; Echocardiography

A previously healthy 23-year-old panel beater present with a 3-month history of progressive dyspnoea on exertion. He was on no medication. Physical examination revealed a loud, grade 3/4 ejection systolic murmur in the pulmonary area, radiating over the upper right posterior chest wall. The rest of the examination was unremarkable. The resting 12-lead ECG was normal.

Chest X-ray demonstrated a superior mediastinal mass (Figure 1). Transthoracic echocardiography (TTE) revealed a mildly dilated main pulmonary artery, with turbulent flow at the level of the bifurcation. Both left and right pulmonary arteries appeared narrowed. An increased velocity of 3.5 m/s (peak gradient 45–55 mmHg) was documented within the proximal right pulmonary artery (Figure 2, see Supplementary data online, Movie Clip 1).


Figure 1
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  		Figure 1 Chest X-ray demonstrating widening of the superior mediastinum consistent with a mass.

 


Figure 2
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  		Figure 2 TTE image demonstrating increased velocity in the right pulmonary artery.

 
A subsequent CT thorax confirmed an extensive soft tissue mass encasing the great vessels with compression of the right pulmonary artery consistent with a malignant neoplasm (Figure 3). A biopsy confirmed a high-grade non-Hodgkin's lymphoma (NHL). An appropriate course of chemotherapy led to a decrease in tumour bulk (Figure 4) and improvement in his dyspnoea.


Figure 3
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  		Figure 3 CT thorax showing compression of the right pulmonary artery (white arrow).

 


Figure 4
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  		Figure 4 CT thorax following chemotherapy showing decrease in tumour bulk and relief of pulmonary artery compression.

 

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Acquired pulmonary stenosis in adults is rare and is usually caused by extrinsic pulmonary artery or right ventricular outflow tract compression by tumour. Mediastinal lymphoma can compress vascular structures, such as the thoracic aorta and superior vena cava.1 However, it is uncommon for mediastinal lymphoma to compress the heart or pulmonary arteries sufficiently to produce murmurs or evidence of haemodynamically significant obstruction. This may be due to the tendency for mediastinal tumours to enlarge laterally, rather than antero-posteriorly.2 The commonest presenting complaints are chest pain (69%) and dyspnoea (60%), with an ejection systolic murmur occurring in 81%. Causative mechanisms are anterior mediastinal tumours, aortic aneurysms, mediastinal cysts, sternal tumours, and fibrosing mediastinitis. In a series of case reports, NHL was responsible for only 9% of cases of extrinsic pulmonary artery compression by tumour.3 The prognostic significance of acquired pulmonary stenosis in this setting is unknown.4

We have demonstrated the utility of TTE as a useful non-invasive method for the diagnosis of extrinsic compression of the pulmonary arteries. Further evaluation requires other imaging modalities and tissue diagnosis.


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Supplementary data are available at European Journal of Echocardiography online.

Conflict of interest: none declared.


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  1. Faunce HF, Ramsay GC, Sy W. Protracted yet variable pulmonary artery compression in sarcoidosis. Radiology (1976) 119:313–4.[Abstract]
  2. Waldhausen JA, Lambardo CR, Morrow AG. Pulmonic stenosis due to compression of the pulmonary artery by an intrapericardial tumour. J Thor Surg (1957) 37:679–86.
  3. Marshall ME, Trump DL. Acquired extrinsic pulmonic stenosis caused by mediastinal tumours. Cancer (1982) 49:1496–9.[Medline]
  4. Mandysova E, Neuzil P, Niederle P, Behlolavek O, Kozak T, Madys V. Pulmonary stenosis caused by external compression of non-Hodgkin lymphoma. Echocardiography (2004) 21:565–7.[CrossRef][Web of Science][Medline]

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This Article
Right arrow Abstract Freely available
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