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Right ventricular outflow tract obstruction due to a mediastinal lymphoma
 
Published on 2013-12-01 by Gilbert Abi Nader
 
 
History and Findings
 

The case was compiled by Anna Marzaro, M.D.

Cardiology fellow at Middle East Instiute of Health, Bsalim

Lebanon

History:

A twenty year old man previously healthy presented for medical clearance before elective rhinoplasty.

Physical examination was remarkable for pallor and grade 2/ 6 crescendo decrescendo systolic murmur in the 2nd  intercostal space.

Lab test showed normocytic anemia with hemoglobin = 9.2 mg/dl

Chest x-ray showed an enlargement of the upper anterior mediastinum. The resting 12-leads ECG was within normal limits.        

Transthoracic two-dimensional echocardiography showed an extrinsic compression of the right ventricular outflow tract and main pulmonary artery (MPA) by a huge mediastinal mass (videos 1 and 2). There was near complete collapse of the PA in diastole. In systole there is partial collapse with a Continuous-wave Doppler demonstrated peak systolic pressure gradient of 40 mm Hg at the site of the MPA compression.

A subsequent CT chest showed a huge heterogeneous mediastinal mass partially necrotic measuring 9.6x13x10.5 encasing the great vessels with compression of the right pulmonary artery consistent with a malignant neoplasm.                                                                                                       Endoscopy  was performed and 2 additional lesions were found in the stomach which were biopsied. Pathology revealed large B cell lymphoma.

The radiotherapy department was consulted and treatment plan plus dosimetry was prepared for a conformal 3D radiotherapy in case of emergency. The patient was treated with the standard CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone).

 
 
 
video 1 mass and compressed RVOT | video 2 color flow of RVOT
 
 
   
 
 
Discussion
 

Discussion

Acquired pulmonary artery stenosis is rarely found in adults and is usually caused by extrinsic pulmonary artery or right ventricular outflow tract compression by tumor. Marshall et al reported that teratoma and Hodgkin’s lymphoma were most frequent. Primary mediastinal large B-cell non-Hodgkin’s lymphoma is a rare subtype of large B-cell non-Hodgkin’s lymphoma.

This rare type is reported to have involvements of the surrounding vessels in one-third of patients. 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 tumors to enlarge laterally, rather than antero-posteriorly.4

In a series of case reports, NHL was responsible for only 9% of cases of extrinsic pulmonary artery compression by tumour.1

The MPA has a large lumen that does not easily cause a stenosis with impeding blood flow.

Therefore, it is very rare that the MPA involvement impedes blood flow with significant haemodynamic impairment as seen in the present case.

 

REFERENCES

1.Marshall ME, Trump DL. Acquired extrinsic pulmonic stenosis caused by mediastinal tumors. Cancer1982;49:1496–9.

2.Popat U,Przepiork D, Champlin R,et al. High-dose chemotherapy for relapsed and refractory diffuse large B-cell lymphoma: mediastinal localization predicts for a favorable outcome. J Clin Oncol1998;16:63–9.

3.Robinson T,Lynch J, Grech E. Non-Hodgkin’s lymphoma causing extrinsic pulmonary artery compression. Eur J Echocardiogr2008;9:577–8  

4. Waldhausen JA, Lambardo CR, Morrow AG. Pulmonic stenosis due to compression of the pulmonary artery by an intrapericardial tumour. J Thor Surg 1957;37:679-8

 
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