Severe pulmonary hypertension with arterial calcification

Presentation

Down's syndrome, congenital heart disease and Eisenmenger 's syndrome

Patient Data

Age: 30
Gender: Male

Dilated pulmonary arteries and cardiomegaly noted, unchanged.  Calcified densities project over the lung apices and right lower zone peribronchial thickening (possibly representing brochiectasis) are also unchanged, with lungs otherwise clear.  No suspicious bone lesion.

In the upper mediastinum, the oesophagus is dilated but not dilated in the mid and distal oesophagus and no cause for this oesophageal dilatation is identified. The mediastinum is otherwise normal as is both pleural spaces. There is gross cardiac enlargement with changes of an enormous pulmonary outflow tract and grossly enlarged pulmonary arteries bilaterally with calcification in the pulmonary arteries and plethoric lungs right down to the lung bases bilaterally. These changes of severe pulmonary arterial hypertension would be in keeping with a non-intracardiac shunt and now the established presence of Eisenmenger's syndrome.

There is no evidence of aspiration pneumonia. No mass lesion seen. Large pulmonary arteries are noted. There is some bronchial wall thickening in both lung bases particularly at the left bases but the bronchi are not frankly bronchiectatic. There are no other abnormal features.

Conclusion

  1. Changes of severe pulmonary arterial hypertension noted, pulmonary arteries are calcified.
  2. There is gross cardiac enlargement.
  3. There are changes of pulmonary plethora bilaterally.
  4. Bronchial wall thickening but no frank bronchiectatic changes identified.
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Case information

rID: 21991
Case created: 3rd Mar 2013
Last edited: 24th Jan 2016
System: Vascular
Inclusion in quiz mode: Included

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