Plexogenic arteriopathy

Case contributed by Dr Rachael O'Rourke


Long history of SOBOE due to idiopathic pulmonary hypertension diagnosed when 9 years old. RVSP 119 mmHg estimated on echo. On medical therapy with home oxygen. Presents for transplant assessment.

Patient Data

Age: 15
Gender: Female

Right heart enlargement with loss of the normal AP window. Dilated pulmonary arteries with pruning. 


Dilated pulmonary arteries relative to the aorta. Right ventricular dilation and hypertrophy with flattening of the interventricular septum. Dilated bronchial arteries.

Tortuous pulmonary arteries with increased peripheral vascularity and plexiform arteriopathy causing heterogenous, ground glass opacification centered around peripheral tortuous vessels.


Case Discussion

Plexiform arteriopathy can be mistaken for non specific ground glass changes incorrectly attributed to infection, inflammation or edema on routine CT chest imaging. It is a finding more common in cases of idiopathic pulmonary hypertension, however other causes of pulmonary hypertension eg. congenital shunt, can also exhibit this finding. Features that confirm the ground glass opacities as plexiform arteriopathy include:

  • close association with dilated, tortuous peripheral vessels
  • abnormal systemic collaterals
  • features of pulmonary hypertension on CT
    • dilated pulmonary trunk
    • peripheral pruning and tortuousity of pulmonary arteries
    • right ventricular dilation and/ or hypertrophy
    • interventricular septal flattening/ bowing
    • right atrial dilation
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Case information

rID: 44218
Published: 12th Apr 2016
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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