Plexogenic arteriopathy

Case contributed by Rachael O'Rourke

Presentation

Chronic breathlessness on exertion due to idiopathic pulmonary hypertension which was diagnosed when she was 9 years old. Right ventricular systolic pressure (RVSP) 120 mmHg estimated on echo. On medical therapy with home oxygen. Presents for transplant assessment.

Patient Data

Age: 15 years
Gender: Female
x_ray

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

ct

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 may result in non-specific ground glass changes which can be incorrectly attributed to infection, inflammation or edema on CT chest. It is a finding more common in cases of idiopathic pulmonary hypertension, however other causes of pulmonary hypertension e.g. congenital shunt, can also show this finding. Features that confirm the ground glass opacities as plexiform arteriopathy include:

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