35-year-old female with increasing dyspnoea. Right heart failure,
- Largely unremarkable lung parenchyma with main findings on arteries.
Changes in the vessels changes have been caused by pulmonary hypertension.
Muscular arteries (diameter 0.1-1 mm):
- Hyperplasia of media (corresponds to grade I of hypertensive lung
- Onion-bulb-like thickening of intima with varying proportion of
cells and fibres. Narrowing of lumen to the point of obliteration
(depending on number and extent of change grade II or II)
- Net-like division of lumen by cell-rich septa (plexiform lesions):
often in smaller, thin-walled and dilated side branches of largely
obliterated muscular arteries. Angioma-like increase of dilated
cavernous vessels (angiomatoid lesions, grade IV)
Arterioles (diameter <80 um): muscularisation, i.e continuous
elastic lamella and smooth muscle in the vessel wall.
Elastic arteries (diameter >1 mm): pulmonary sclerosis, i.e arteriosclerotic
changes comparable with lesions in the large cardiovascular system.
The diagnosis " plexogenous pulmonary arteriopathy" can
be made with the presence of plexiform lesions (Caution: can be
mistaken for organized emboli)
All given specimen show numerous hypertensive lesions up to grade
III. Plexiform lesions are not evident on all specimen as they are
Occurrence: in cardiac shunting, rarely in liver cirrhosis, in
some HIV-infected patients, idiopathic, in the 70s following intake
of the appetite suppressant Aminorex