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30-year-old otherwise healthy woman presents to her physician with complaints of fatigue and dyspnea. Physical examination reveals normal breath sounds and the presence of third and fourth heart sounds. Chest x-ray shows clear lung fields but right ventricular enlargement, main pulmonary artery enlargement, and "pruning" of the peripheral vasculature. Electrocardiogram shows right axis deviation and right ventricular hypertrophy. Left ventricular function appears normal on echocardiography. Serologic studies show antinuclear antibodies. Which of the following pathological findings would this patient also show, either at autopsy or if an appropriate biopsy was taken?


A. Mural thrombus of the right atrium

B. Necrosis and scarring of the left ventricle

C. Plexogenic pulmonary vasculopathy

D. Pulmonary artery stenosis

E. Severe pulmonary fibrosis

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plexogenic pulmonary vasculopathy. This rare condition is suspected of being related to the collagen vascular diseases.Both primary and secondary forms of pulmonary hypertension are associated with prominent changes in the pulmonary vasculature, which can include muscularization of smaller arterioles, concentric hypertrophy of the intima ("onion skinning"), and a distinctive plexiform lesion (plexogenic pulmonary vasculopathy) in which the smallest arterioles become markedly dilated with lumens partially occluded by endothelial (or possibly mesenchymal) cells and sometimes, thrombus.

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