PMC8654615_01
Oblique-coronal maximum intensity projection (left), and angiography (right) demonstrate AVM's (circles).
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Thorax computed tomography scan (CT-scan) showed multiple pulmonary arteriovenous malformations (PAVM's), neovascularization, an inhomogeneous perfusion pattern, signs of pulmonary bleeding, diffuse ground-glass opacities, and thickened interlobar septal lines (Fig. 1).
Timeline. Course spanning 195 days.
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In the first month of dual therapy, the patient deteriorated with increased shortness of breath, more profound desaturation and a decline in exercise capacity (timeline shown in Fig. 2). At that moment sildenafil was stopped and not re-introduced (Fig. 2).
Lung tissue showing segmental capillary hyperproliferation at the right side of the slides (asterisk), bordering more normal parenchyma at the left side of the slides (hematoxylin-eosin staining magnification 100x (A) and CD34 positive staining magnification 50x (B) and 100x (C)).
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Furthermore, microscopic evaluation of lung tissue samples showed segmental capillary hyperproliferation, iron accumulation and segmental pulmonary venous obstruction, which are signs of pulmonary veno-occlusive disease (PVOD) including pulmonary capillary hemangiomatosis (PCH) (Fig. 3, Fig. 4).