case_images: PMC10978528_01
case_images: Axial view of computed tomography pulmonary angiography showing severe stenosis of the distal right pulmonary artery. Also in view is calcified mediastinal fibrosis, a calcified pulmonary mass, and a calcified pleural plaque.
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case_images: Class (NYHA FC) 2 symptoms and echocardiographic PH. 6-minute walk test (6MWT) distance at that time was 384 m with no oxygen requirement. Contrasted computed tomography (CT) of the chest showed multiple large calcified pulmonary nodules with bulky calcified mediastinal adenopathy and mediastinal fibrosis causing severe stenosis of the distal right main pulmonary artery (PA) and mild stenosis of the bronchus intermedius and right middle and lower lobe bronchi (Fig. 1).
case_images: Anterior projection perfusion scan showing perfusion defects in the region of the right middle and right lower lung zones.
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case_images: Ventilation/perfusion (V/Q) scan demonstrated a marked right middle and right lower lobe perfusion defect (Fig. 2).
case_images: PMC10978528_02
case_images: Coronal slice of computed tomography pulmonary angiography showing severe stenosis of the right pulmonary artery and mediastinal, hilar, and parenchymal fibrosis.
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case_images: Chest CT showed right mediastinal, hilar, and parenchymal fibrotic changes with near complete stenosis of the right sided pulmonary arterial system (Fig. 3).
case_images: Left anterior oblique perfusion scan showing nearly absent perfusion to the right lung.
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case_images: Perfusion scan estimated the right lung received only 3.5% of total pulmonary perfusion (Fig. 4).