PMC7097520_01
PA and lateral view of the chest demonstrates post-operative changes of bilateral lung transplant and mild interstitial pulmonary edema. There is small left pleural effusion.
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Chest radiograph (Fig. 1) shows mild interstitial pulmonary edema and small left pleural effusion.
Representative coronal contrast CT images on the day of 50 status post-transplant ((a) and (b)), demonstrating complete occlusion of the superior pulmonary veins bilaterally. In comparison with similar coronal images on the day of 16 status post-transplant ((c) and (d)), on which showing both superior pulmonary veins are patent.
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Mediastinal window chest CT demonstrates new occlusion of the bilateral superior pulmonary veins in comparison with prior contrast HRCT (Fig. 2).
Representative high-resolution axial CT lung window images though the upper and lower lobes on the 50 days status post-transplant. It demonstrates ground glass opacities and interlobular septa and fissure thickening, suggestive of pulmonary edema.
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Lung window images (Fig. 3) show slightly respiratory phase with geographic mosaic attenuation, mild ground glass opacities and interlobular septa thickening.
Trans-esophageal echocardiogram demonstrates echogenic thrombus within the superior pulmonary vein.
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Trans-esophageal echocardiogram is performed, intraluminal echogenic thrombus is demonstrated in the expected location of the superior pulmonary veins (Fig. 4) bilaterally.
(a) In veno-occlusive disease, thickening of the small blood vessels and venules in the pleura and subpleural space is a common histologic feature. Proliferation of small capillaries within the alveolar septa is highlighted with CD31 immunostaining, consistent with hemangiomatosis ((b)- H&E stain; (c) - CD31 immunohistochemistry of the same area). Often, hemangiomatosis is more evident adjacent to interlobar septum, as shown here.
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The pathological microscopic images of H&E stain and CD31 immunohistochemistry (Fig. 5) demonstrates thickening of the small blood vessels/venules in the pleura and subpleural space, as well as alveolar septa small capillary proliferation, compatible with PVOD/PCH.
(a) Delayed phase of right superior pulmonary angiogram demonstrates patent pulmonary artery and significant stenosis/occlusion of the right superior pulmonary vein. (b) Fluoroscopic image status post right superior pulmonary vein stent placement.
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Since there is no definite optimal treatment of PVOD/PCH in this newly lung transplant patient, the decision from the multidisciplinary team meeting was made to proceed with a pulmonary venous thrombectomy and stent the occluded superior pulmonary vein via interventional access to the left atrium via trans-septal puncture from right heart (Fig. 6).