Presentation
Long clinical history of exertional dyspnea, fatigue, and sporadic chest pain.
Patient Data
Diagnostic opacification of the pulmonary arteries without direct signs of pulmonary embolism up to a subsegmental level. No webs or strictures. The pulmonary artery is dilated in keeping with pulmonary hypertension, with the trunk measuring 3.1 cm in diameter. The right ventricle is dilated, small pericardial effusion. Small bilateral hilar and mediastinal lymph nodes without worrisome features.
There are widespread centrilobular ground-glass nodules with uniform distribution throughout both lungs. No pleural effusion.
Given the severity of her symptoms, she was later offered bilateral lung transplantation.
Macroscopy: Left and right native lung - Left lung weighing 300 g measuring 180 x 150 x 75 mm. Pleura appears pale tan/grey and mottled with areas of hemorrhage, and smooth texture. Bronchovascular resection margins are clear, with hilar lymph nodes appear carbon pigmented. On sectioning, parenchyma appears pale tan/cream and mottled with a mildly firmer texture, with a subpleural (apical)/patchy distribution. Elsewhere parenchyma appears pale tan and spongy. No lesions are identified. No thrombi were seen within vessels.
Right lung weighing 350 g measuring 185 x 150 x 75 mm. Pleura appears pale tan/cream and mottled with focal areas of hemorrhage and overall smooth texture. On sectioning, parenchyma appears predominantly modelled cream/tan with a mildly firmer texture. Within the middle/upper lobes there is residual pale tan spongy parenchyma. No lesions are identified. No thrombi were seen within vessels.
Microscopy: Sections from both specimens show lung tissue with a preserved architecture. There is patchy focal recent intra-alveolar hemorrhage. Centrilobular arteries show concentric thickening of their muscular walls, with a marked reduction in luminal diameter. Interlobar septa contain patent veins, without evidence of fibrous venous obliteration. Atheroma formation is present within large arteries. There is no arteritis. There is mild patchy interstitial chronic inflammation with minor interstitial fibrous expansion in a peribronchiolar distribution. Hilar lymph nodes show reactive sinus histiocytosis. There are no granulomas. No hyaline membranes are seen. There is no capillary haemangiomatosis. There is no malignancy.
Conclusion: Left and right native lungs, explants: Pulmonary arterial hypertension with mild peribronchiolar interstitial pneumonitis.