Pulmonary veno-occlusive disease (PVOD) and pulmonary arterial hypertension (PAH) are both classified clinically as group 1 pulmonary hypertension, with PVOD having a special designation as group 1′. It is important to differentiate these two conditions because of their vastly different treatment strategies and prognosis. While PVOD affects both genders equally, PAH has a female predilection. Both PVOD and PAH can be sporadic or familial. Biallelic mutations in translation initiation factor 2 alpha kinase 4 (EIF2AK4) are seen in all familial cases of PVOD. The radiographic appearance of the classical PVOD is characteristic but can be seen in PAH. Clinical features including symptoms, pulmonary function test, and cardiac catheterization are largely overlapping for PVOD and PAH. Histologically, PVOD is characterized by diffuse involvement of the venous system by intimal thickening leading to subtotal or complete venous occlusion, and PAH shows mainly arterial involvement with intimal/medial thickening and plexiform lesions in severe cases. Secondary capillary congestion and hemosiderin-laden macrophages are commonly seen in PVOD but not in PAH. Unlike PAH, there is no successful medication for PVOD, and the prognosis is dismal if lung transplantation is not performed rapidly.
- Pulmonary hypertension
- Pulmonary arterial hypertension
- Pulmonary veno-occlusive disease
- Pulmonary capillary hemangiomatosis
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Saeed, O.A.M., Zhang, C. (2022). Pulmonary Veno-Occlusive Disease Versus Pulmonary Arterial Hypertension. In: Xu, H., Ricciotti, R.W., Mantilla, J.G. (eds) Practical Lung Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-031-14402-8_57
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