Abstract
Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH) characterized by post-capillary pulmonary venular obstruction, resulting in pulmonary vascular congestion, right heart failure, and death. PVOD represents an uncommon yet devastating complication of hematopoietic stem cell transplantation (HSCT) that provides several diagnostic challenges. While the actual incidence of PVOD is unknown, the disease may frequently go unrecognized ante mortem. Although the etiology and natural history of PVOD are poorly understood, the disease shares many pathophysiologic features of vascular injury associated with more common complications of HSCT, such as acute lung injury (ALI) and hepatic venoocclusive disease (HVOD). Treatment options for PVOD are limited and have critical implications unique from other forms of treatment for PAH. Given limited therapeutic options and rapid advancement of disease, PVOD portends a poor prognosis.
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Bunte, M.C., Burns, L.J. (2011). Pulmonary Venoocclusive Disease Following Hematopoietic Stem Cell Transplantation. In: Azoulay, E. (eds) Pulmonary Involvement in Patients with Hematological Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-15742-4_37
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