TY - JOUR
T1 - Pulmonary veno-occlusive disease
T2 - illustrative cases and literature review
AU - Lechartier, Benoit
AU - Boucly, Athénaïs
AU - Solinas, Sabina
AU - Gopalan, Deepa
AU - Dorfmüller, Peter
AU - Radonic, Teodora
AU - Sitbon, Olivier
AU - Montani, David
N1 - Publisher Copyright: © The authors 2024.
PY - 2024/1/31
Y1 - 2024/1/31
N2 - Pulmonary veno-occlusive disease (PVOD), also known as “pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement”, is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, leading to increased pulmonary vascular resistance and right ventricular failure. Environmental risk factors have been associated with the development of PVOD, such as occupational exposure to organic solvents and chemotherapy, notably mitomycin. PVOD may also be associated with a mutation in the EIF2AK4 gene in heritable forms of disease. Distinguishing PVOD from PAH is critical for guiding appropriate management. Chest computed tomography typically displays interlobular septal thickening, ground-glass opacities and mediastinal lymphadenopathy. Life-threatening pulmonary oedema is a complication of pulmonary vasodilator therapy that can occur with any class of PAH drugs in PVOD. Early referral to a lung transplant centre is essential due to the poor response to therapy when compared with other forms of PAH. Histopathological analysis of lung explants reveals microvascular remodelling with typical fibrous veno-occlusive lesions. This review covers the main features distinguishing PVOD from PAH and two clinical cases that illustrate the challenges of PVOD management.
AB - Pulmonary veno-occlusive disease (PVOD), also known as “pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement”, is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, leading to increased pulmonary vascular resistance and right ventricular failure. Environmental risk factors have been associated with the development of PVOD, such as occupational exposure to organic solvents and chemotherapy, notably mitomycin. PVOD may also be associated with a mutation in the EIF2AK4 gene in heritable forms of disease. Distinguishing PVOD from PAH is critical for guiding appropriate management. Chest computed tomography typically displays interlobular septal thickening, ground-glass opacities and mediastinal lymphadenopathy. Life-threatening pulmonary oedema is a complication of pulmonary vasodilator therapy that can occur with any class of PAH drugs in PVOD. Early referral to a lung transplant centre is essential due to the poor response to therapy when compared with other forms of PAH. Histopathological analysis of lung explants reveals microvascular remodelling with typical fibrous veno-occlusive lesions. This review covers the main features distinguishing PVOD from PAH and two clinical cases that illustrate the challenges of PVOD management.
UR - http://www.scopus.com/inward/record.url?scp=85182762312&partnerID=8YFLogxK
U2 - https://doi.org/10.1183/16000617.0156-2023
DO - https://doi.org/10.1183/16000617.0156-2023
M3 - Review article
C2 - 38232988
SN - 0905-9180
VL - 33
JO - European Respiratory Review
JF - European Respiratory Review
IS - 171
M1 - 230156
ER -