In this study, we used Foxf1WT/S52F mice which recapitulate both genetic and pathological findings in ACDMPV patients14, to identify the impairment of BMP9/ACVRL1 signaling in Foxf1WT/S52F EPCs. This evidence concerns the gene GDF2 and alveolar capillary dysplasia with misalignment of pulmonary veins.