FIGURE 6.

Pathogenesis of vascular remodelling in pulmonary veno-occlusive disease (PVOD). 1) Marked fibrosis of the intima and hypertrophy of the media lead to occlusion of the pulmonary postcapillary vasculature. 2) Venous occlusions cause increasing blood pressure in the pulmonary capillaries (*). Pressure overload induces excessive neoangiogenesis by sprouting and intussusceptive pillar formation likely driven by increased flow and shear stress, which results in the formation of pulmonary capillary haemangiomatosis (PCH). 3) Congestion of the pulmonary capillary and postcapillary vasculature result in pulmonary hypertension associated with subsequent sclerotic remodelling of pulmonary arteries and arterioles. Reproduced from [67] with permission.