Figure 5.

A–D: the four types of plexiform lesions from four different patients with IPAH. PA indicates pulmonary artery, and the asterisks mark the lesions. The arrowheads in A and B indicate the monopodial branch leaving the parent artery. In D, the arrowhead indicates the open lumen distal to the obstruction. Supplemental Videos S2–S5 scan through the four different lesions in A–D. The scale bar in D (200 µm) is valid for A–D. E–H: all four types of plexiform lesions from the dye-injected lung from the BMPR2-mutation carrier. PA marks open lumen proximal to the plexiform lesion in all four images and the asterisks mark the lesions. The arrowhead in E indicates the monopodial branch leaving the parent artery. Scale bars in E–H = 200 µm. IPAH, idiopathic pulmonary arterial hypertension.