The development of pulmonary hypertension (PH) during the course of COPD is a well-known phenomenon, with the prevalence depending on the severity of airway obstruction. When mean pulmonary pressure (mPAP) level at rest is ≥ 35 mm Hg or ≥ 25 mm Hg with low cardiac index, the term severe PH is used. For these patients, little is known on the underlying histologic lesions. Our objective was to describe these lesions. From the explants of patients undergoing lung transplantation, we compared retrospectively three groups of patients with COPD: severe PH-COPD (n = 10), moderate PH-COPD (mPAP between 25 and 34 mm Hg without low cardiac index) (n = 10), and no PH (mPAP < 25 mm Hg) (n = 10). Histologic analysis of the explanted lungs examined the wall of medium-size arteries, the remodeling of microvessels, and the pulmonary capillary density using morphometric measurements performed on three sections per patient. Compared with the moderate PH group, the remodeling score of the microvessels was significantly higher (P = .0045) and the capillary density was lower (P = .0049) in the severe PH-COPD group. The alterations of the medium-size arteries, important in group 1 PH, seemed less discriminating. Patients with severe PH-COPD appear to have a specific histologic pattern, different from that observed in patients with COPD with moderate PH or without PH.