Presse Med 2014 Sep 11;43(9):945-56. Epub 2014 Aug 11.
Hospices civils de Lyon, hôpital Louis-Pradel, Centre national de référence des maladies pulmonaires rares, centre de compétences de l'hypertension pulmonaire, service de pneumologie, 69677 Lyon cedex, France; Université de Lyon, université Claude-Bernard - Lyon-1, INRA, UMR754, 69007 Lyon, France. Electronic address:
Pulmonary hypertension is frequent in advanced chronic respiratory diseases, with an estimated prevalence at the time of pulmonary transplantation of 30-50 % in idiopathic pulmonary fibrosis, 30-50 % in chronic obstructive pulmonary disease, 50 % in combined pulmonary fibrosis and emphysema, 75 % in sarcoidosis, and more than 75 % of cases in pulmonary Langerhans cell histiocytosis. Histologic features include varying degrees of pulmonary arterial remodeling (prominent), vascular rarefaction (emphysema), fibrosis or specific involvement of the pulmonary arteries (idiopathic pulmonary fibrosis, sarcoidosis, lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis), in situ thrombosis, and frequently associated involvement of the pulmonary veins (idiopathic pulmonary fibrosis, sarcoidosis). Pulmonary hypertension is usually detected using echocardiography with Doppler, however right heart catheterisation is required to confirm precapillary pulmonary hypertension defined by pulmonary artery pressure ≥ 25 mm Hg, with pulmonary artery wedge pressure ≤ 15 mm Hg. Read More