title: [Pulmonary hypertension in COPD and interstitial lung diseases].
abstract: Pulmonary hypertension (PH) is a common complication of chronic obstructive pulmonary disease (COPD) and of interstitial lung diseases (ILD) such as idiopathic pulmonary fibrosis and sarcoidosis. When present in these patients, PH is usually mild to moderate. When severe PH is diagnosed in COPD and ILD patients, other potentially better treatable underlying causes should be ruled out. In COPD patients, PH is associated with an increased risk of severe exacerbations and a reduced life expectancy. Similarly, in patients with ILD, the presence of PH correlates with a poor prognosis. Doppler echocardiography is the best non-invasive method for the diagnosis of PH, but is frequently inaccurate in patients with advanced lung diseases. Thus, when clinical suspicion remains high, right heart catheterization in a reference center is required to ultimately confirm the presence of PH. Treatment of PH in COPD and ILD is primarily based on long term oxygen therapy. Drugs approved for pulmonary arterial hypertension, such as prostanoids, phosphodiesterase inhibitors, and endothelin receptor antagonists, may represent promising options for COPD and ILD patients, however, their use may be hampered by potentially deleterious effects on gas exchange and their efficacy yet remains to be proven in appropriately designed and controlled clinical trials. Lung transplantation may be considered in all patients with an advanced disease.
authors.affiliation: Zentrum für Innere Medizin, Medizinische Klinik II und Poliklinik, Universitätsklinikum Giessen und Marburg, Standort Giessen.
authors.forename: P
authors.initials: P
authors.lastname: Markart
authors.forename: H A
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: F
authors.initials: F
authors.lastname: Grimminger
authors.forename: A
authors.initials: A
authors.lastname: Günther
mesh_terms: D006328:Cardiac Catheterization; D015150:Echocardiography, Doppler; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D017563:Lung Diseases, Interstitial / Q000150:complications*; D016040:Lung Transplantation; D010102:Oxygen Inhalation Therapy; D010726:Phosphodiesterase Inhibitors / Q000009:adverse effects / Q000627:therapeutic use; D011379:Prognosis; D011453:Prostaglandins / Q000009:adverse effects / Q000627:therapeutic use; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications*; D011658:Pulmonary Fibrosis / Q000150:complications; D011659:Pulmonary Gas Exchange / Q000187:drug effects; D012307:Risk Factors; D017565:Sarcoidosis, Pulmonary / Q000150:complications
chemical_list: D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins