title: [Pulmonary hypertension in chronic lung diseases].
abstract: Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the .authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP]<25mmHg); COPD/IPF/CPFE with PH (mPAP25mmHg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP 35 mmHg or mPAP 25 mmHg with low cardiac index [CI <2.0.l/min/m2]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on this severe PH group, and for the time being, these patients should be transferred to expert centers for individualized patient care. (J Am Coll Cardiol 2013;62:D109-16) ©2013 by the American College of Cardiology Foundation.
authors.forename: Werner
authors.initials: W
authors.lastname: Seeger
authors.forename: Yochai
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authors.lastname: Adir
authors.forename: Joan Albert
authors.initials: JA
authors.lastname: Barberà
authors.forename: Hunter
authors.initials: H
authors.lastname: Champion
authors.forename: John Gerard
authors.initials: JG
authors.lastname: Coghlan
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Teresa
authors.initials: T
authors.lastname: De Marco
authors.forename: Nazzareno
authors.initials: N
authors.lastname: Galiè
authors.forename: Stefano
authors.initials: S
authors.lastname: Ghio
authors.forename: Simon
authors.initials: S
authors.lastname: Gibbs
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.forename: Marc J
authors.initials: MJ
authors.lastname: Semigran
authors.forename: Gerald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vachiéy