ISSN 1016-5169 | E-ISSN 1308-4488
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Pulmonary hypertension in chronic lung diseases [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2014; 42(1): 142-152

Pulmonary hypertension in chronic lung diseases

Werner Seeger1, Yochai Adir2, Joan Albert Barberà3, Hunter Champion4, John Gerard Coghlan5, Vincent Cottin6, Teresa De Marco7, Nazzareno Galiè8, Stefano Ghio9, Simon Gibbs10, Fernando J. Martinez11, Marc J. Semigran12, Gerald Simonneau13, Athol U. Wells14, Jean-luc Vachiéry15
1Lung Center of Giessen and Marburg University (UGM LC), a member of the German Center for Lung Research, Max-Planck-Lung and Heart Research Institute, Giessen / Bad Nauheim, Germany
2Pulmonary Department, Lady Davis Carmel Medical Center, School of Medicine, The Technion Institute of Technology, Haifa, Israel
3University of Barcelona, Hospital Clinic-IDIBAPS Hospital, Respiratory Disease Network on Biomedical Research Center, Barcelona, Spain
4UPMC Montefiore Hospital, Pittsburgh, Pennsylvania
5Royal Free Hospital, Department of Cardiology, London, England
6Hospices Civils de Lyon, Louis Pradel Hospital, Respiratory Diseases, National Reference Center for Rare Pulmonary Diseases, Regional Competence Center for Severe Pulmonary Arterial Hypertension, University Claude Bernard Lyon 1, INRA, Lyon, France
7University of California, San Francisco, San Francisco, California, USA
8DIMES, Bologna University Hospital, Experimental, Diagnostic and Specialist Medical Department, Bologna, Italy
9Department of Cardiology, Division Fondazione IRCCS Policlinico di San Matteo, Pavia, Italy
10National Heart and Lung Institute, Imperial College London and Department of Cardiology, National Pulmonary Hypertension Service, National Pulmonary Hypertension Service, Hammersmith Hospital, London, England
11Pulmonary and Critical Care Division, University of Michigan Medical Center, Ann Arbor, Michigan, USA
12Massachusetts General Hospital, Heart Failure and Cardiac Transplantation Center, Boston, Massachusetts, USA
13Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud, Hôpital Antoine Béclèr to, Clamart, France
14Royal Brompton and Harefield NHS Foundation Trust, London, England
15Department of Cardiology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium

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); PHCOPD, 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. (JAmColl Cardiol 2013;62: D109–16) ©2013 by the American College of Cardiology Foundation.

Keywords: Combined pulmonary fibrosis and emphysema, COPD, exhausted circulatory reserve, exhausted ventilatory reserve; lung fibrosis; pulmonary hypertension in chronic lung disease

How to cite this article
Werner Seeger, Yochai Adir, Joan Albert Barberà, Hunter Champion, John Gerard Coghlan, Vincent Cottin, Teresa De Marco, Nazzareno Galiè, Stefano Ghio, Simon Gibbs, Fernando J. Martinez, Marc J. Semigran, Gerald Simonneau, Athol U. Wells, Jean-luc Vachiéry. Pulmonary hypertension in chronic lung diseases. Turk Kardiyol Dern Ars. 2014; 42(1): 142-152

Corresponding Author: Werner Seeger, Germany
Manuscript Language: Turkish


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