RT Journal Article
SR Electronic
T1 Baseline characteristics and survival of patients with pulmonary hypertension in interstitial lung disease in the "HYPID" study
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 4839
VO 42
IS Suppl 57
A1 Vincent Cottin
A1 Martine Reynaud-Gaubert
A1 Hilario Nunes
A1 David Montani
A1 Benoit Wallaert
A1 Boubou Camara
A1 Chahéra Khouatra
A1 Dominique Israel-Biet
A1 Yürdagul Uzunhan
A1 Emmanuel Gomez
A1 Sylvain Marchand-Adam
A1 Ana Nieves
A1 Xavier Jaïs
A1 Lidwine Wemeau-Stervinou
A1 Julie Traclet
A1 David Launay
A1 Claire Dromer
A1 Romain Magnier
A1 Grégoire Prévot
A1 Stéphanie Polazzi
A1 Sabrina Zeghmar
A1 Anne-Marie Schott
A1 Marc Humbert
A1 Jean-François Cordier
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/4839.abstract
AB Background. Pulmonary hypertension (PH) in patients with interstitial lung disease (ILD) is frequent and associated with a shorter survival.Objective. To study the clinical, pulmonary function, haemodynamic characteristics, and survival in a large cohort of patients with ILD-PH at right heart catheterization.Methods. A prospective multicenter observational study (HYPID) was initiated in July 2010 in French expert centers for rare pulmonary diseases and/or PH (NCT01443598).Results. Out of 220 patients included (mean age 63 ±10 years; 135 males), ILD was idiopathic pulmonary fibrosis (n=37), combined pulmonary fibrosis and emphysema syndrome (n=51), systemic sclerosis with ILD (n=38), sarcoidosis (n=33), and other ILD (n=61). NYHA class was I-II in 19% of patients, III in 59%, IV in 20%, N/A in 2%. Six-min walk distance was 282 ± 147m. Hemodynamic characteristics were mean pulmonary arterial pressure (mPAP) 39 ± 10 mmHg, cardiac index (CI) 2.7 ± 0.7 L/min/m², and pulmonary vascular resistance (PVR) 542 ± 265 dyn.s.cm-5. mPAP was ≥35 mmHg in 59% of the cases and ≥40 mmHg in 40% of the cases. PVR poorly correlated with FVC (r=0.516, p=0.028) or PaO2 (r=0.593, P=0.007). mPAP poorly correlated with FVC (r=0.587, p=0.007) and PaO2 (r=0.593, P=0.007). The median time to death or transplant was 3.85 years, with no significant difference between patients with mPAP < or ≥ 35 or 40 mmHg.Conclusion. Hemodynamic characteristics do not correlate with pulmonary function in ILD-PH. Preliminary data suggest that the level of mPAP (< or ≥ 35 or 40 mmHg) does not impact survival.