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Baseline characteristics and survival of patients with pulmonary hypertension in interstitial lung disease in the "HYPID" study

Vincent Cottin, Martine Reynaud-Gaubert, Hilario Nunes, David Montani, Benoit Wallaert, Boubou Camara, Chahéra Khouatra, Dominique Israel-Biet, Yürdagul Uzunhan, Emmanuel Gomez, Sylvain Marchand-Adam, Ana Nieves, Xavier Jaïs, Lidwine Wemeau-Stervinou, Julie Traclet, David Launay, Claire Dromer, Romain Magnier, Grégoire Prévot, Stéphanie Polazzi, Sabrina Zeghmar, Anne-Marie Schott, Marc Humbert, Jean-François Cordier
European Respiratory Journal 2013 42: 4839; DOI:
Vincent Cottin
1Respiratory Medicine, Louis Pradel Hospital, University Lyon 1, Lyon, France
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Martine Reynaud-Gaubert
2Respiratory Medicine, University Marseille, Marseille, France
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Hilario Nunes
3Respiratory Medicine, Avicenne University Hospital, Paris, France
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David Montani
4Respiratory Medicine, Bicetre Hospital, University Paris Sud, Paris, France
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Benoit Wallaert
5Respiratory Medicine, University Hospital, Lille, France
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Boubou Camara
6Respiratory Medicine, University Hospital, Grenoble, France
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Chahéra Khouatra
1Respiratory Medicine, Louis Pradel Hospital, University Lyon 1, Lyon, France
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Dominique Israel-Biet
7Respiratory Medicine, HEGP University Hospital, Paris, France
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Yürdagul Uzunhan
3Respiratory Medicine, Avicenne University Hospital, Paris, France
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Emmanuel Gomez
8Respiratory Medicine, University Hospital, Nancy, France
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Sylvain Marchand-Adam
9Respiratory Medicine, University Hospital, Tours, France
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Ana Nieves
2Respiratory Medicine, University Marseille, Marseille, France
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Xavier Jaïs
4Respiratory Medicine, Bicetre Hospital, University Paris Sud, Paris, France
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Lidwine Wemeau-Stervinou
5Respiratory Medicine, University Hospital, Lille, France
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Julie Traclet
1Respiratory Medicine, Louis Pradel Hospital, University Lyon 1, Lyon, France
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David Launay
10Internal Medicine, University Hospital, Lille, France
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Claire Dromer
11Respiratory Medicine, University Hospital, Bordeaux, France
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Romain Magnier
12Respiratory Medicine, University Hospital, Caen, France
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Grégoire Prévot
13Respiratory Medicine, University Hospital, Toulouse, France
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Stéphanie Polazzi
14Pole IMER, University Hospital, Lyon, France
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Sabrina Zeghmar
1Respiratory Medicine, Louis Pradel Hospital, University Lyon 1, Lyon, France
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Anne-Marie Schott
14Pole IMER, University Hospital, Lyon, France
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Marc Humbert
4Respiratory Medicine, Bicetre Hospital, University Paris Sud, Paris, France
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Jean-François Cordier
1Respiratory Medicine, Louis Pradel Hospital, University Lyon 1, Lyon, France
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Abstract

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.

  • Pulmonary hypertension
  • Interstitial lung disease
  • Idiopathic pulmonary fibrosis
  • © 2013 ERS
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Baseline characteristics and survival of patients with pulmonary hypertension in interstitial lung disease in the "HYPID" study
Vincent Cottin, Martine Reynaud-Gaubert, Hilario Nunes, David Montani, Benoit Wallaert, Boubou Camara, Chahéra Khouatra, Dominique Israel-Biet, Yürdagul Uzunhan, Emmanuel Gomez, Sylvain Marchand-Adam, Ana Nieves, Xavier Jaïs, Lidwine Wemeau-Stervinou, Julie Traclet, David Launay, Claire Dromer, Romain Magnier, Grégoire Prévot, Stéphanie Polazzi, Sabrina Zeghmar, Anne-Marie Schott, Marc Humbert, Jean-François Cordier
European Respiratory Journal Sep 2013, 42 (Suppl 57) 4839;

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Baseline characteristics and survival of patients with pulmonary hypertension in interstitial lung disease in the "HYPID" study
Vincent Cottin, Martine Reynaud-Gaubert, Hilario Nunes, David Montani, Benoit Wallaert, Boubou Camara, Chahéra Khouatra, Dominique Israel-Biet, Yürdagul Uzunhan, Emmanuel Gomez, Sylvain Marchand-Adam, Ana Nieves, Xavier Jaïs, Lidwine Wemeau-Stervinou, Julie Traclet, David Launay, Claire Dromer, Romain Magnier, Grégoire Prévot, Stéphanie Polazzi, Sabrina Zeghmar, Anne-Marie Schott, Marc Humbert, Jean-François Cordier
European Respiratory Journal Sep 2013, 42 (Suppl 57) 4839;
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