Pulmonary vascular limitation to exercise and survival in idiopathic pulmonary fibrosis
Mart N. van der Plas
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorCoen van Kan
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Search for more papers by this authorJudith Blumenthal
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorHenk M. Jansen
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorAthol U. Wells
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
Search for more papers by this authorCorresponding Author
Paul Bresser
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Correspondence: Paul Bresser, Onze Lieve Vrouwe Gasthuis, PO Box 95500, 1090 HM Amsterdam, the Netherlands. Email: [email protected]Search for more papers by this authorMart N. van der Plas
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorCoen van Kan
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Search for more papers by this authorJudith Blumenthal
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorHenk M. Jansen
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Search for more papers by this authorAthol U. Wells
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
Search for more papers by this authorCorresponding Author
Paul Bresser
Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
Correspondence: Paul Bresser, Onze Lieve Vrouwe Gasthuis, PO Box 95500, 1090 HM Amsterdam, the Netherlands. Email: [email protected]Search for more papers by this authorAbstract
Background and objective
Pulmonary hypertension is frequently observed in advanced idiopathic pulmonary fibrosis (IPF) and is associated with poor prognosis. Cardiopulmonary exercise testing (CPET) can be used to detect less advanced pulmonary vascular impairment, and therefore may be of prognostic use. We studied the predictive value of non-invasive exercise parameters that were associated with elevated systolic pulmonary artery pressure (sPAP) for survival in IPF patients.
Methods
From our interstitial lung disease database, we reviewed records of consecutive patients with IPF in whom CPET and echocardiography were performed within 2 weeks (n = 38).
Results
Eleven patients (29%) had increased sPAP (≥40 mm Hg). From all non-invasive CPET parameters, only the ventilatory equivalent for CO2 (V'E/V'CO2) at anaerobic threshold differed significantly between patients with and without sPAP ≥ 40 mm Hg. The receiver-operator characteristic curve for V'E/V'CO2 resulted in areas under the curve of 0.77 (95% CI: 0.569–0.970; P = 0.026), with a cut-off value for predicting sPAP ≥ 40 mm Hg of >45.0. Patients with V'E/V'CO2 > 45.0 had significantly worse survival compared with patients with V'E/V'CO2 ≤ 45.0 (P = 0.001). In contrast, sPAP did not predict survival.
Conclusions
V'E/V'CO2, the only CPET parameter associated with elevated sPAP, appears a potentially useful non-invasive marker for early detection of pulmonary vascular impairment, and therefore may be of use for a more accurate prognostic assessment in IPF patients.
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