• Mart N van der Plas, Coen van Kan, Judith Blumenthal, Henk M Jansen, Athol U Wells, and Paul Bresser.
• Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands.
• Respirology. 2014 Feb 1; 19 (2): 269-275.

Background And ObjectivePulmonary 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.MethodsFrom 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).ResultsEleven 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.ConclusionsV'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.© 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.

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