• Ekkehard Grünig, Henning Tiede, Esi Otuwa Enyimayew, Nicola Ehlken, Hans-Jürgen Seyfarth, Eduardo Bossone, Antonello D'Andrea, Robert Naeije, Horst Olschewski, Silvia Ulrich, Christian Nagel, Michael Halank, and Christine Fischer.
• Centre for Pulmonary Hypertension at Thoraxclinic Heidelberg, Heidelberg, Germany (E.G., E.O.E., N.E., C.N.); Department of Pneumology, University of Gießen Lung Center, Gießen, Germany (H.T.); Department of Pneumology, University of Leipzig, Leipzig, Germany (H.-J.S.); Cardiology Department, University of Salerno and IRCCS Policlinico San Donato, Milan, Italy (E.B.); Second University of Naples, Monaldi Hospital Naples, Naples, Italy (A.D.); Erasme University Hospital, Free University of Brussels, Brussels, Belgium (R.N.); Division of Pulmonology, Department Internal Medicine, Medical University of Graz, Graz, Austria (H.O.); Pulmonary Hypertension Program, University Hospital Zurich, Switzerland (S.H.); Department of Pneumology, University of Dresden, Dresden, Germany (M.H.); and Department of Human Genetics; University of Heidelberg, Heidelberg, Germany (C.F.).
• Circulation. 2013 Oct 29;128(18):2005-15.

BackgroundThis study sought to analyze a new approach to assess exercise-induced pulmonary artery systolic pressure (PASP) increase by means of stress Doppler echocardiography as a possible measure of right ventricular contractile reserve in patients with severe pulmonary hypertension and right heart failure.Methods And ResultsIn this prospective study, patients with invasively diagnosed pulmonary arterial hypertension or inoperable chronic thromboembolic pulmonary hypertension and impaired right ventricular pump function despite a stable targeted pulmonary arterial hypertension medication underwent a broad panel of noninvasive assessments, including stress echocardiography and cardiopulmonary exercise testing. On the basis of the assumption that exercise-induced PASP is a measure of right ventricular contractile reserve, patients were classified into 2 groups according to an exercise-induced PASP increase above or below the median. Patients were followed up for 3.0 ± 1.8 years. Univariate and multivariate analyses were used for factors predicting survival. Of 124 patients, 66 were below the median exercise-induced PASP increase of 30 mm Hg (low PASP), and 58 patients were above the median (high PASP). These groups were not significantly different in terms of medication and resting hemodynamics. Low PASP was associated with a significantly lower 6-minute walking distance, peak o2 per kilogram, and 1-, 3-, and 4-year survival rates (92%, 69%, and 48%, respectively, versus 96%, 92%, and 89%). In the multivariate Cox model analysis adjusted for age and sex, PASP increase during exercise and peak o2 per kilogram remained independent prognostic markers (hazard ratio, 2.56 for peak o2 per kilogram and 2.84 for PASP increase).ConclusionsExercise-induced PASP increase is of high clinical and prognostic relevance in pulmonary hypertension patients and may indicate right ventricular contractile reserve. Stress Doppler echocardiography may be a useful tool for prognostic assessment in pulmonary hypertension patients.

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