Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: A multicenter study

Luna Gargani, Alberto Pignone, Gergely Agoston, Antonella Moreo, Eugenia Capati, Luigi P. Badano, Marica Doveri, Laura Bazzichi, Marco Fabio Costantino, Andrea Pavellini, Francesco Pieri, Francesco Musca, Denisa Muraru, Oscar Epis, Eleonora Bruschi, Benedetta De Chiara, Federico Perfetto, Fabio Mori, Oberdan Parodi, Rosa SicariStefano Bombardieri, Albert Varga, Marco Matucci Cerinic, Eduardo Bossone, Eugenio Picano

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Abstract

Background: Patients with systemic sclerosis (SSc) are at risk for developing pulmonary hypertension, which is associated with a poor prognosis. Exercise Doppler echocardiography enables the identification of exercise-induced increase in pulmonary artery systolic pressure (PASP) and may provide a thorough noninvasive hemodynamic evaluation. Aim: The aim of this study was to evaluate the clinical and echocardiographic determinants of exercise-induced increase in PASP in a large population of patients with SSc. Methods: We selected 164 patients with SSc (age 58 ± 13 years, 91% female) with normal resting PASP (<40 mm Hg) who underwent a comprehensive 2-dimensional and Doppler echocardiography and graded bicycle semisupine exercise Doppler echocardiography. Pulmonary artery systolic pressure, cardiac output, and pulmonary vascular resistance (PVR) were estimated noninvasively. Cutoff values of PASP ≥50 mm Hg and PVR ≥3.0 Wood Units at peak exercise were considered a significant exercise-induced increase in PASP and PVR, respectively. Results: Sixty-nine (42%) patients showed a significant exercise-induced increase in PASP. Among them, peak PVR ≥3 Wood Units was present only in 11% of patients, about 5% of the total population. Univariate analysis showed that age, presence of interstitial lung disease, and both right and left diastolic dysfunction are predictors of peak PASP ≥50 mm Hg, but none of these parameters predict elevated peak PVR. Conclusions: Exercise-induced increase in PASP occurs in almost one-half of patients with SSc with normal resting PASP. Peak exercise PASP is affected by age, interstitial lung disease, and right and left ventricular diastolic dysfunction and, only in 5% of the patients, is associated with an increase in PVR during exercise, suggesting heterogeneity of the mechanisms underlying exercise-induced pulmonary hypertension in SSc.

Original languageEnglish
Pages (from-to)200-207
Number of pages8
JournalAmerican Heart Journal
Volume165
Issue number2
DOIs
Publication statusPublished - Feb 1 2013

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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    Gargani, L., Pignone, A., Agoston, G., Moreo, A., Capati, E., Badano, L. P., Doveri, M., Bazzichi, L., Costantino, M. F., Pavellini, A., Pieri, F., Musca, F., Muraru, D., Epis, O., Bruschi, E., De Chiara, B., Perfetto, F., Mori, F., Parodi, O., ... Picano, E. (2013). Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: A multicenter study. American Heart Journal, 165(2), 200-207. https://doi.org/10.1016/j.ahj.2012.10.020