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

• Published in: The American heart journal Vol. 165; no. 2; pp. 200 - 207
• Main Authors: Gargani, Luna, MD, Pignone, Alberto, MD, PhD, Agoston, Gergely, MD, Moreo, Antonella, MD, Capati, Eugenia, MD, Badano, Luigi P., MD, FESC, FACC, Doveri, Marica, MD, PhD, Bazzichi, Laura, MD, Costantino, Marco Fabio, MD, Pavellini, Andrea, MD, Pieri, Francesco, MD, Musca, Francesco, MD, Muraru, Denisa, MD, Epis, Oscar, MD, Bruschi, Eleonora, MD, De Chiara, Benedetta, MD, Perfetto, Federico, MD, PhD, Mori, Fabio, MD, Parodi, Oberdan, MD, Sicari, Rosa, MD, PhD, Bombardieri, Stefano, MD, PhD, Varga, Albert, MD, PhD, FESC, Cerinic, Marco Matucci, MD, PhD, Bossone, Eduardo, MD, PhD, FESC, Picano, Eugenio, MD, PhD, FESC
• Format: Journal Article
• Language: English
• Published: United States Mosby, Inc 01.02.2013; Elsevier Limited
• Subjects: Carbon monoxide; Cardiovascular; Cardiovascular disease; Echocardiography, Doppler - methods; Exercise - physiology; Exercise Test - adverse effects; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Male; Middle Aged; Multivariate analysis; Patients; Pulmonary arteries; Pulmonary hypertension; Pulmonary Wedge Pressure; Quality control; Raynaud disease; Retrospective Studies; Scleroderma, Systemic - complications; Scleroderma, Systemic - physiopathology; Vascular Resistance
• ISSN: 0002-8703; 1097-6744
• DOI: 10.1016/j.ahj.2012.10.020

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.