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


OBJECTIVE
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.
Institute of Clinical Physiology, National Council of Research, Pisa, Italy. gargani@ifc.cnr.it
Luna
L
Gargani
Alberto
A
Pignone
Gergely
G
Agoston
Antonella
A
Moreo
Eugenia
E
Capati
Luigi P
LP
Badano
Marica
M
Doveri
Laura
L
Bazzichi
Marco Fabio
MF
Costantino
Andrea
A
Pavellini
Francesco
F
Pieri
Francesco
F
Musca
Denisa
D
Muraru
Oscar
O
Epis
Eleonora
E
Bruschi
Benedetta
B
De Chiara
Federico
F
Perfetto
Fabio
F
Mori
Oberdan
O
Parodi
Rosa
R
Sicari
Stefano
S
Bombardieri
Albert
A
Varga
Marco Matucci
MM
Cerinic
Eduardo
E
Bossone
Eugenio
E
Picano
D015150:Echocardiography, Doppler / Q000379:methods*; D015444:Exercise / Q000502:physiology*; D005080:Exercise Test / Q000009:adverse effects*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D014655:Vascular Resistance