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

Institute of Clinical Physiology, National Council of Research, Pisa, Italy. Electronic address: .
American heart journal (Impact Factor: 4.46). 02/2013; 165(2):200-7. DOI: 10.1016/j.ahj.2012.10.020
Source: PubMed


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.
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.
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.
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.
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.

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    ABSTRACT: High prevalence of exaggerated pulmonary artery pressure response to exercise (EPAPR) was reported in patients with systemic sclerosis (SSc). However, pathophysiology of this phenomenon has not been well defined. Therefore, we evaluated the frequency and potential aetiology of EPAPR in SSc patients. We included 85 patients (79 female, 6 male, mean age 54.3 ± 13.9 years) with SSc. Transthoracic echocardiography followed by exercise Doppler echocardiography (EDE) were performed. A positive EDE was defined when at least 20 mmHg increase of tricuspid regurgitation peak gradient (TRPG) was recorded. Right heart catheterization (RHC) with exercise was performed in positive EDE patients and in subjects with resting TRPG >31 mmHg. Resting TRPG >31 mmHg and/or positive EDE was found in 30 patients and they were referred to RHC. Finally, RHC was performed in 20 patients (16 pts resting TRPG >31 mmHg and 4 others normal resting TRPG and positive EDE). In 12 (60 %) of them an EPAPR with elevated pulmonary capillary wedge pressure (PCWP) was observed. Interestingly, mean left atrium (LA) diameter was greater in an EPAPR with elevated PCWP patients than in subjects with normal exercise response (39.36 ± 5.6 vs. 35.53 ± 3.48, p = 0.03). In EPAPR with elevated PCWP group greater mean value of E/E' of mitral lateral annulus was observed (7.98 ± 3.35 vs. 6.27 ± 1.94, p = 0.03). In the univariate logistic regression analysis increased LA diameter was significant predictor of EPAPR with elevated PCWP (OR 1.199, 95 % CI 1.029-1.396, p = 0.019). Despite very well-known risk of PAH in systemic sclerosis patients, the excessive increase of PAP during exercise is more commonly caused by left ventricular diastolic dysfunction than pulmonary arterial vasculopathy.
    Full-text · Article · Jul 2013 · Clinical Research in Cardiology

  • No preview · Article · Sep 2013 · American heart journal
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    ABSTRACT: Background Cardiac involvement in systemic sclerosis (SSc) is often clinically occult. The aim of this study was the evaluation of early subclinical right ventricular (RV) involvement in patients with limited form of systemic sclerosis by tissue Doppler. Methods Twenty female patients with limited cutaneous SSc and 20 control female subjects, matched for age were studied with transthoracic echocardiography and tissue Doppler imaging (TDI) to assess RV function. Patients with pulmonary hypertension, chronic renal failure, diabetes mellitus, hypertension, heart failure, left ventricular hypertrophy, ischemic or rheumatic heart disease were excluded. Results Patients with limited form SSc had significant lower tricuspid annulus peak systolic velocities (ST) (9.95 ± 1.78 vs. 11.05 ± 1.53 cm/s, p < 0.044), early (ET) (9.65 ± 1.30 vs. 12.50 ± 1.23 cm/s, p < 0.0001), late (AT) diastolic velocity (12.60 ± 2.01 vs. 18.15 ± 1.81 cm/s, p < 0.0001), and tricuspid annular plane systolic excursion (TAPSE) (23.05 ± 3.50 vs. 26.50 ± 1.90, p < 0.001) compared to controls. Myocardial performance index (MPI) of the RV was higher in SSc patients compared to controls (0.41 ± 0.05 vs. 0.30 ± 0.02, p < 0.0001). There were significant correlations between disease duration and ST and RV MPI (r = −0.883, p < 0.0001; r = 0.686, p < 0.001, respectively). Conclusions Patients with limited form of SSc present with pulsed-tissue Doppler imaging indices indicative of right ventricle dysfunction, which had significant correlations with disease duration. Tissue Doppler is a valuable non-invasive tool for detecting RV myocardial involvement in patients with limited SSc.
    Full-text · Article · Sep 2013 · Egyptian Heart Journal
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