Abstract

Background: Dobutamine stress echocardiography (DSE) has limited application in systemic sclerosis (SSc). We examined DSE usefulness in revealing pulmonary arterial hypertension (PAH) in selected SSc patients whose resting echocardiography for pulmonary hypertension (PH) was non-diagnostic. Methods: Forty SSc patients underwent right heart catheterization (RHC) and, simultaneously, low-dose DSE (incremental doses up to 20 μg/kg/min). Inclusion criteria were: preserved left and right ventricular (RV) function (tricuspid annulus plane systolic excursion [TAPSE] ≥ 16 mm and tissue Doppler imaging-derived systolic velocity of tricuspid annulus [RVS’] > 10 cm/s), normal pulmonary function tests, and baseline maximal tricuspid regurgitation (TR) velocity of 2.7–3.2 m/s. Results: Of 36 patients who completed DSE, resting RHC diagnosed PAH in 12 patients (33.3%). At 20 μg/kg/min, patients with PAH had higher TR velocity, higher pulmonary arterial pressure measured by RHC, and lower RV inotropic response compared with patients without PAH. A cut-off value of maximal TR velocity >3.1 m/s had a sensitivity of 80%, a specificity of 84.2%, and an accuracy of 82.4% for the detection of PAH. Conclusions: Low-dose DSE has a satisfactory diagnostic accuracy for the early detection of PAH in highly selected SSc patients whose baseline echocardiographic measurements for PH lie in the gray zone.

Highlights

  • Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc) [1,2] associated with increased morbidity and mortality [3]

  • Exercise stress echocardiography has been applied in the past for the identification of pulmonary hypertension (PH) in SSc patients [9], but the lack of standardization and of prospective confirmatory data has downgraded its role for the detection of PH in the 2015 ESC/ERS guidelines [2]

  • We explored the potential value of low-dose Dobutamine stress echocardiography (DSE) to identify pulmonary arterial hypertension (PAH) in selected asymptomatic or mildly symptomatic patients with SSc whose resting echocardiography was equivocal for the presence of PH, i.e., the systolic pulmonary arterial pressure (PAP) was between 35 and

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Summary

Introduction

Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc) [1,2] associated with increased morbidity and mortality [3]. A diagnosis of pulmonary hypertension (PH) is established by right heart catheterization (RHC), the measurement of maximal tricuspid regurgitation (TR) velocity at rest by Doppler echocardiography is an important tool for the non-invasive estimation of pulmonary arterial pressure (PAP) [2]. There are very few data on the role of dobutamine stress echocardiography (DSE) in SSc or PAH patients either for the detection of wall motion abnormalities of the left ventricle (LV) or for the assessment of right ventricular (RV) inotropic reserve [10,11,12]. We examined DSE usefulness in revealing pulmonary arterial hypertension (PAH) in selected SSc patients whose resting echocardiography for pulmonary hypertension (PH). Methods: Forty SSc patients underwent right heart catheterization (RHC) and, simultaneously, low-dose DSE (incremental doses up to 20 μg/kg/min). At 20 μg/kg/min, patients with PAH had higher TR velocity, higher pulmonary arterial pressure measured by RHC, and lower RV inotropic response compared with patients without PAH

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