Abstract

ObjectiveHigh 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.MethodsWe 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.ResultsResting 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).ConclusionsDespite 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.

Highlights

  • Pulmonary hypertension (PH) is a well-known complication of systemic sclerosis and together with interstitial lung diseases is the leading cause of SSc-related deaths

  • Despite very well-known risk of PAH in systemic sclerosis patients, the excessive increase of pulmonary artery pressure (PAP) during exercise is more commonly caused by left ventricular diastolic dysfunction than pulmonary arterial vasculopathy

  • We assumed that impaired left ventricular (LV) diastolic function present in SSc patients is responsible for exaggerated PAP responses to exercise and can lead to post-capillary hypertension

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Summary

Introduction

Pulmonary hypertension (PH) is a well-known complication of systemic sclerosis and together with interstitial lung diseases is the leading cause of SSc-related deaths. The prevalence of pulmonary arterial hypertension in SSc patients is 7–12 % [1, 2]. Recent data suggest that there is an unexpectedly high prevalence of inappropriate pulmonary artery pressure (PAP) responses to exercise in patients with SSc [8,9,10,11,12]. Some authors suggested that SSc patients with normal resting PAP and excessive exercise increase in PAP present an early stage of pulmonary arteriolar vasculopathy [13]. In this group of patients progressive cardiac fibrosis impairing left ventricular (LV) diastolic function was reported [14, 15]. We assumed that impaired LV diastolic function present in SSc patients is responsible for exaggerated PAP responses to exercise and can lead to post-capillary hypertension

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