Heart diastolic dysfunction in patients with systemic sclerosis

Abstract

IntroductionThere are limited data on left (LV) and right ventricular (RV) diastolic function in systemic sclerosis (SSc) patients especially in relation to biomarkers of matrix remodeling. The aim of the study was to analyze LV and RV myocardial diastolic function in SSc patients at baseline and after at least 1 year of follow-up and its relation to serum tissue inhibitors of metalloproteinase 1 (TIMP-1) level.Material and methodsWe prospectively studied 111 SSc patients (101 female, 10 male, age 54.2 ±13.8 years) and 21 age-matched controls (18 female, 3 male, age 49.3 ±10.5 years). After at least 1 year of observation (3.0 ±1.1 years) we reevaluated 69 of the SSc patients. Transthoracic echocardiography (Philips, iE33) for assessment of LV and RV diastolic function was performed and TIMP-1 serum level was measured.ResultsImpaired LV relaxation was observed in 38 (34%) SSc patients and in 1 (5%) of the controls (p < 0.001). The mean E/A ratio was lower in patients with SSc than in controls (p = 0.002) and significantly decreased after the follow-up period (p = 0.02). Impaired RV relaxation was detected in 25 (22.5%) SSc patients and in 1 (5%) control subject (p < 0.001) but did not deteriorate after follow-up. Mean serum level of TIMP-1 was significantly elevated in the follow-up group compared to baseline examination (p = 0.0001). Serum TIMP-1 level correlated positively with E/E’, both septal and lateral (r = 0.4, p = 0.002 and r = 0.32, p = 0.01).ConclusionsThe LV and RV relaxation is impaired in SSc patients. Moreover, left ventricular diastolic function deteriorated after the follow-up period. The TIMP-1 serum levels correlate with echocardiographic parameters, providing a potent link for LV diastolic function and matrix remodeling in patients with SSc.

Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort

“…As only two patients had an LV ejection fraction < 45%, the majority of patients in this study can be considered to have diastolic dysfunction, which is consistent with prior literature . In echocardiographic studies of unselected patients, diastolic dysfunction is present in 23–62% of SSc subjects, with most estimates around 50%, which is a higher prevalence than healthy controls . In SSc patients, diastolic dysfunction tends to worsen over time, parameters of diastolic function correlate with exercise performance and LV diastolic dysfunction is independently associated with risk for death .…”

“…[10][11][12] In echocardiographic studies of unselected patients, diastolic dysfunction is present in 23-62% of SSc subjects, with most estimates around 50%, which is a higher prevalence than healthy controls. 2,10,[13][14][15][16][17] In SSc patients, diastolic dysfunction tends to worsen over time, 13,15 parameters of diastolic function correlate with exercise performance 14,18 and LV diastolic dysfunction is independently associated with risk for death. 15,17 Most of these studies, however, did not have a large number of PH patients, so the contribution of LHD to PH in SSc patients is less well defined.…”

Diastolic Dysfunction

“…29 In systemic sclerosis, TIMP-1 levels are associated with diastolic dysfunction and LV matrix remodeling. 30 In premenopausal, obese women with asymptomatic diastolic dysfunction, plasma MMP-2/-9 and TIMP profiles are altered. 31 …”

Searching for a good model for systemic sclerosis: the molecular profile and vascular changes occurring in UCD-200 chickens strongly resemble the early phase of human systemic sclerosis

“…During human SSc, despite severe tissue hypoxia, adapted angiogenesis does not occur. Indeed, defective vascularization contrasts with the elevated levels of VEGFA found in the serum and in the skin of SSc patients throughout the different SSc phases [ 24 , 25 ]. VEGFA exerts its effects by binding to VEGFR-1 and VEGFR-2.…”