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Objective: In systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) cardiac involvement is very common, and it might be asymptomatic. We aimed to assess the presence of fragmented QRS (fQRS) - possible indicator of a scar, diffuse damage or conduction system disturbances in those patients., Methods: We examined 74 patients with SSc, 77 with SLE and 40 healthy controls. The incidence of fQRS in ECG was examined according to criteria by Das et al. Disease severity was estimated by Rodnan and SLICCC/ACRDI scores in SSc and SLE respectively., Results: Patients with SSc were slightly older than those with SLE (53 ± 14 vs 46 ± 14 yrs), women constituted 91% and 88% of studied groups (p = 0.80). The duration of disease in both groups was similar (p = 0.59). Median of Rodnan and SLICCC/ACR-DI scores were 5 and 4 points, respectively. Left ventricle ejection fraction in both groups was similar (65.5 vs 65.1%, p = 0.51), hypertension incidence was lower in SSc (24 vs 48%, p = 0.004), patients with SLE presented prolonged corrected QT interval (432 vs 424 ms, p = 0.03). Of note, fQRS were observed in 34 (46%) SSc and 29 (38%) SLE patients, p = 0.33. Notched S waves were significantly more frequent in SSc (in 26% vs 10%, p = 0.02). fQRS occurred in 3 (7.5%) of healthy subjects only., Conclusion: The incidence of fQRS is similar in SSc and SLE, but is much more common than in healthy subjects. It is possible that fQRS may be considered an additional marker of heart involvement in these rare diseases., Competing Interests: Declarations of interest The authors have no conflicts of interest to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: Pulmonary hypertension (PH) is an ominous complication in systemic sclerosis patients (SSc) and echocardiography is a screening tool for its detection. The goal of this study was to assess the reliability of resting and exercise echo Doppler parameters with data obtained by right heart catheterization (RHC)., Material and Methods: We included 91 patients (84 F, 53.3 ± 15.2 years) with SSc. Transthoracic echocardiography followed by exercise Doppler-echocardiography (EDE) were performed. A positive EDE was defined as a ≥20 mmHg increase in tricuspid regurgitation peak gradient (TRPG). RHC with exercise was performed in positive EDE patients and/or in subjects with resting TRPG > 31 mmHg., Results: Finally, RHC was performed in 20 patients. The correlation for the echocardiography and invasive measurement of systolic (sPAP) and mean (mPAP) pulmonary artery pressure was r = 0.66 (p = 0.001) and r = 0.7 (p = 0.001), respectively. We also found significant correlation between echocardiography and invasive measurement of exercise sPAP r = 0.68 (p = 0.001) and exercise mPAP r = 0.67 (p = 0.002). There was a correlation between pulmonary vascular resistance (PVR) assessed by echocardiography and measured by RHC r = 0.49, p = 0.027. The equation derived within our population was: PVR by echocardiography = 9.6*TRV/TVI RVOT +0.068. We also performed ROC analysis to predict PVR > 2 WU. Our results highlight that sPAP has the highest AUC (0.802, 95% CI 0.585-1)., Conclusion: Doppler resting and exercise echocardiography may provide a reliable, noninvasive method for determining resting and exercise sPAP, mPAP, and PVR in SSc patients, although it may underestimate or overestimate these values in some individuals. Doppler echocardiography does not replace RHC for definite hemodynamic assessment of suspected PH., (Copyright © 2019. Published by Elsevier B.V.)

Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are connective tissue diseases presenting cardiac complications including different arrhythmias, then direct electrocardiographic comparison may be useful in everyday clinical decision making. We examined 86 adult SSc patients, 76 with SLE and 45 healthy controls. Among other examinations all subjects underwent 24-h Holter monitoring with time-domain heart rate variability and heart rate turbulence evaluation. Patients with various co-existing conditions which might markedly influence arrhythmias and autonomic modulation were excluded from further analysis (SSc n = 12, SLE n = 6). Finally, 76 SSc and 70 SLE subjects were eligible for this study, mean age 51.9 ± 13.1 and 46.5 ± 12.7 years (p = 0.11), with median disease duration 6.0 and 8.5 years (p = 0.15), respectively. As compared to SLE, patients with SSc were characterised by more frequent incidence of various supraventricular and ventricular arrhythmias. As compared to SSc, patients with SLE presented prolonged corrected QT intervals and also significant correlations between corrected QT length and heart rate variability indices. Both SSc and SLE subjects presented impaired sympathetic cardiac autonomic modulation, while indices associated with parasympathetic activity in SLE were not diminished. Disease duration was not associated with arrhythmias' occurrence (except for ventricular tachycardia in SSc, p = 0.02) and also with autonomic function in both groups of patients. Patients with SSc and SLE differ in terms of arrhythmias, conduction disturbances and cardiac autonomic tone. Regular Holter monitoring should be considered as a part of routine evaluation in connective tissue diseases patients, especially in systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune disorder characterized by skin and internal organs fibrosis and concomitant vascular abnormalities. Although SSc is considered mainly fibrosing disease, underlying vascular pathology plays a fundamental role in its pathogenesis. We have focused on positive and negative serum markers of angiogenesis and fibrosis (pigment epithelium-derived factor [PEDF], vascular endothelial growth factor [VEGF], and soluble VEGF receptor [sVEGFR]), in progressive SSc patients at baseline and after follow-up in relation to cardiopulmonary complications (systemic hypertension [HT], pulmonary arterial hypertension [PAH] and pulmonary fibrosis [PF]). VEGF and PEDF but not sVEGFR were reciprocally regulated in SSc progression. Moreover, VEGF/PEDF ratio significantly increased during follow up suggesting that it might be used as a biomarker of disease progression. No correlation between the studied markers and cardiopulmonary complications was observed. In conclusion, VEGF and PEDF level, and the VEGF/PEDF ratio are significantly changed in the course of SSc progression and these markers can be used to assess SSc activity., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Objectives: Systemic sclerosis (SSc) leads to pulmonary circulation dysfunctionand there are some indications of systemic circulation impairment. We evaluated the influence of SSc on the elastic properties of large systemic arterial walls and potential correlations between systemic and pulmonary circulation involvement., Method: We examined 75 consecutive women (mean age 53.13±10.1 years) with confirmed SSc [mean disease duration (DD) 7.1±9.1 years] and 21 age-matched female volunteers (mean age 52.6±8.3 years, ns). Pulse wave velocity (PWV) and transthoracic echocardiography were performed. SSc patients were divided into two groups according to the median of DD: ≤3 years (39 patients) and >3 years (36 patients)., Results: Patients with DD>3 years had higher PWV than those with DD≤3 years and controls (log PWV: 2.23±0.23 vs. 2.13±0.16 and vs. 2.11±0.16 m/s; p=0.028 and 0.029, respectively). In addition, echocardiographic indices showed impaired right ventricular (RV) function in the patients with DD>3 years. Also in these SSc patients, PWV correlated with clinical and echocardiographic parameters of pulmonary circulation: age (r=0.64, p<0.0001), acceleration time of pulmonary ejection (AcT; r=-0.38, p=0.021), and tricuspid regurgitation peak gradient (TRPG; r=0.34, p=0.04). Multiple linear regression analysis showed that PWV was independently associated with DD (β=0.22, p==0.02), AcT (β=-0.215, p=0.03), and age (β=0.44, p<0.001)., Conclusions: In patients with SSc lasting more than 3 years, the disease is characterized by increased stiffness of the large systemic arteries. Longer duration of SSc leads simultaneously to the increased stiffness of the large systemic arteries and to the progressive impairment of RV function and its coupling to the pulmonary arterial bed.

Background: Heart and pulmonary involvement is a leading cause of systemic sclerosis (SSc)-related deaths., Objectives: The aim of our study was to assess if biochemical markers of right ventricular (RV) overload, endothelial function and collagen metabolism can predict RV dysfunction assessed by Doppler echocardiography in SSc patients., Methods: We prospectively studied 111 consecutive patients (101 F, 10 M, age 54.2 ± 13.8 years) with diagnosed SSc (mean disease duration 9.4 ± 11.4 years) and a group of 21 age-matched subjects (18 F, 3 M, age 49.3 + 10.5 years). We performed transthoracic echocardiography (Phillips iE 33) and measured serum endothelin-1 (ET-1), N-terminal pro-brain natriuretic peptide (NT-proBNP), asymmetric dimethylarginine (ADMA), endoglin and human tissue inhibitor of matrix metalloproteinase (TIMP-1) concentration., Results: Median serum NT-proBNP level in SSc patients was 133.5 (range 21.86-17,670 pg/ml) and was significantly higher than in controls (p = 0.0002). Moreover, the median serum ET-1 level of 1.49 (range 0.26-8.75 pg/ml) was higher in SSc patients (p = 0.002). However, no significant differences in ADMA, TIMP-1 and endoglin serum concentration between SSc patients and controls were observed. Serum NT-proBNP concentration correlated positively with echocardiographic signs of RV overload: tricuspid regurgitation pressure gradient (r = 0.38, p = 0.0004) and RV Tei index (r = 0.25, p = 0.01). ET-1 serum level correlated negatively with tricuspid annular plane systolic excursion (r = -0.4, p = 0.01) and positively with inferior vena cava diameter measured at expiration (r = 0.38, p = 0.0002). The echocardiographic signs of RV overload were significantly more pronounced in the highest NT-proBNP tertile (>195 pg/ml) group than in the lowest one (<88 pg/ml)., Conclusions: Serum ET-1 and NT-proBNP, but not endoglin, ADMA and TIMP-1 levels correlating with the echocardiographic parameters of RV overload, can be considered as noninvasive indicators of RV dysfunction in SSc patients.

Objective: 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., Methods: 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., Results: 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)., Conclusions: 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.

Objectives: Heart and pulmonary involvement is a leading cause of systemic sclerosis (SSc)-related deaths. The six-minute walk test (6MWT) is a simple and reproducible test commonly used to evaluate exercise capacity. We tried to assess a potential relationship between exercise capacity assessed by 6MWT, echocardiographic parameters of right ventricular function and serum levels of endothelin-1 and NTproBNP., Methods: We prospectively studied 111 consecutive patients (101F, 10M, age 54.2±13.8 years) with diagnosed SSc (mean disease duration 9±12.4 years) and a group of 21 age-matched subjects (18F, 3M, age 49.3±10.5 years). In addition to routine evaluation, 6MWT and transthoracic echocardiography (Phillips iE 33) were performed. We also measured serum endothelin-1 (Human Endothelin-1 immunoassay R & D Systems) and NT-proBNP levels (Elecsys pro-BNP immunoassay; Roche Diagnostics)., Results: The mean 6MWT distance was significantly shorter in the SSc group than in the controls (562.8±60.3 vs. 514.7±102.5 m, p=0.03). In the SSc group 6MWT distance correlated with ET-1 (r=-0.5, p<0.0001), NTproBNP (r=-0.4, p=0.0008) levels, and echocardiographic indices AcT (r=0.4, p=0.0002) and TRPG (r=-0.4, p=0.0011). Moreover, in patients with 6MWT distance <450 m NTproBNP and endotothelin-1 levels were significantly higher than in patients with distance >450 m (311.2, 31.1-17237 vs. 105.3, 5-17670 pg/ml, p=0.0138 and 2.9±2.2 vs. 1.4±0.7 pg/ml, p=0.0032)., Conclusions: Decreased exercise capacity significantly correlates with biochemical and echocardiographic parameters of right ventricular dysfunction and neurohormonal activation providing a potential link for neuroendocrine derangement in patients with SSc.

Although scleroderma is generally considered a fibrosing disease, it is now recognized that the underlying vascular pathology is playing a fundamental role in its pathogenesis. The present study was aimed at testing the prevalence of anti-endothelial cell antibodies (AECA) in systemic scleroderma (SSc) patients with and without pulmonary hypertension (PH) and in relation to the presence of pulmonary fibrosis. Fifty four SSc patients (50 females and 4 male, mean age 55.7 ± 16.3 years) were prospectively screened. All patients underwent transthoracic echocardiography with the estimation of pulmonary artery pressure (PAP) and tricuspid regurgitant peak gradient (TRPG). All patients suspected to have pulmonary hypertension were referred for right heart catheterization. Restrictive lung disease was confirmed by HRCT. A healthy control group included (n = 27; 7 men and 20 women, mean age 49.8 ± 12.1 years). The study of AECA was performed using the indirect immunofluorescence method on commercially available human umbilical vein endothelial cells. The HRCT scans in patients with suspected interstitial lung disease revealed signs of lung fibrosis in 15 (out of the 36 examined patients). TRPG at rest of 31 mmHg was demonstrated in 14 (21%) patients. During cardiac catheterization, arterial PH was found in two patients. Resting venous PH was found in one patient and an excessive post capillary PAP elevation at rest was demonstrated in 11 patients. At the baseline, 14/54 patients (26%) were positive for AECA. In the control group, the frequency of the antibodies was 3/27 (11%). No statistical correlation between antibody titter and the presentation of the disease existed. AECA were highly prevalent in a subgroup of patients suffering from interstitial pulmonary fibrosis. Out of the 15 patients suffering from lung fibrosis, 7 were AECA positive. The presence of AECA correlated very well with antinuclear antibodies (ANA), but was not related to the profile of ANA. Our findings support evidence that endothelial cell damage is involved in SSc, as there was increased prevalence of circulating AECA of the IgG isotype in SSc patients. AECA may also be related to the complications of SSc, like pulmonary fibrosis.

Background: In systemic sclerosis (SSc), changes in the lungs and pulmonary hypertension (PH) are complications most adversely affecting the prognosis. Given the availability of specific treatment, early diagnosis of PH is very important. Exercise echocardiography, by increasing the patient's cardiac output, makes it possible to identify patients with elevated pulmonary artery pressure (PAP) during exercise. The diagnostic role of exercise echocardiography is still unclear, mainly because of the lack of prospective studies., Aim: To identify SSc patients with abnormally elevated PAP at rest or with a significant increase PAP during exercise, subsequently verified by right heart catheterisation (RHC)., Methods: A total of 71 consecutive patients (67 females and 4 males, mean age 56.9 ± 17.1 years) with SSc diagnosed according to the American College of Rheumatology criteria were enrolled in this prospective study. The patients underwent transthoracic echocardiography (Philips iE33) with the measurement of tricuspid regurgitation peak gradient (TRPG) and an exercise test involving the standard treadmill exercise according to the Bruce protocol with the evaluation of TRPG at 1 min following the completion of exercise. The PH was suspected when TRPG at rest was 〉 31 mm Hg (V(max) 〉 2.8 m/s) or increased by at least 20 mm Hg from baseline following exercise. Patients with suspected PH were referred for resting and exercise RHC., Results: The exercise testing was performed in 67 patients revealing normal left ventricular (LV) systolic function in all of them. The mean LV ejection fraction was 66.1% ± 3.9%. The TRPG at rest could be recorded in 65 (97%) patients with the mean value of 26.9 ± ± 7.6 mm Hg (range 17-57 mm Hg). A resting TRPG of 〉 31 mm Hg, suggestive of possible PH, was demonstrated in 14 (21%) patients. During exercise test 56 (84%) patients achieved the maximum heart rate. A Doppler spectrum enabling the measurement of TRPG following the exercise was obtained in 66 (98.5%) patients. The gradient following the exercise could not be measured in one patient with a resting TRPG of 30 mm Hg. The mean post-exercise TRPG was 40.3 ± 4.1 mm Hg (range 17-70) and the mean post-exercise increase in TRPG was 12.9 ± 8.5 mm Hg (range 2-38). A TRPG increase of 〉 20 mm Hg was found in 11 (16%) patients (including 4 patients with resting values exceeding 31 mm Hg and 7 patients with normal resting values). Twenty-one (31%) patients with echocardiographic suspicion of PH (TRPG 〉 31 mm Hg at rest and/or a post-exercise increase in TRPG of more than 20 mm Hg) were referred for RHC with 16 patients actually undergoing the procedure. Four out of these 16 patients were qualified because of the "positive" exercise echocardiography in the presence of normal TRPG values. During catheterisation arterial PH was found in 2 patients, and an excessive precapillary PAP elevation in 2 further patients. Resting venous PH was found in 1 patient and an excessive postcapillary PAP elevation at rest was demonstrated in 11 patients., Conclusions: Exercise echocardiography is a safe and useful screening tool for PH diagnosis in patients with SSc. It enables to identify patients with normal systolic PAP at rest but a significant increase during exercise. The final confirmation of PH and differentiation between precapillary arterial and postcapillary venous PH requires RHC.

Background: Arrhythmias, conduction disturbances, and cardiac autonomic nervous system dysfunction are the most frequent cardiovascular complications in systemic sclerosis (scleroderma). The aim of the study was to assess heart rate turbulence (HRT) in systemic sclerosis patients and to identify the relationship between HRT and occurrence of arrhythmias., Methods: Forty-five patients with scleroderma (aged 54.6 +/- 14.7 years) and 30 healthy sex- and age-matched subjects were examined. In addition to routine studies, 24-hour Holter monitoring with assessment of HRT was performed., Results: As compared to controls, HRT was significantly impaired in systemic sclerosis patients. Abnormal HRT defined as turbulence onset (TO) > or =0.0% and/or turbulence slope (TS) < or =2.5 ms/RR (ms/RR interval) was found in 19 (42%) scleroderma patients and in no members of the control group. Serious ventricular arrhythmias Lown class IV (VA-LownIV), for example, couplets and/or nonsustained ventricular tachycardias, were observed in 16 (36%) scleroderma patients. The median value of TS was significantly lower in systemic sclerosis patients with VA-LownIV than in patients without VA-LownIV (3.68 vs 7.00 ms/RR, P = 0.02). The area under curve of ROC analysis for prediction of VA-LownIV was 0.72 (95% confidence interval [CI] 0.56-0.87) and revealed that TS <9.0 ms/RR was associated with VA-Lown IV occurrence, with sensitivity of 93.7% and specificity of 44.8%. Univariate and multivariate analyses confirmed that lower values of TS were associated with VA-LownIV occurrence (odds ratio 1.52, 95% CI 1.09-2.12, P = 0.01)., Conclusions: Patients with systemic sclerosis are characterized by significant HRT impairment. Assessment of HRT and especially TS is useful in the identification of patients at risk for ventricular arrhythmias.

Objective: To assess the heart rate turbulence (HRT) in patients with SSc as a method of assessment for cardiac autonomic nervous function., Methods: We prospectively studied 68 consecutive patients with SSc before inclusion in the study. After a detailed clinical evaluation, including echocardiography, 45 subjects [aged 54.6 (14.7) years; 40 women] underwent 24-h Holter monitoring for HRT and time- and frequency-domain heart rate variability (HRV) assessment. Results were compared with those in 30 age- and sex-matched healthy controls., Results: As compared with controls, HRT was impaired in SSc patients: the median turbulence onset (TO) was higher (P = 0.0001) and the median turbulence slope (TS) was lower (P = 0.0003). Abnormal HRT (TO > or =0.0% and/or TS < or =2.5 ms/RR) was found in 42% of SSc patients. Moreover, SSc duration correlated negatively with values of TS (r = -0.3; P = 0.045). HRT did not differ between diffuse and limited SSc groups. All estimated time- and the majority of frequency-domain values of HRV parameters in SSc were significantly lower than in controls. Significant correlations were also demonstrated between HRT and HRV parameters., Conclusions: HRT, like HRV assessment, indicates a frequent impairment of the cardiac autonomic nervous system in SSc patients, irrespective of the SSc type.

Elevated serum brain natriuretic peptide (BNP) released from myocytes of ventricles upon stretch have been found in patients with isolated right ventricular (RV) pressure overload. However, limited data suggest that serum BNP may be elevated in systemic sclerosis (SSc) patients, especially with RV dysfunction. We assessed serum N-terminal proBNP (NT-proBNP) in SSc and evaluated whether it reflects the severity of RV overload. We prospectively studied 51 consecutive patients (47F, mean age 53.3 +/- 15.2 years) with SSc (mean disease duration 9 +/- 12.4 years). The control group formed 31 healthy subjects (27F, mean age 52.6 +/- 12.1 years). NT-proBNP level, 6-minute walking test (6MWT), and transthoracic echocardiography (TTE) for the assessment of RV overload were performed. Serum NT-proBNP exceeded the reference value of 125 pg/mL in 31 (61%) SSc patients. The mean serum log NT-proBNP concentration in SSc was higher than in controls (2.138 +/- 0.527 vs. 1.634 +/- 0.420 pg/mL, p < 0.001). 13 (25%) SSc patients have tricuspid regurgitation peak gradient (TRPG) exceeding 31 mmHg reflecting pulmonary arterial hypertension (PAH). The SSc presented other echocardiographic signs of RV overload. Mean 6MWT distance was shorter in SSc than in controls (528 +/- 100 vs. 617 +/- 80 m, p < 0.001). NT-proBNP level correlated positively with TRPG, RV diameter, RV Tei index and negatively with 6MWT distance. ROC analysis identified >115 pg/ml as the best NT-proBNP threshold predicting PAH for SSc patients (sensitivity 92%, specificity 44%). Results of our study suggest that NT-proBNP measurement is a useful screening method for PAH in SSc patients. Since elevated plasma NT-proBNP level reflects the degree of right ventricular overload and limitation of exercise capacity, abnormal NT-proBNP levels should imply further evaluation including echocardiography.

Background: Systemic sclerosis (SSc) is a connective tissue disease characterised by vascular changes and immunologically induced fibrosis of the skin and internal organs. Systemic sclerosis may be associated with both right (RV) and left ventricular (LV) diastolic dysfunction., Aim: To analyse RV and LV myocardial diastolic function in patients with SSc and its relation to exercise capacity., Methods: We prospectively studied 51 consecutive patients (47 females, 4 males, age 53.3+/-15.2 years) with SSc (mean disease duration 9+/-12.4 years) and a group of 31 age-matched healthy subjects (28 females, 3 males, age 52.68+/-12.1 years). In addition to conventional investigation, transthoracic echocardiography (TTE) for assessment of RV and LV myocardial diastolic function and 6-minute walking test (6MWT) were performed., Results: Abnormal LV filling, as expressed by an inverted mitral E/A ratio (Mit E/A <1), was detected in 28 (55%) SSc patients and in 8 (25%) controls (p <0.001). The mean value of Mit E/A in the SSc group was lower than in controls (1.0+/-0.3 vs. 1.2+/-0.3, p=0.04). There were no differences in pulmonary venous flow between SSc patients and controls. The mean value of Tei index for the LV was higher in SSc than in controls (0.44+/-0.08 vs. 0.38+/-0.05, p <0.001). Abnormal RV filling, as expressed by an inverted tricuspid E/A ratio (TR E/A <1), was detected in 16 (31%) SSc patients and in 5 (16%) controls (p <0.001). Patients with SSc were found to have an inverted Tr E/A ratio (Tr E/A <1), indicating abnormal RV filling. The mean value of Tr E/A in SSc was lower than in controls (1.0+/-0.2 vs. 1.2+/-0.3, p=0.04). The mean value of Tei index for the RV was higher in SSc patients than in controls (0.35+/-0.07 vs. 0.29+/-0.03, p <0.001). In multiple regression analysis Tr E/A ratio was independently correlated with Mit E/A ratio (r=0.65, p=0.01). The mean 6MWT distance was shorter in the SSc group than in controls (528+/-100.6 vs. 617.7+/-80 m, p <0.001) and the mean saturation of capillary blood after the 6-MWT was lower in SSc patients (92.7+/-4.9 vs. 97.2+/-1.2%, p <0.001). Mean desaturation after test and D sat was significantly more pronounced in the SSc group than in controls (3.4+/-3.1 vs. 0.7+/-0.9, p <0.001). The Tr E/A ratio and Mit E/A ratio correlated positively with 6MWT distance (r=0,49, p=0.01 and r=0.48, p=0.02)., Conclusions: Impaired RV and LV relaxation is observed in a significant percentage of SSc patients and is associated with decreased exercise capacity.

Background: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and visceral organs, in which the heart is frequently (40-70% of patients) and severely involved. Pulmonary hypertension affects 10-15% of patients with SSc and is one of the most important complications adversely influencing their survival., Case Report: The case report presents a 59-year-old male patient with advanced systemic sclerosis whose initial examination revealed pulmonary hypertension, rhythm and atrioventricular conduction disturbances, and elevated level of NT-proBNP. After six months the patient deteriorated; an increase in NT-proBNP level and progression of pulmonary hypertension were observed., Conclusions: The described case is followed by a discussion of cardiovascular involvement in systemic sclerosis and emphasizes that heart involvement in SSc may have very serious clinical implications.