Background: Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity. Methods: Α prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test). Results: Both IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p < 0.001) and increased loop width (15.7 ± 3.9 μm and 15.8 ± 1.9 μm vs. 11.5 ± 2.3 μm, p < 0.001). More than half of patients with IPAH presented microhaemorrhages on capillary nailfold, while increased shape abnormalities in capillary morphology and more capillary thrombi per linear mm were detected in patients with CTEPH compared to patients with IPAH and healthy controls. All PH patients presented a non-specific NVC pattern compared to controls (p < 0.001). Conclusion: The findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

Adverse cardiovascular events and increased mortality are associated with cardiac autonomic nervous system dysfunction in the early stages of the systemic sclerosis (SSc), even prior to the development of cardiac fibrosis. The objective of the study was to evaluate the impact of a three-month exercise training regimen and a subsequent comparable period of detraining on the activity of the cardiac autonomic nervous system in patients with SSc. A total of forty patients with SSc were randomized to either the control group (Group COΝ) or the exercise training group (Group ET). Cardiopulmonary exercise testing was performed at baseline, three months later, and six months later to assess peak oxygen uptake (VO₂peak). They also had 24 h electrocardiogram monitoring for heart rate variability (HRV) and heart rate turbulence analysis. The following time-domain indices were evaluated in the context of HRV analysis: the standard deviation of NN intervals (SDNN), the root mean square of successive RR interval differences (rMSSD), and the percentage of successive RR intervals that differ by more than 50 ms (pNN50). Additionally, regarding the frequency-domain indicators, the low-frequency (LF) and high-frequency (HF) components, as well as the LF/HF ratio, were evaluated. Independent t-tests and Chi-square tests were used for baseline comparisons, while two-way repeated measures ANOVA with Bonferroni post hoc tests assessed changes over time and between groups. Linear and multiple regression analyses were conducted to explore relationships among variables and identify predictors of HRV indices and VO₂peak. Group ET implemented a three-month mixed-type exercise training program, while Group COΝ received standard care. Group ET improved indices of vagal activity [rMSSD by 32.6% (p = 0.017), pNN50 by 57.1% (p = 0.01) and HF by 20.1% (p = 0.01)] and sympathovagal activity [SDNN by 15.5% (p = 0.002) and LF/HF by 12.03% (p = 0.004)] after three months. Exercising patients also increased their VO₂peak by 20.8% (p = 0.001). A robust positive correlation was observed between ΔVO₂peak and ΔSDNN (r = 0.754, p < 0.001). After three months, there was no statistically significant difference in the VO₂peak or any HRV index in the group COΝ. Compared to the baseline values, there was no statistically significant difference in group ET at 6 months, whereas the control group exhibited a decline. In summary, a three-month mixed-type exercise training program can enhance the cardiorespiratory efficiency and cardiac autonomic nervous system function of patients with SSc, as well as alleviate the deterioration that arises following the detraining period.

Background: Vascular dysfunction and its concomitant multi-organ involvement, including cardiac involvement, affects prognosis in systemic sclerosis (SSc) patients. Regular exercise has demonstrated to be able to improve vascular function in SSc. However, the effects of an exercise program on the heart and specifically in right ventricular (RV) morphology and function in SSc have yet to be explored. The study aimed to examine whether a 3-month combined exercise program can affect RV morphology and function in SSc patients. Methods: Twenty-eight SSc patients were randomly allocated to either the exercise training (ET) or the control (CON) group. Baseline and follow-up assessments consisted of a cardiopulmonary exercise test along with both a conventional and a two-dimensional speckle tracking echocardiography (2DSTE) focused on RV morphology and function. Following the baseline assessments, Group ET participated in a supervised combined exercise program for 12 weeks, while group CON received their usual care. Results: The ET group demonstrated increases in peak oxygen consumption by 25.1% (p < 0.001), global RV free wall longitudinal systolic strain by 6.69% (p < 0.03), RV free wall longitudinal systolic strain of the basal segment by 13.5% (p < 0.001), and global RV four-chamber longitudinal systolic strain by 6.76% (p < 0.03) following the exercise program. No differences were observed in group CON. Conclusions: Combined exercise improved cardiorespiratory efficiency and indices of RV systolic function, as assessed by the 2DSTE, in SSc patients.