5 research outputs found
Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry
Introduction: Pulmonary hypertension (PH) is a common complication in
patients with congenital heart disease (CHD), aggravating the natural,
post-operative, or post-interventional course of the underlying anomaly.
The various CHDs differ substantially in characteristics, functionality,
and clinical outcomes among each other and compared with other diseases
with pulmonary hypertension. Objective: To describe current management
strategies and outcomes for adults with PH in relation to different
types of CHD based on real-world data. Methods and results: COMPERA
(Comparative, Prospective Registry of Newly Initiated Therapies for
Pulmonary Hypertension) is a prospective, international PH registry
comprising, at the time of data analysis, >8200 patients with various
forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to
CHD, who were included between 2007 and 2018 in 49 specialized centers
for PH and/or CHD located in 11 European countries. At enrollment, the
patients’ median age was 44 years (67% female), and patients had either
pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital
left heart or aortic disease, or miscellaneous other types of CHD. Upon
inclusion, targeted therapies for pulmonary arterial hypertension (PAH)
included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin
analogues, and soluble guanylate cyclase stimulators. Eighty patients
with Eisenmenger syndrome were treatment-naive. While at inclusion the
primary PAH treatment for the cohort was monotherapy (70% of patients),
with 30% of the patients on combination therapy, after a median
observation time of 45.3 months, the number of patients on combination
therapy had increased significantly, to 50%. The use of oral
anticoagulants or antiplatelets was dependent on the underlying
diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after
follow-up and receiving targeted PAH therapy (n = 511), 91 patients died
over the course of a 5-year follow up. The 5-year Kaplan-Meier survival
estimate for CHD associated PH was significantly better than that for
idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH
group, survival estimates differed particularly depending on the
underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD,
the overall survival of patients with CHD associated PH was dependent on
the underlying diagnosis and treatment status, but was significantly
better as than that for idiopathic PAH. Nevertheless, overall survival
of patients with PAH due to CHD was still markedly reduced compared with
survival of patients with other types of CHD, despite an increasing
number of patients on PAH-targeted combination therapy
Effects of 60-day bed rest with and without exercise on cellular and humoral immunological parameters
Exercise at regular intervals is assumed to have a positive effect on immune functions. Conversely, after spaceflight and under simulated weightlessness (e.g., bed rest), immune functions can be suppressed. We aimed to assess the effects of simulated weightlessness (Second Berlin BedRest Study; BBR2-2) on immunological parameters and to investigate the effect of exercise (resistive exercise with and without vibration) on these changes. Twenty-four physically and mentally healthy male volunteers (20-45 years) performed resistive vibration exercise (n=7), resistance exercise without vibration (n=8) or no exercise (n=9) within 60 days of bed rest. Blood samples were taken 2 days before bed rest, on days 19 and 60 of bed rest. Composition of immune cells was analyzed by flow cytometry. Cytokines and neuroendocrine parameters were analyzed by Luminex technology and ELISA/RIA in plasma. General changes over time were identified by paired t-test, and exercise-dependent effects by pairwise repeated measurements (analysis of variance (ANOVA)). With all subjects pooled, the number of granulocytes, natural killer T cells, hematopoietic stem cells and CD45RA and CD25 co-expressing T cells increased and the number of monocytes decreased significantly during the study; the concentration of eotaxin decreased significantly. Different impacts of exercise were seen for lymphocytes, B cells, especially the IgD(+) subpopulation of B cells and the concentrations of IP-10, RANTES and DHEA-S. We conclude that prolonged bed rest significantly impacts immune cell populations and cytokine concentrations. Exercise was able to specifically influence different immunological parameters. In summary, our data fit the hypothesis of immunoprotection by exercise and may point toward even superior effects by resistive vibration exercise.Cellular & Molecular Immunology advance online publication, 10 November 2014; doi:10.1038/cmi.2014.106