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Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies. Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry. Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment. Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH. Clinical Trial Registration: clinicaltrials.gov/ct2/show/NCT03959748

Background: Percutaneous pulmonary interventions require extensive and accurate navigation planning and guidance, especially in regard to the three-dimensional (3D) relationships between anatomical structures.In this study, we are demonstrating the feasibility of novel visualization techniques: 3D printing (3DP) and augmented reality (AR) in planning transcatheter pulmonary interventions.Methods: Two patients were qualified for balloon pulmonary angioplasty (BPA) for treatment of chronic thromboembolic pulmonary hypertension (CTEPH) and stent implantation for pulmonary artery stenosis, respectively. Computed tomography images of both patients were processed with segmentation algorithms and subsequently submitted to 3D modelling software. Microsoft HoloLens ® AR headsets with dedicated CarnaLife Holo ® software were utilized to display surface and volume rendering of pulmonary vessels as holograms.Results: Personalized life-sized models of the same structures were additionally 3D-printed for preoperative planning. Holograms were shown to physicians throughout the procedure and were used as a guidance and navigation tool. Operative team was able to manipulate the hologram and multiple users of the AR system could share the same image in real time. Clinicians expressed their satisfaction with the quality of imaging and potential clinical benefits.Conclusions: This study reports the potential value of AR in pulmonary interventions, however, prospective trials need to be conducted to decide on whether novel 3D visualization techniques affect perioperative treatment and outcomes.

(1) Background: Prostacyclin analogues (epoprostenol, treprostinil, and iloprost) induce vasodilation in pulmonary arterial hypertension (PAH) but also inhibit platelet function. (2) Objectives: We assessed platelet function in PAH patients treated with prostacyclin analogues and not receiving prostacyclin analogues. (3) Methods: Venous blood was collected from 42 patients treated with prostacyclin analogues (49.5 ± 15.9 years, 81% female) and 38 patients not receiving prostacyclin analogues (55.5 ± 15.6 years, 74% female). Platelet reactivity was analyzed by impedance aggregometry using arachidonic acid (AA; 0.5 mM), adenosine diphosphate (ADP; 6.5 µM), and thrombin receptor-activating peptide (TRAP; 32 µM) as agonists. In a subset of patients, concentrations of extracellular vesicles (EVs) from all platelets (CD61+), activated platelets (CD61+/CD62P+), leukocytes (CD45+), and endothelial cells (CD146+) were analyzed by flow cytometry. Platelet-rich thrombus formation was measured using a whole blood perfusion system. (4) Results: Compared to controls, PAH patients treated with prostacyclin analogues had lower platelet reactivity in response to AA and ADP (p = 0.01 for both), lower concentrations of platelet and leukocyte EVs (p ≤ 0.04), delayed thrombus formation (p ≤ 0.003), and decreased thrombus size (p = 0.008). Epoprostenol did not affect platelet reactivity but decreased the concentrations of platelet and leukocyte EVs (p ≤ 0.04). Treprostinil decreased platelet reactivity in response to AA and ADP (p ≤ 0.02) but had no effect on the concentrations of EVs. All prostacyclin analogues delayed thrombus formation and decreased thrombus size (p ≤ 0.04). (5) Conclusions: PAH patients treated with prostacyclin analogues had impaired platelet reactivity, EV release, and thrombus formation, compared to patients not receiving prostacyclin analogues.