Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: a multicentre registry.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is a promising therapy for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy. AIMS: The present study aimed to evaluate the safety and efficacy of BPA for CTEPH using the first multicentre registry of a single European country. METHODS: Data were obtained from the Database of Pulmonary Hypertension in the Polish Population (NCT03959748), a prospective, multicentre registry of adult and paediatric pulmonary arterial hypertension (PAH) and CTEPH, for a total of 236 patients with confirmed CTEPH (124 women; mean age 67 years) who underwent 1,056 BPA procedures at eight institutions in Poland. RESULTS: In 156 patients who underwent follow-up assessments after a median of 5.9 (IQR: 3.0-8.0) months after final BPA, the mean pulmonary arterial pressure decreased from 45.1±10.7 to 30.2±10.2 mmHg (p<0.001) and pulmonary vascular resistance from 642±341 to 324±183 dynes (p<0.001), and the six-minute walking test (6MWT) improved from 341±129 to 423±136 m (p<0.001). Pulmonary injury related to the BPA procedure occurred in 6.4% of all sessions. Eighteen patients (7.6%) died during follow-up, including 4 (1.7%) who died within 30 days after BPA. Overall survival was 92.4% (95% confidence interval [CI]: 87.6%-94.9%) three years after the initial BPA procedure. CONCLUSIONS: This multicentre registry confirmed significant improvement of haemodynamic, functional, and biochemical parameters after BPA. Complication rates were low and overall survival comparable to the results of another registry. Therefore, BPA may be an important therapeutic option in patients with CTEPH in Poland.

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease.

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females ≥65 years old. The most frequent type of PAH was idiopathic (n = 444; 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, n = 356; 36.7%), and PAH associated with connective tissue disease (CTD-PAH, n = 132; 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.

Expert opinion on the creating and operating of the regional Pulmonary Embolism Response Teams (PERT). Polish PERT Initiative.

Pulmonary Embolism Response Team (PERT) is a multidisciplinary team established to stratify risk and choose optimal treatment in patients with acute pulmonary embolism (PE). Established for the first time at Massachusetts General Hospital in 2013, PERT is based on a concept combining a Rapid Response Team and a Heart Team. The growing role of PERTs in making individual therapeutic decisions is identified, especially in hemodynamically unstable patients with contraindications to thrombolysis or with co-morbidities, as well as in patients with intermediate-high risk in whom a therapeutic decision may be difficult. The purpose of this document is to define the standards of PERT under Polish conditions, based on the experience of teams already operating in Poland, which formed an agreement called the Polish PERT Initiative. The goals of Polish PERT Initiative are: improving the treatment of patients with PE at local, regional and national levels, gathering, assessing and sharing data on the effectiveness of PE treatment (including various types of catheter-directed therapy), education on optimal treatment of PE, creating expert documents and supporting scientific research, as well as cooperation with other communities and scientific societies.

An implantable pump Lenus pro® in the treatment of pulmonary arterial hypertension with intravenous treprostinil.

BACKGROUND: Subcutaneous treprostinil is a prostacyclin analogue used to treat pulmonary arterial hypertension (PAH). Due to local pain it can cause a deterioration of heart related quality of life (HRQoL) or even abandonment of treatment. The aim of this paper was to assess the feasibility of treatment with intravenous treprostinil administered by means of the Lenus Pro® implantable pump. METHODS: This was a retrospective, multi-center study involving 12 patients (8 females) with PAH treated with a subcutaneous infusion of treprostinil with intolerable pain at the infusion site. Clinical evaluation, including HRQoL assessment with SF-36 questionnaire was performed, before pump implantation and 2-9 months after. The median time of follow-up time was 14 months (4-29 months). RESULTS: After implantation of the Lenus Pro® pump, no statistically significant changes were observed in the 6-min walking distance and NT-proBNP. After implantation 50% of patients were in II WHO functional class (33% before, p = 0,59). There was a significant improvement in HRQoL within the Physical Component Score (28 ± 7 vs 38 ± 8 pts., p < 0,001) and in specific domains of SF-36 form: physical role (31 ± 7 pts. vs. 41 ± 12 pts., p = 0,03), bodily pain (31 ± 12 vs. 50 ± 14 pts., p = 0,02), and vitality (37 ± 8 pts. vs. 50 ± 14 pts., p = 0,03). During the periprocedural period, one patient developed a recurrent haematoma at the implantation site. During follow-up in one patient, the drug delivering cannula slipped out of the subclavian vein, what required repositioning repeated twice, and in another patient an unexpected increase in the drug administration rate was observed. CONCLUSIONS: In patients with PAH who do not tolerate subcutaneous infusion of treprostinil, the use of the Lenus Pro® implantable pump results in significant subjective improvement of vitality and physical aspect of the HRQoL with acceptable safety profile.

Value of perfusion-weighted MR imaging in the assessment of early cerebral alterations in neurologically asymptomatic HIV-1-positive and HCV-positive patients.

BACKGROUND AND PURPOSE: Asymptomatic central nervous system (CNS) involvement occurs in the early stage of the human immunodeficiency virus (HIV) infection. It has been documented that the hepatitis C virus (HCV) can replicate in the CNS. The aim of the study was to evaluate early disturbances in cerebral microcirculation using magnetic resonance (MR) perfusion-weighted imaging (PWI) in asymptomatic HIV-1-positive and HCV-positive patients, as well as to assess the correlation between PWI measurements and the clinical data. MATERIALS AND METHODS: Fifty-six patients: 17 HIV-1-positive non-treated, 18 HIV-1-positive treated with combination antiretroviral therapy (cART), 7 HIV-1/HCV-positive non-treated, 14 HCV-positive before antiviral therapy and 18 control subjects were enrolled in the study. PWI was performed with a 1.5T MR unit using dynamic susceptibility contrast (DSC) method. Cerebral blood volume (CBV) measurements relative to cerebellum (rCBV) were evaluated in the posterior cingulated region (PCG), basal ganglia (BG), temporoparietal (TPC) and frontal cortices (FC), as well as in white matter of frontoparietal areas. Correlations of rCBV values with immunologic data and liver histology activity index (HAI) were analyzed. RESULTS: Significantly lower rCBV values were found in the right TPC and left FC as well as in PCG in HIV-1-positive naïve (p = 0.009; p = 0.020; p = 0.012), HIV-1 cART treated (p = 0.007; p = 0.009; p = 0.033), HIV-1/HCV-positive (p = 0.007; p = 0.027; p = 0.045) and HCV-positive patients (p = 0.010; p = 0.005; p = 0.045) compared to controls. HIV-1-positive cART treated and HIV-1/HCV-positive patients demonstrated lower rCBV values in the right FC (p = 0.009; p = 0.032, respectively) and the left TPC (p = 0.036; p = 0.005, respectively), while HCV-positive subjects revealed lower rCBV values in the left TPC region (p = 0.003). We found significantly elevated rCBV values in BG in HCV-positive patients (p = 0.0002; p<0.0001) compared to controls as well as to all HIV-1-positive subjects. There were no significant correlations of rCBV values and CD4 T cell count or HAI score. CONCLUSIONS: PWI examination enables the assessment of HIV-related as well as HCV-related early cerebral dysfunction in asymptomatic subjects. HCV-infected patients seem to reveal the most pronounced perfusion changes.

Evaluation of early cerebral metabolic, perfusion and microstructural changes in HCV-positive patients: a pilot study.

BACKGROUND & AIMS: The aim of the study was to evaluate early metabolic perfusion, and microstructural cerebral changes in patients with the hepatitis C virus (HCV) infection and normal appearing brain on plain MR using advanced MR techniques, as well as to assess correlations of MR measurements with the liver histology activity index (HAI). METHODS: Fifteen HCV-positive patients and 18 control subjects underwent single voxel MR spectroscopy (MRS), perfusion weighted imaging (PWI), and diffusion tensor imaging (DTI), using a 1.5T MR unit. MRS metabolite ratios (NAA/Cr, Cho/Cr, mI/Cr) were calculated. PWI values of relative cerebral blood volume (rCBV) were assessed from 8 areas including several cortical locations, basal ganglia, and fronto-parietal white matter. DTI fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained from 14 white matter tracts. RESULTS: Compared to controls, HCV-positive patients showed significantly (p < 0.05) lower NAA/Cr ratios within frontal and parietal white matters, lower rCBV values within frontal and temporo-parietal cortices, decreased FA values, as well as increased ADC values in several white matter tracts. We also found elevated rCBV values in basal ganglia regions. The increase in mI/Cr and Cho/Cr ratio was correlated with a higher HAI score. CONCLUSIONS: The results of advanced MR techniques indicate neurotoxicity of HCV reflected by neuronal impairment within white matter, cortical hypoperfusion, and disintegrity within several white matter tracts. Hyperperfusion in basal ganglia may be an indicator of brain inflammation in HCV patients. Our findings may suggest a biologic link between HCV-related liver disease and cerebral dysfunction.

Evaluation of metabolic changes within the normal appearing gray and white matters in neurologically asymptomatic HIV-1-positive and HCV-positive patients: magnetic resonance spectroscopy and immunologic correlation.

OBJECTIVE: The aim of the study was to evaluate early metabolic changes using proton MR spectroscopy (MRS) in asymptomatic HIV-1-positive and HCV-positive patients without abnormalities in the structural MR examination. METHODS: Sixty-five asymptomatic patients: 21 HIV-1-positive naive, 20 HIV-1-positive with combination antiretroviral therapy (cART), 9 HIV-1/HCV-positive naive, 15 HCV-positive naive and 18 normal subjects were enrolled in the study. The MRS examinations were performed with a 1.5T MR scanner. Voxels were located in the following regions: posterior cingulate gyrus (PCG), anterior cingulate gyrus (ACG), parietal white matter (PWM), left basal ganglia (BG) and frontal white matter (FWM). The NAA/Cr, Cho/Cr, mI/Cr ratios and correlations of MRS measurements with the immunologic data were analyzed. RESULTS: There was a significant decrease (p<0.05) of the NAA/Cr ratios in PCG, ACG and PWM regions in HIV-1-positive cART treated patients compared to the normal subjects. The significantly decreased NAA/Cr ratios in PWM and FWM were observed in HCV infected patients. The subjects with HIV-1/HCV co-infection revealed significantly lower NAA/Cr ratios in the ACG area. Other metabolite ratios in all analyzed regions, as well as the NAA/Cr ratios in BG showed no significant differences. The decrease of CD4n T cell count was associated with the decease of the NAA/Cr ratio in the PCG area and the increase of Cho/Cr ratio in the FWM region. CONCLUSIONS: The metabolic changes - reduction of NAA/Cr ratios are most pronounced in HIV-1-positive patients using cART. The low CD4n T cell count is a risk factor for neurocognitive impairment in HIV-1-positive patients.

Analysis of metabolic changes of brain in HIV-1 seropositive patients with proton magnetic resonance spectroscopy.

BACKGROUND: Asymptomatic central nervous system involvement may occur in the early stages of the HIV infection. The aim of the study was to evaluate early brain metabolic changes by means of proton MR spectroscopy (H1MRS) in the HIV-1 seropositive patients without neurological deficits or significant abnormalities in the plain MR study. MATERIAL/METHODS: The H1MRS examinations were performed with the use of a MR GE Signa 1,5T system. There were 39 subjects examined, aged 21 to 57 years (mean age 35 years) were examined, including 25 patients infected with HIV-1 and 14 healthy volunteers who constituted a control group. The examinations were performed using the Single Voxel Spectroscopy technique with the PRESS sequence, with following parameters: TR=1500 ms, TE=35 ms, number of acquisitions =128, time of acquisition =3 min. 43 sec. Voxels of 8 cm(3) (20×20×20 mm) in size were located in the following 5 regions: posterior cingulate gyrus, grey matter of the frontal area, left basal ganglia, white matter of the left parietal area and white matter of the frontal area. The NAA/Cr, Cho/Cr, mI/Cr ratios in the defined regions of interest were statistically analyzed. RESULTS: There was a statistically significant decrease (p<0.05) in the NAA/Cr ratios in the posterior cingulate area and white matter of the left parietal area in HIV-1 seropositive patients, as compared to the control group. Other metabolite ratios in all the above mentioned locations showed no statistically significant differences, as was also the case for NAA/Cr ratios in grey matter of the frontal area, left basal ganglia and white matter of the frontal area. CONCLUSIONS: The reduction of NAA/Cr values revealed in H1MRS studies suggests loss of neurons/neuronal activity in the posterior cingulate area and white matter of the left parietal area, in patients with HIV-1 at the stage before clinical manifestations of retroviral infection and structural changes in the plain MR study. This may reflect a direct neurotropic activity of HIV.