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.

Right heart catheterisation (RHC) is the 'gold standard' for haemodynamic assessment of the pulmonary circulation. For the diagnosis of pulmonary hypertension (PH), the guidelines of the European Society of Cardiology require a mean pulmonary arterial pressure ≥ 25 mm Hg to be confirmed by direct haemodynamic measurement. Additionally, RHC provides a lot of valuable information about the differential diagnosis and severity of PH, and also helps determine the patient's prognosis. Acute vasoreactivity testing performed in patients with pulmonary arterial hypertension is intended to identify the group of patients who should be treated with calcium channel blockers. Patients referred for heart transplantation require advanced pulmonary vascular disease to be ruled out either on resting examination or during vasoreactivity testing. RHC is a component of such interventional procedures as balloon atrial septostomy, closure of intracardiac shunts in congenital heart and great vessel defects, valvuloplasty for pulmonary valve stenosis, and pulmonary angioplasty. Pulmonary angiography is an examination recommended when selecting patients for pulmonary endarterectomy or balloon pulmonary angioplasty in thromboembolic PH. Due to the dynamic growth in the number of patients diagnosed with and treated for PH in Poland, the Boards of the Polish Cardiac Society's Working Group on Pulmonary Circulation and Association of Cardiovascular Interventions have undertaken a joint project to develop recommendations to standardise guidelines for RHC procedure, acute vasoreactivity testing and pulmonary angiography at cardiac wards and haemodynamic laboratories in Poland. This document has been prepared by experts delegated by the Working Group on Pulmonary Circulation and the Association of Cardiovascular Interventions, and subsequently approved by the Boards of both organs of the Polish Cardiac Society.

We report two patients with methylmalonic acidemia (MMA) in whom renal biopsy demonstrated interstitial nephritis, bringing the total of such reported cases to four. In addition, hypertension, observed in one of our patients, has not been previously reported as the presentation of renal disease in MMA. The etiology of interstitial nephritis in MMA did not appear to be due to urate nephropathy. To date, 15 patients with MMA have been reported with renal complications, including chronic renal failure, making it imperative that children with MMA have their renal status evaluated.

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

NCT03959748) is the first multicenter and prospective registry of adult and pediatric patients with PAH and CTEPH created in any of the Central-Eastern European countries. In the present report, we describe the design of the registry. Objectives of the BNP-PL registry The BNP-PL registry is an initiative of the Working Group on Pulmonary Circulation of the Polish Cardiac Society in cooperation with Polish PH reference centers 6-9 to assess prospectively the epidemiology, clinical course, and disease management of patients with PAH and CTEPH. The specific objectives of the study are shown in Supplementary material, Table S1. Introduction Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare types of pulmonary hypertension (PH). Consequently, patient registries are key instruments that provide data for clinical research and improve patient care and healthcare planning. 1 Currently, most of our knowledge on PAH and CTEPH epidemiology, management, and treatment outcomes come from registries originating in the Western populations .2-5 However, a global view on the epidemiology of PH reveals important geographical differences. 1 The Database of Pulmonary Hypertension in the Polish population (Baza Nadciśnienia Płucnego [BNP-PL]) (ClinicalTrials.gov identifier,

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Funding Acknowledgements Type of funding sources: None. Introduction Pulmonary arterial hypertension (PAH) is a rare disease, with an incidence of 15–50 cases per million annually. The disease is associated with further progress and worsening of symptoms despite pharmacotherapy. The severity of PAH symptoms and their effects reduce the patients" quality of life (QoL). Numerous studies show that the quality of life is related to the therapeutic effect of the treatment. Available studies demonstrate that patients QoL deteriorates as the condition progresses. Consequently, the goals of PAH therapy have expanded from increasing survival to improving health-related quality of life. The aim of the study was to determine the clinical and sociodemographic factors influencing the quality of life in PAH patients. Methods The study involved 55 patients with PAH who were examined using the Polish version of the PAH-SYMPACT quality of life questionnaire. The PAH-SYMPACT questionnaire results were correlated with several  variables. Results It was shown that the quality of life correlates significantly (p˂0,05) with WHO Functional Class, NT-pro BNP (N-terminal prohormone of brain natriuretic peptide) value, elderly age (the higher the value, the worse the QoL). On the other hand, the lower the 6MWT (6 minuts walk test) result, the worse the QoL. Moreover, the intensity of the accompanying PAH symptoms, such as chest pain, dyspnoea, arrhythmias, fatigue and weakness were associated with a lower quality of life in patients (p < 0,05). Conclusion Patients" quality of life correlates with many variables, both clinical and sociodemographic. In addition, there is a correlation between the quality of life of patients with PAH and the severity of several symptoms associated with this condition. Determination of factors affecting the quality of life of patients with PAH enables the selection of a more comprehensive therapy.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept. Methods and results: A total of 160 patients were consulted by the CTEPH team between December 2015 and September 2018. Patient baseline characteristics, CTEPH team decisions and implementation rates of diagnostic and therapeutic procedures were analysed. Change in World Health Organization (WHO) functional class and survival rates were evaluated by treatment strategy. A total of 51 (32%) patients were assessed as operable and 109 (68%) were deemed inoperable. Thirty-one (61% of operable patients) underwent PEA. Patients treated with PEA, BPA(+MT) and MT alone were 50.9 ± 14.7, 62.9 ± 15.1 and 68.9 ± 12.7 years old, respectively. At the follow-up, PEA patients had the highest WHO functional class improvement. Patients treated with BPA(+MT) had significantly better survival than PEA (p = 0.04) and MT patients (p = 0.04; 2-year survival of 92%, 79% and 79%, respectively). Conclusions:The CTEPH team ensures that necessary diagnostic procedures are performed. A relatively low proportion of patients was assessed by the CTEPH team as operable and underwent surgery, which in survivors resulted in the best functional improvement. Although patients undergoing BPA(+MT) were older than patients treated with PEA, their survival was better than patients subjected to PEA or MT alone. The reviews of this paper are available via the supplemental material section.