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.

Background: Currently, there are no data on the association between right ventricular (RV) structure and ECG changes specific for idiopathic pulmonary arterial hypertension (IPAH). Therefore, we aimed to assess the accuracy of the recommended ECG criteria for predicting RV hypertrophy (RVH) and dilation in patients with IPAH. Methods and Results:Twelve-lead ECG and cardiovascular magnetic resonance imaging (CMR) were performed in 23 consecutive patients with IPAH aged 49.8±16.3 years. ECG criteria were referred to RV mass index and RV end-diastolic volume index as measured by CMR. Only the ECG voltage criteria based on R wave amplitude in lead V1, R wave amplitude in aVR, P wave amplitude in II and ventricular activation time in V1 were useful for differentiating between patients with and without RVH. A ventricular activation time in lead V1 of <0.01 s excluded RVH, whereas R in V1 >6 mm, R:S in V1 >1, R in aVR >4 mm, R:S in V5 to R:S in V1 <0.04 and P in II >2.5 mm confirmed the diagnosis. Only the ventricular activation time in V1 correlated with RV dilation and when >0.045 s confirmed its diagnosis. Conclusions:Only a few of the recommended ECG criteria proved to be useful in the diagnosis of RVH or RV dilation in patients with IPAH. Changes in the cut-off values improved their accuracy. (Circ J 2012; 76: 1744 - 1749

INTRODUCTION    Interpretation of the electrocardiogram (ECG) is an essential skill in most medical specialties; however, the best method of teaching how to read ECGs has not been determined. OBJECTIVES    The aim of the study was to compare the effectiveness of collaborative (C‑eL) and self (S‑eL) e‑learning of ECG reading among medical students. PATIENTS AND METHODS    A total of 60 fifth‑year medical students were randomly assigned to the C‑eL and S‑eL groups. S‑eL students received 15 ECG recordings with a comprehensive description by email (one every 48 hours), while C‑eL students received the same ECG recordings without description. C‑eL students were expected to analyze each ECG together within the subgroups using an internet platform and to submit the interpretation within 48 hours. Afterwards, they received a description of each ECG. C‑eL students' activity was assessed based on the number of words written on the internet platform during discussion. A final test consisted of 10 theoretical questions and 10 ECG recordings. The final score was a sum of points obtained for the interpretation of ECG recordings. The main endpoint of the study was the number of students whose final score was 56% or higher. RESULTS    The final test was completed by 53 students (88.3%). The main endpoint was achieved in 20 C‑eL students (77%) and in 13 S‑eL students (48.1%), P = 0.03. The final score was 6.4 (interquartile range [IQR], 5.8-7.6) in the C‑eL group and 5.6 (IQR, 4.2-7.2) in the S‑eL group, P = 0.04. It correlated with the results of the theoretical test and students' activity during C‑eL (r = 0.42, P = 0.002 and r = 0.4, P = 0.04, respectively). CONCLUSIONS    C‑eL of ECG reading among fifth‑year medical students is superior to S‑eL.

Introduction Several diagnostic tests have been recommended for risk assessment in pulmonary hypertension (PH), but the role of electrocardiography (ECG) in monitoring of PH patients has not been yet established. Therefore the aim of the study was to evaluate which ECG patterns characteristic for pulmonary hypertension can predict hemodynamic improvement in patients treated with targeted therapies. Methods Consecutive patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) were eligible to be included if they had had performed two consecutive right heart catheterization (RHC) procedures before and after starting of targeted therapies. Patients were followed up from June 2009 to July 2017. ECG patterns of right ventricular hypertrophy according to American College of Cardiology Foundation were assessed. Results We enrolled 80 patients with PAH and 11 patients with inoperable CTEPH. The follow-up RHC was performed within 12.6 ± 10.0 months after starting therapy. Based on median change of pulmonary vascular resistance, we divided our patients into two subgroups: with and without significant hemodynamic improvement. RV1, max⁡RV1,2 + max⁡SI,aVL − SV1, and PII improved along with the improvement of hemodynamic parameters including PVR. They predicted hemodynamic improvement with similarly good accuracy as shown in ROC analysis: RV1 (AUC: 0.75; 95% CI: 0.63–0.84), PII (AUC: 0.67, 95% CI: 0.56–0.77), and max⁡RV1,2 + max⁡SI,aVL − SV1 (0.73; 95% CI: 0.63–0.82). In Cox regression only change in RV1 remained significant mortality predictor (HR: 1.12, 95% CI: 1.01–1.24). Conclusion Electrocardiogram may be useful in predicting hemodynamic effects of targeted therapy in precapillary pulmonary hypertension. Decrease of RV1, max⁡RV1,2 + max⁡SI,aVL − SV1, and PII corresponds with hemodynamic improvement after treatment. Of these changes a decrease of R wave amplitude in V1 is associated with better survival.

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,

BackgroundChronic anticoagulation is a standard of care in idiopathic pulmonary arterial hypertension (IPAH). However, hemostatic abnormalities in this disease remain poorly understood. Therefore, we aimed to study markers of thrombogenesis and fibrinolysis in patients with IPAH.MethodsWe studied 27 consecutive patients (67% female) with IPAH aged 50.0 years (IQR: 41.0 - 65.0) and 16 controls without pulmonary hypertension. Prothrombin fragment 1+2 (F1+2) and thrombin-antithrombin (TAT) complexes were measured to assess thrombogenesis; tissue-type plasminogen activator (tPA) antigen and plasmin-anti-plasmin complex to characterize activation of fibrinolysis; plasminogen activator inhibitor 1 (PAI-1) to measure inhibition of fibrinolysis; and endothelin-1 (ET-1) and interleukin-6 (IL-6) to assess endothelial activation and systemic inflammation, respectively. In addition, in treatment-naive IPAH patients these markers were assessed after 3 months of PAH-specific therapies.ResultsTPA (10.1[6.8-15.8] vs 5.2[3.3-7.3] ng/ml, p<0.001), plasmin-anti-plasmin (91.5[60.3-94.2] vs 55.8[51.1-64.9] ng/ml, p<0.001), IL-6 (4.9[2.5-7.9] vs 2.1[1.3-3.8] pg/ml, p=0.001) and ET-1 (3.7 [3.3-4.5] vs 3.4[3.1-3.5], p= 0.03) were higher in patients with IPAH than in controls. In IPAH patients plasmin-anti-plasmin and tPA correlated positively with IL-6 (r=0.39, p=0.04 and r=0.63, p<0.001, respectively) and ET-1 (r=0.55, p=0.003 and r=0.59, p=0.001, respectively). No correlation was found between tPA or plasmin-anti-plasmin and markers of thrombogenesis. Plasmin-anti-plasmin decreased after 3 months of PAH specific therapy while the other markers remained unchanged.ConclusionsIn the present study we showed that markers of fibrynolysis were elevated in patients with IPAH however we did not find a clear evidence for increased thrombogenesis in this group of patients. Fibrinolysis, inflammation, and endothelial activation were closely interrelated in IPAH.

IntroductionMetabolic alterations have been recently associated with onset and progression of idiopathic pulmonary arterial hypertension (IPAH). We aimed to determine the prevalence and prognostic role of cardiovascular risk factors in patients with IPAH.Material and methodsBetween February 2009 and January 2015 we recruited consecutive IPAH patients. Clinical assessment included medical history, fasting glucose, lipid profile, N-terminal pro-brain natriuretic peptide concentration, 6-minute walk test distance, WHO functional class and hemodynamic evaluation. Patients’ risk was estimated based on the Swedish PAH Register grading system.ResultsThe study group included 61 IPAH patients, and the control group included 2413 Polish residents. When compared to the general population, IPAH patients had lower low-density lipoprotein cholesterol (LDL-C) and a higher triglyceride to high-density lipoprotein cholesterol (TG/HDL-C) ratio. Female patients were characterized by elevated glucose level, higher prevalence of diabetes and lower HDL-C than controls. PAH severity grade correlated positively with age and TG/HDL-C ratio (R = 0.29, p = 0.02) and inversely with LDL-C (R = –0.28, p = 0.03) and HDL-C (R = –0.39, p = 0.02) concentrations. After a follow-up of 48 (23–79) months we recorded 28 deaths in the IPAH group. In the regression analysis lower LDL-C (p = 0.002) and HDL-C (p = 0.0002) levels, and higher TG/HDL-C ratio (p = 0.003) and glucose level (p = 0.003) were associated with all-cause mortality after adjustment for age, sex or PAH severity grade.ConclusionsPatients with IPAH are characterized by an altered profile of lipid and glucose metabolism. Lowered levels of LDL-C and HDL-C and increased TG/HDL-C ratio correlate with disease severity and together with elevated plasma glucose level predict poor survival in IPAH.

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