INTRODUCTION:Shortness of breath while bending and its more objective version, bending oxygen saturation index (BOSI), are the latest additions to pulmonary arterial hypertension (PAH) symptom and signs armamentarium. In this study, we aimed to evaluate the association between BOSI and clinical outcomes in patients with PAH and to explore its potential to complement current risk estimation schemes. METHODS:In this single-center, prospective, observational study, we enrolled patients with PAH who are under stable treatment. Baseline mortality risk was estimated using established risk schemes. Primary endpoint was defined as the combination of all-cause hospitalization and all-cause mortality at 1 year. The discriminative performance of BOSI was evaluated using ROC curve analysis. RESULTS:A total of 102 patients were enrolled into the study. BOSI was equal to or more than 3 in 33 patients (32.4%). Primary endpoint occurred in 20 (60.6%) in BOSI ≥3 group and 16 (23.2%) in BOSI <3 group (p < 0.001). ROC analysis showed that BOSI had a significant discriminative ability (AUC 0.687, p = 0.002). Cox regression analysis showed that a BOSI ≥3 was significantly associated with adverse events, even after adjustment for baseline risk estimated by the four most used risk schemes (REVEAL, REVEAL Lite, COMPERA, and European Society of Cardiology/European Respiratory Society risk scores). CONCLUSION:BOSI is independently associated with adverse events in patients with PAH and its addition to current risk scores may improve baseline risk estimation.

Medication non-adherence is common in patients with acute coronary syndrome (ACS) and may increase the risk of cardiac readmissions and mortality. To evaluate the effect of a clinical pharmacist-led, behavioural theory-based discharge service designed to promote medication adherence on 30-day cardiac readmissions (primary outcome) and on clinical, humanistic, and healthcare utilisation outcomes over 360 days in patients with ACS. In this single-centre, parallel-group randomised controlled trial, adult patients hospitalised with ACS were assigned by permuted block randomisation (block size 8) to the intervention or control group. The intervention comprised a clinical pharmacist-led, behavioural theory-based discharge service to promote medication adherence. The intervention consisted of medication reconciliation, medication review and patient counselling based on components of the behaviour change technique taxonomy and Health Belief Model. The primary outcome was 30-day hospital readmission for cardiac reasons. Secondary outcomes included all-cause and cardiac readmissions, emergency department visits, all-cause and cardiac mortality, medication adherence, LDL (low-density lipoprotein) target attainment, and quality of life over 360 days. A total of 167 patients were analysed (intervention: n = 80; control: n = 87). The primary outcome occurred in 0/80 (0.0

STUDY OBJECTIVES:We previously demonstrated that 80% of patients with chronic thromboembolic pulmonary hypertension (CTEPH) exhibit sleep-related breathing disorders (SBDs), primarily obstructive sleep apnea (OSA), followed by isolated sleep-related hypoxemia (ISRH). In this follow-up study, we aimed to assess the distribution of SBDs and predictors of echocardiography-based residual pulmonary hypertension (PH) risk after pulmonary endarterectomy (PEA) in the same cohort. METHODS:Overnight polysomnography and echocardiography were performed in 24 CTEPH patients approximately 112 ± 65 days after PEA. Residual PH risk was defined as peak tricuspid regurgitation velocity (TRV) > 2.8 m/s and systolic pulmonary artery pressure (SPAP) ≥ 30 mmHg. RESULTS:In the overall cohort, PEA significantly reduced TRV (from 3.7 ± 0.9 to 2.5 ± 0.9 m/s, p < 0.001) and SPAP (58.3 ± 26.9 to 29.8 ± 17.7 mmHg, p < 0.001). Among six patients with preoperative ISRH, two improved, while four developed OSA postoperatively. Of the 16 patients with preoperative OSA, 14 remained in the same category after surgery. Residual PH risk was observed in 13 patients (54.2%), all of whom had OSA, while no residual PH risk was detected in patients without SBDs (p = 0.003). In multivariate logistic regression, postoperative apnea-hypopnea index was independently associated with echo-based residual PH risk (odds ratio 1.19; 95% CI 1.02-1.38; p = 0.025), after adjustment for age, sex, body mass index, and baseline mean pulmonary arterial pressure. CONCLUSIONS:PEA improves pulmonary hemodynamics and resolves ISRH in some patients with CTEPH. However, residual PH risk remains frequent and is independently associated with OSA severity. These findings emphasize the importance of systematic screening, early diagnosis, and management of OSA to potentially mitigate residual PH risk and improve postoperative outcomes in CTEPH.

Patients with connective tissue diseases (CTDs) are at increased risk for the development of pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH), which is a specific subtype of PH characterized by progressive remodeling of precapillary pulmonary arterioles. Evidence suggests that early detection of PH through screening in this patient group may be associated with better outcomes. Various methods, including cardiac and thoracic imaging, blood biomarkers, pulmonary function tests, and composite algorithms, have been employed for screening of PH. Here, a multidisciplinary consensus group composed of 10 rheumatologists, 4 cardiologists, and 3 pulmonologists was formed with the objective of developing recommendations and a screening algorithm for PH in patients with CTD. A systematic literature review in the PubMed database focusing on the studies evaluating the performance of different screening methods on detecting PH in CTDs was conducted. The literature review identified 33 relevant articles after title, abstract, and full-text evaluation. The included studies had considerable heterogeneity regarding hemodynamic definitions of PH and PAH, the diagnostic cutoff values of screening methods utilized, and the symptom status of the patients. With the exception of 2 studies, the populations in included studies consisted of only patients with systemic sclerosis (SSc). Consensus-based recommendations and an algorithm prioritizing echocardiography for screening and early detection of PH in patients with SSc and patients with CTD exhibiting overlap features of SSc were developed based on literature data and incorporating the perspectives of group members. No recommendations could be made for asymptomatic patients with CTDs without overlap features of SSc due to limited data.

Background and Objectives: Comorbidities, the coexistence of additional conditions with a primary disease, are increasingly prevalent, complicating disease management and clinical outcomes. While CTEPH is a well-studied condition in terms of risk factors and outcomes, the specific impact of comorbidity burden on clinical presentation, treatment decisions, and survival remains insufficiently explored. This study aims to assess the prevalence and burden of comorbidities in CTEPH and to examine their associations with initial clinical characteristics, treatment allocation, and survival, stratified by pulmonary endarterectomy (PEA) status. Materials and Methods: We included 187 CTEPH patients from eight tertiary PH centers (2009–2020). Cardiovascular and non-cardiovascular comorbidities were identified and categorized as 0, 1–2, or ≥3. Their impact on baseline six-minute walk distance (6MWD), hemodynamic parameters, operability decision, and survival was assessed. Results: Comorbidities were prevalent (90%), with 49% of patients having three or more. Hypertension, diabetes, coronary artery disease, and chronic kidney disease (CKD) were associated with lower 6MWD. Hypertension, atrial fibrillation, left heart failure, and CKD were linked to elevated right atrial and pulmonary arterial wedge pressures. Comorbidities rendered 39% of anatomically operable patients ineligible for surgery. No single comorbidity predicted survival. Among PEA patients, those with ≥3 cardiovascular comorbidities had worse survival (p = 0.010). In contrast, the comorbidity burden did not impact survival in non-PEA patients. PEA surgery (HR 0.342, 95% CI 0.130–0.899, p = 0.030) and baseline 6MWD (HR 0.997, 95% CI 0.994–1.000, p = 0.036) were identified as independent predictors of mortality. Conclusions: A high comorbidity burden is common in CTEPH and influences functional status, hemodynamics, and operability decisions. It may worsen long-term outcomes after PEA but appears to be less prognostic in non-operated patients, where disease severity seems to be the primary determinant of outcomes. These findings underscore the importance of careful operability assessment and proactive comorbidity management.

This study we evaluated the association between preoperative clinical, respiratory, and hemodynamic parameters and the post-operative thrombus level in the pulmonary endarterectomy (PEA) material of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Our hypothesis was that whether the level of thrombus is associated with preoperative pulmonary functions. To the best of our knowledge, this is the first study evaluating the relationship between anatomical classification of endarterectomy materials with clinical, respiratory, and hemodynamic parameters. This single-center retrospective cross-sectional study included patients aged 18 and older diagnosed with CTEPH who had PEA at Marmara University School of Medicine Hospital Istanbul between 2013 and 2022. A total of 150 patients with CTEPH who had PEA procedure. Patients whose degree of vascular involvement is unclear were excluded (n = 4). Patients whose post-operative pathology compatible other than thrombi (sarcoma, etc.) were excluded n = 3 (Fig. 2). Patients’ preoperative clinical parameters [New York Heart Association (NYHA) Functional Class (FC), 6-minute walk distance (6MWD)], PFTs, DLCO values, echocardiographic data and hemodynamic parameters measured with right heart catheterization were evaluated along with the thrombi levels of PEA specimens. The study included 143 patients diagnosed with CTEPH who had PEA. When the distribution of thrombus materials was examined according to anatomical classification; 18.9

Chronic thromboembolic pulmonary disease (CTEPD) includes both chronic thromboembolic pulmonary hypertension (CTEPH) and disease without pulmonary hypertension. Although the main cause of CTEPH is mostly due to thromboembolic events, other rare non-thrombotic etiologies may also contribute to chronic pulmonary artery obstruction. This case series presents a unique observation of foreign material embolization related to prior sclerotherapy procedures, confirmed histopathologically after pulmonary endarterectomy (PEA). From a prospectively maintained database of 1,105 patients undergoing PEA between 2011 and 2025, four patients (three women and one man; median age 32.5 years, range: 28–41) with a history of sclerotherapy were identified. All underwent sclerotherapy for varicose vein treatment. They were referred for surgery with a preoperative diagnosis of CTEPD, with or without pulmonary hypertension. The final diagnosis was confirmed by histopathological examination of surgical specimens. All four patients had segmental or lobar perfusion defects and vascular obstruction consistent with organized embolic material. Preoperative mean pulmonary artery pressure (mPAP) was 24.3 ± 7.4 mmHg, and mean pulmonary vascular resistance (mPVR) was 219.3 ± 104.6 dyn·s/cm⁻⁵. Although the surgery was challenging because of difficulty establishing dissection plane, no perioperative morbidity or mortality occurred. Postoperative hemodynamic improvement was observed, with mPAP reduced to 16.3 ± 1.5 and mean PVR to 119.3 ± 45.8 dyn·s/cm⁻⁵ (p > 0.05). The mean six-minute walk test distance increased from 381.5 ± 63.2 m preoperatively to 470.0 ± 66.8 m after surgery (p > 0.05). Histopathological analysis confirmed the presence of sclerotherapy-related foreign material in all cases. All patients had unilateral lobar obstruction. During a median follow-up of 50 months, no mortality or recurrence of symptoms or pulmonary hypertension was observed. This report is the first case series to document a direct histopathological link between sclerotherapy and chronic pulmonary artery obstruction. These findings emphasize the need to consider iatrogenic etiologies in patients with unexplained pulmonary vascular disease and support the diagnostic and therapeutic value of PEA in selected cases.

Background Pulmonary endarterectomy is a potentially curative treatment option for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to identify predictors of short- and long-term outcomes after pulmonary endarterectomy for CTEPH patients, including age. Methods Patients who underwent surgery between March 2014 and January 2024 were included in the study. Perioperative and follow-up data were retrospectively studied, including age, in-hospital mortality, one- and five-year survival, and the length of intensive care unit and hospital stays after pulmonary endarterectomy. Results In total, 834 consecutive patients (mean age 51±15.3 years) underwent pulmonary endarterectomy and were included in the analysis. The in-hospital mortality rate was 7.8% (n = 65), while overall mortality rates at one and five years were 10.6% and 11.3%, respectively. The in-hospital mortality rate was 6.7% for patients < 70 years compared to 12.4% for patients ≥70 years (p=0.029). In the multivariate analysis of mortality, age (p=0.007), and length of intensive care unit stay (p= 0.028) emerged as independent predictors of in-hospital mortality, while the Charlson Comorbidity Index (p<0.001) and six-minute walk distance (p=0.005) were also significant predictors of one-year survival. Conclusion Despite higher short-term mortality rates, pulmonary endarterectomy was feasible and well tolerated among elderly patients. Despite surgical advancements, careful patient selection remains crucial, particularly in the presence of comorbidities. Significant clinical and hemodynamic improvements were observed, along with favorable long-term survival outcomes.

Background: Although high left ventricular filling pressures [left ventricular (LV) enddiastolic pressure or pulmonary capillary wedge pressure (PCWP)] are widely taken as surrogates for LV diastolic dysfunction, the actual distending pressure that governs LV diastolic stretch is transmural pressure difference (∆P TM ).Clinically, preferring ∆P TM over PCWP may improve diagnostic and therapeutic decision-making.We aimed to compare the clinical implications of diastolic function characterization based on PCWP or ∆P TM . Methods:We retrospectively screened our hospital database for adult patients with a clinical diagnosis of heart failure who underwent right heart catheterization.Echocardiographic diastolic dysfunction was graded according to the current guidelines.LV end-diastolic properties were assessed with construction of complete end-diastolic pressure-volume relationship (EDPVR) curves using the single-beat method.Survival status was checked via the electronic national health-care system.Results: A total of 693 cases were identified in our database; the final study population comprised 621 cases.∆P TM -based, but not PCWP-based, EDPVR diastolic stiffness constants were significantly predictive of advanced diastolic dysfunction.PCWP-based diastolic stiffness constants were not able to predict 5-year mortality, whereas ∆P TM -based EDPVR stiffness constants and volumes all turned out to have significant predictive power for 5-year mortality. Conclusion:Left ventricular diastolic function assessment can be improved using ∆P TM instead of PCWP.As ∆P TM ultimately linked to right-sided functions, this approach emphasizes the limitations of taking LV diastolic function as an isolated phenomenon and underlines the need for a complete hemodynamic assessment involving the right heart in therapeutic and prognostic decision-making processes.

Background Unilateral chronic thromboembolism pulmonary disease (CTEPD) is very rare. There is limited information on the safety and efficacy of pulmonary endarterectomy (PEA) in this population. This study investigated the effectiveness of PEA in this unique disease. Methods This multicentre study included patients with unilateral CTEPD from three referral centres in the United States, Turkey, and Iran. The patients’ demographic information, 6-minute walk test distance (6MWD), New York Heart Association (NYHA) functional class, and haemodynamics were evaluated. Result Of the 1,031 patients who had undergone PEA, 39 patients (3.7%) had pure unilateral involvement, of whom 28 were female (71.8%). There was a significant improvement in the mean pulmonary artery pressure (mPAP, 26 mmHg vs 21 mmHg; p=0.011) and pulmonary vascular resistance (PVR, 202 vs 136 dynes∗sec1∗cm-5; p=0.014). There was also a significant improvement in NYHA functional class (p<0.001) and 6MWD (360 vs 409 m; p<0.001). In the nine patients with normal haemodynamic parameters at rest, there was no significant change in median 6MWD (448.5 vs 449 m; p=0.208), mPAP (19 mmHg vs 16.5 mmHg; p=0.397), and PVR (129 vs 84.5 dynes∗sec1∗cm-5; p=0.128). The most common postoperative complication was ipsilateral pleural effusion. One patient needed extracorporeal membrane oxygenation support. No patient died within the 1-year follow up. Conclusion Pulmonary endarterectomy is a safe and effective procedure for improving the symptoms and haemodynamic parameters of patients with unilateral CTEPH. Symptomatic patients with unilateral chronic thromboembolic disease are suitable for PEA.

Pulmonary hypertension (PH) is a progressive vascular disease characterized by elevated pulmonary arterial pressure and resistance in the pulmonary vascular bed. It is associated with high morbidity and mortality. Although right heart catheterization (RHC) is the gold standard for diagnosis, noninvasive methods, such as echocardiography, are essential for early detection and management. This study aimed to elucidate the potential of right ventricular outflow tract fractional shortening (RVOT-FS) as a noninvasive diagnostic tool for PH. This single-center observational cohort study was conducted between November 2023 and May 2024. The study included 141 patients referred to the PH clinic (75 with confirmed PH and 66 controls). Echocardiographic measurements were performed using standard protocols, and RVOT-FS was calculated. Hemodynamic parameters were obtained via RHC. RVOT-FS was significantly lower in the PH group (35.71

We retrospectively analyzed patients who underwent prone positioning (PP) for acute respiratory failure after pulmonary endarterectomy (PEA).A total of 125 patients underwent PEA and the outcome related to patients who underwent PP for acute respiratory failure after surgery was analyzed.In all 13 patients (10%) underwent PP at the mean duration of 28.2 ± 10.6 hours after surgery and the mean prone time was 29.4 ± 9.8 hours. Compared with the pre-prone values, there was a significant improvement in the mean arterial oxygen to fraction of inspired oxygen ratio at the end of PP (119.4 ± 12.4 versus 202 ± 58.3) (p = 0.0002). Eight patients (61%) revealed a significant improvement in oxygenation with PP. Five patients who remained unresponsive underwent extracorporeal membrane oxygenation and four of them were weaned off successfully. In multivariate logistic stepwise analysis, the need for a moderate inotropy (odds ratio [OR]: 3.1) and low preoperative cardiac index (OR: 0.2) were independent predictors of PP. Under PP, the most common complication was ventilator-associated pneumonia (n = 9, 70%) and PP was found to be an independent predictor of ventilator-associated pneumonia (OR: 10.3). Early mortality was seen in three patients (23%, sepsis in two and adult respiratory distress syndrome in one).In the early care of acute respiratory failure following PTE, PP may be a feasible option, despite an increased risk of ventilator-associated pneumonia. More research involving a larger sample size is necessary.

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years’ observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. The most common symptoms were typical of those expected for CTEPH. Almost 90

Background: Herein, we aimed to develop and test machine learning (ML) models to predict disease severity and/or progression in hospitalized COVID-19 patients through baseline laboratory features. Methods: In this retrospective study of hospitalized COVID-19 patients admitted to a tertiary care center, we evaluated routine admission data to determine the accuracy rates of different ML algorithms: k-nearest neighbor classifier, bagging classifier, random forest (RF), and decision tree. These models were compared over three outcomes: those who needed oxygen supplementation vs. who did not on admission (Analysis 1, n: 180), those who later developed oxygen requirement vs. those who did not (Analysis 2, n: 112), and those who needed invasive mechanical ventilation vs. those who did not during hospitalization (Analysis 3, n: 164). Results: The median age of the patients was 55 (44-68) years, with males constituting 47.2% of the subjects. At admission, 37.8% of the patients required oxygen supplementation. During hospitalization, 17.5% needed mechanical ventilation, and 8.3% died. For all analyses, RF had the highest accuracy in classifying the need for oxygen supplementation on admission (89.4%) or during hospitalization (91.1%) and for invasive mechanical ventilation (92.2%). These were followed by a bagging classifier for Analysis 1 (88.3%) and Analysis 3 (91.0%) and by a decision tree for Analysis 2 (88.4%). C-reactive protein, monocyte distribution width, and high-sensitive troponin-T were the most crucial laboratory contributors to Analysis 1, Analysis 2, and Analysis 3, respectively. Conclusion: Our study showed that ML algorithms could predict the need for oxygen supplementation and mechanical ventilation during hospitalization using baseline laboratory data, suggesting a slight superiority of RF, among others.

Although current pulmonary hypertension (PH) guidelines recommend a pulmonary capillary wedge pressure (PCWP) >15 mm Hg for the detection of a postcapillary component, the rationale of this recommendation may not be quite compatible with the peculiar hemodynamics of PH. We hypothesize that a high PCWP alone does not necessarily indicate left-sided disease, and this diagnosis can be improved using left ventricle transmural pressure difference (∆ PTM). In this 2-center, retrospective, observational study, we enrolled 1,070 patients with PH who underwent heart catheterization, with the final study population comprising 961 cases. ∆ PTM was calculated as PCWP minus right atrial pressure. The patients with group II PH had significantly higher ∆ PTM values (12.6 ± 6.6 mm Hg) compared with the other groups (1.1 ± 4.8 in group I, 12.4 ± 6.6 in group II, 2.5 ± 6.4 in group III, and 0.8 ± 8.0 in group IV, p <0.001) despite overlapping PCWP values. A ∆ PTM cutoff of 7 mm Hg identifies left heart disease when PCWP is >15 (area under curve 0.825, 95% confidence interval 0.784 to 0.866, p <0.001). Five-year mortality was significantly higher in patients with high ∆ PTM and PCWP subgroups compared with low ∆ PTM plus high PCWP (26.1% vs 18.5%, p = 0.027) and low ∆ PTM and PCWP subgroups (26.1% vs 15.6%, p <0.001). ∆ PTM has supplementary discriminatory power in distinguishing patients with and without postcapillary PH. In conclusion, a new approach utilizing ∆ PTM may improve our understanding of PH pathophysiology and may identify a subpopulation that may potentially benefit from PH-specific treatments.

Objective: We aimed to investigate the effect of right ventricular energy failure (RVEF) on hemodynamic and clinical outcomes in patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) surgery or balloon pulmonary angioplasty (BPA). Patients and Methods: A total of 100 CTEPH patients planned for PEA or BPA were included in the study. Based on the presence of RVEF during diagnosis, patients divided into two groups. Hemodynamic data from right heart catheterization (RHC) were compared before and after procedures in 3-6 months follow up period. Results: Patients with RVEF revealed a decrease in mean pulmonary artery pressure (mPAP) from 54.67 +/- 12.27 mmHg to 36.12 +/- 11.76 mmHg (p:<0.001), mean right atrial pressure (mRAP) from 13.40 +/- 4.08 mmHg to 9.76 +/- 4.56 mmHg (p:0.003), and pulmonary vascular resistance (PVR) from 11.36 +/- 5.15 Wood Units (WU) to 5.46 +/- 3.30 WU (p <0.001). In the non-RVEF group, mPAP decreased from 38.82 +/- 12.61 mmHg to 30.81 +/- 10.57 mmHg (p:<0.001), mRAP from 7.09 +/- 3.02mmHg to 7.15 +/- 3.07mmHg (p: 0.917), and PVR from 6.33 +/- 3.65 WU to 4.09 +/- 2.31 WU (p:<0.001). Conclusion:The presence of RVEF at the time of diagnosis in CTEPH patients does not have a negative impact on early perioperative and 3-month postoperative outcomes following PEA or BPA. This high-risk patient group demonstrated significant hemodynamic and clinical benefits from both PEA and BPA.

Objective: This study was conducted to examine the effect of a Health Promotion Model-based health education program on increasing the health-promoting behaviors of acute coronary syndrome patients. Methods: This is an experimental study that used a pretest-posttest design with a control group. The sample consisted of 101 patients hospitalized with the diagnosis of acute coronary syndrome in Turkey. The data were collected by using a Patient Monitoring Form and the Health Promoting Lifestyle Profile-II. While the patients in the control group received routine procedures, the patients in the experimental group were included in the health education program. Results: The health promoting-behaviors of the control group were found to be higher than those of the experimental group in the first follow-up (p

Abstract Background and aims In this retrospective analysis of a nation-wide registry on pulmonary arterial hypertension (PAH), group IV and V, we evaluated management strategies, morbidity and mortality across three consecutive time periods. Methods This study included 903 patients enrolled from 24 cardiology centers participating in the RegiStry on clInical outcoMe and sUrvival in pulmonaRy hypertension Groups (SIMURG II). Patients were divided into three enrollment periods: before 2016, the era of monotherapy or sequential combination therapies; between 2016 and 2019, post-approval and re-imbursement of macitentan in our country, marking a shift towards more proactive sequential combinations; and after 2019, signifying the initiation of earlier sequential combinations with selexipag in PAH. ESC/ERS 2022, COMPERA 2.0 and REVEAL lite 2 models were used for risk predictions. Composite endpoint (CEP) definitions were adopted from the SERAPHIN and GRIPHON trials. Results Age (mean +SD) was 51.27 + 21.37 years, and 72 % were female. Incident cases were noted in 65.9 % of patients. Idiopathic PAH (IPAH), PAH-associated with congenital heart diseases (CHD-PAH), PAH-associated with connective tissue diseases (CTD-PAH), porto-PAH, Group IV and V pulmonary hypertension (PH) were documented in 33 %, 33.5 %, 12 %, 1.6 %, 20 % and 1.7 % of patients, respectively. Background mono and dual targeted combinations, and sequential triple combinations were noted in 17 %, 57 % and 26 % of patients, respectively. Baseline low-, intermediate-, and high-risk were noted in 1.2 %, 14.1 %, and 84.7 %, respectively. Median follow-up time (day) was 867 (412-1667). Overall rates of mortality and CEP were 26 % and 39%, respectively. There were increases in the % of PAH and group IV PH, and decreases in the % of CHD-PAH across the three periods. Prevalent versus incident cases were associated signifinatly lower mortality and CEP ( p=0.028 and p=0.024, respectively). Outcomes for mortality and CEP were predicted by three risk models (p<0.0001 for all). Mortality and CEP rates were 31 % and 44 % in IPAH, 20 % and 39 % in CHD-PAH, 31 % and 43 % in CTD-PAH, and 25 % and 30 % in group IV patients, respectively. In overall PH population, rates of mortality and CEP documented at 1st, 2nd and 3rd periods were 64 % and 67 %, 29 % and 31 %, and 3.5 % and 5.1 %, respectively (p< 0.001, for all). Mortality rates for 1st, 2nd and 3rd periods were 49 %, 39 % and 11 % in IPAH, 62 %, 33 % and 5.3 % in CHD-PAH, 47 %, 45 % and 7.7 in CTD-PAH, and 37 %, 52 % and 11 % in group IV, respectively (p< 0.001, for all). However, mortality curves showed sudden increases in 2020 that might be attributed to COVID-19. Conclusions Our nation-wide data revealed improving survival in the overall PH population, PAH and Group IV PH that might be associated with risk-based earlier targeted combination strategies. However, this trend seems to be counterbalanced with COVID-19 pandemic.