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Introduction: Pulmonary hypertension due to left heart failure (PH-LHF) is a disease with high prevalence and 3-year mortality rates. Consequently, timely identification of patients with high mortality risk is critical. This study aimed to build a nomogram for predicting 3-year mortality and screening high-risk PH-LHF patients. Methods: This nomogram was developed on a training cohort of 175 patients with PH-LHF diagnosed by right heart catheterization (RHC). Multivariate Cox regression was used to identify independent predictors and develop this nomogram. The median total points obtained from the nomogram were used as a cut-off point, and patients were classified into low- and high-risk groups. The concordance index (C-index) and calibration curve were utilized to ascertain the predictive accuracy and discriminative ability of the nomogram. External validation was performed using a validation cohort of 77 PH-LHF patients from other centers. Results: Multivariate Cox regression showed that the New York Heart Association functional classification (NYHA FC), uric acid level, and mean pulmonary arterial pressure (mPAP) were all independent predictors and incorporated into the nomogram. The nomogram showed good discrimination (C-index of 0.756; 95% CI: 0.688 – 0.854), and good calibration. The Kaplan-Meier survival analysis showed that patients in the high-risk group had worse survival ( p < 0.001). In the external validation, the nomogram showed both good discrimination (C-index of 0.738; 95% CI: 0.591 – 0.846) and calibration. Conclusion: The nomogram had a good performance in predicting 3-year mortality and can effectively identify high-risk patients. The nomogram may help to reduce the mortality of PH-LHF.

ObjectivesTo report the 10-year survival rate and prognostic factors of pulmonary arterial hypertension associated with CTD (CTD-PAH) patients, to compare treatment and survival between patients enrolled before and after 2015, and to validate the discrimination of the recommended four-strata model in predicting 10-year survival at follow-up in Chinese CTD-PAH patients.MethodsThis study was derived from a Chinese national multicentre prospective registry study from 2009 to 2019. Medical records were collected at baseline and follow-up, including PAH-targeted therapy and binary therapy (both CTD and PAH-targeted therapy).ResultsA total of 266 CTD-PAH patients were enrolled and the 10-year survival rate was 59.9% (median follow-up time: 4.85 years). Underlying CTD (SSc), baseline 6-min walking distance and SaO2 were independent risk factors for 10-year survival. The proportion of patients receiving PAH-targeted combination therapy increased from 10.1% (2009–2014) to 26.5% (2015–2019) and that of binary therapy increased from 14.8% to 35%. The 1-year survival rate increased from 89.8% (2009–2014) to 93.9%, and the 3-year survival rate increased from 80.1% (2009–2014) to 86.5% (both P > 0.05). The four-strata strategy performed well in predicting 10-year survival at follow-up (C-index = 0.742).ConclusionThe 10-year survival rate of CTD-PAH patients was reported for the first time. The 10-year prognosis was poor, but there was a tendency for more standardized treatment and better survival in patients enrolled after 2015. The recommended four-strata model at follow-up can effectively predict 10-year survival in CTD-PAH patients.

Rationale: Iron deficiency, in the absence of anemia, is common in patients with idiopathic and heritable pulmonary arterial hypertension (PAH) and is associated with a worse clinical outcome. Oral iron absorption may be impeded by elevated circulating hepcidin concentrations. The safety and benefit of parenteral iron replacement in this patient population is unclear. Objectives: To evaluate the safety and efficacy of parenteral iron replacement in PAH. Methods: In two randomized, double-blind, placebo-controlled 12-week crossover studies, 39 patients in Europe received a single infusion of ferric carboxymaltose (Ferinject) (1,000 mg or 15 mg/kg if weight

Background At present, there is no generally accepted comprehensive prognostic risk prediction model for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients. Methods Consecutive medically treated CTEPH patients were enrolled in a national multicenter prospective registry study from August 2009 to July 2018. A multivariable Cox proportional hazards model was utilized to derive the prognostic model, and a simplified risk score was created thereafter. Model performance was evaluated in terms of discrimination and calibration, and compared to the Swedish/COMPERA risk stratification method. Internal and external validation were conducted to validate the model performance. Results A total of 432 patients were enrolled. During a median follow-up time of 38.73 months (IQR: 20.79, 66.10), 94 patients (21.8%) died. The 1-, 3-, and 5-year survival estimates were 95.5%, 83.7%, and 70.9%, respectively. The final model included the following variables: the Swedish/COMPERA risk stratum (low-, intermediate- or high-risk stratum), pulmonary vascular resistance (PVR, ≤ or > 1600 dyn·s/cm5), total bilirubin (TBIL, ≤ or > 38 µmol/L) and chronic kidney disease (CKD, no or yes). Compared with the Swedish/COMPERA risk stratification method alone, both the derived model [C-index: 0.715; net reclassification improvement (NRI): 0.300; integrated discriminatory index (IDI): 0.095] and the risk score (C-index: 0.713; NRI: 0.300; IDI: 0.093) showed improved discriminatory power. The performance was validated in a validation cohort of 84 patients (C-index = 0.707 for the model and 0.721 for the risk score). Conclusions A novel risk stratification strategy can serve as a useful tool for determining prognosis and guide management for medically treated CTEPH patients. Trial registration: ClinicalTrials.gov (Identifier: NCT01417338).

Background The prognostic predictors of pulmonary hypertension (PH) due to advanced heart failure (HF) have yet to be explored. Objectives To examine the prognostic value of hemodynamics and comorbidities in this patient group. Methods We retrospectively enrolled consecutive patients with PH due to advanced HF diagnosed by echocardiography and right heart catheterization. Follow-up was performed every 6 months ± 2 weeks. Primary endpoints were all-cause mortality and heart or lung transplantation. Results In total, 92 patients were included. The mean age was 46.82 years and mean left ventricular ejection fraction (LVEF) was 26.63%. During a median follow-up time of 9.72 months, 66 patients (71.7%) met primary endpoints. Pulmonary arterial compliance (PAC) was a significant predictor for primary endpoints and patients burdened with more than 3 comorbidities had worse prognoses (P = 0.0114). Conclusions In these patients, PAC can be a potential prognostic predictor and patients with a higher comorbidity burden have worse outcomes.

Nationally representative reports on the characteristics and long-term survival of pulmonary arterial hypertension (PAH) from developing countries are scarce. The applicability of the current main risk stratifications and the longitudinal changes in goal-oriented treatments have yet to be elucidated in real-world settings. Therefore, we aimed to provide insights into the characteristics, goal-oriented treatments and survival of PAH in China and to explore the applicability of the main risk stratifications in our independent cohort.PAH patients were consecutively enrolled from a national prospective multicentre registry. Data on baseline, follow-up re-evaluation and therapeutic changes were collected.A total of 2031 patients were enrolled, with congenital heart disease (CHD)-PAH (45.2%) being the most common aetiology. The mean age was 35 ± 12 years, and 76.2% were females. At baseline, approximately 20% of the patients with intermediate or high risk received combination treatment. At follow-up, approximately half of the re-evaluated patients did not achieve low-risk profiles, and even among patients who received combination therapy at baseline, 4% of them still worsened. The rate of combination therapy increased significantly from 6.7% before 2015 to 35.5% thereafter. The main risk assessment tools demonstrated good performance for predicting survival both at baseline and at follow-up.Chinese PAH patients show both similar and distinct features compared to other countries. Current main risk stratifications can significantly discriminate patients at different risk levels. There were still many patients not achieving low-risk profiles at follow-up, indicating more aggressive treatment should be implemented to optimize the goal-oriented treatment strategy.

Additional file 1. Figure S1. Comparison of Kaplan-Meier survival curves according to the Swedish/COMPERA risk stratum (Low-, intermediate- and high-risk groups). Figure S2. Comparison of Kaplan-Meier survival curves according to the derived risk score (0-3, 4-5 or ≥ 6 points) in newly diagnosed CTEPH patients. Figure S3. Comparison of Kaplan-Meier survival curves according to the derived risk score (0-3, 4-5 or ≥ 6 points) in surgically inoperable CTEPH patients. Figure S4. Comparison of Kaplan-Meier survival curves according to the derived risk score (0-3, 4-5 or ≥ 6 points) in patients without chronic liver disease. Figure S5. Calibration of the derived model (A) and the risk score (B) in the validation cohort. Table S1. Univariate Cox proportional hazards analyses of candidate variables for all-cause mortality in the overall analyzed cohort. Table S2. Baseline characteristics of the 3 risk groups classified by the new derived risk score. Table S3. Baseline characteristics of the newly diagnosed CTEPH patients. Table S4. Baseline characteristics of the surgically inoperable CTEPH patients. Table S5. Baseline characteristics of patients without chronic liver disease. Table S6. Estimated 1-, 3- and 5-year survival of the three risk groups in newly diagnosed CTEPH patients. Table S7. Estimated 1-, 3- and 5-year survival of the three risk groups in surgically inoperable patients. Table S8. Estimated 1-, 3- and 5-year survival of the three risk groups in patients without chronic liver disease. Table S9. Baseline characteristics of the validation cohort.

Objectives: To identify right heart catheterization (RHC)-assessed hemodynamic parameters of pulmonary circulation that were prognostic predictive of long-term survival in acute coronary syndrome (ACS), and to explore the incremental value of those parameters to the established risk models of coronary heart disease. Methods: Consecutive patients with ACS who underwent coronary angiography together with RHC were retrospectively reviewed. The primary endpoint was all-cause mortality. To identify the association between hemodynamic parameters and survival, Cox proportional hazards models were conducted. The optimal parameter identified was included in three subsequent models, which contained the Global Registry of Acute Coronary Events (GRACE), the Long-Term Intervention with Pravastatin in Ischemic Disease (LIPID) and the Cardiovascular Disease Research Using Linked Bespoke Studies and Electronic Health Records (CALIBER) models respectively. Model performance was evaluated before and after the addition of hemodynamic parameters. Results: A total of 251 patients were enrolled, with a median follow-up time of 34.7 months. By Cox analyses, systolic pulmonary arterial pressure [hazard ratio (HR): 1.043, 95% confidence interval (CI): 1.013-1.074], diastolic pulmonary arterial pressure (HR: 1.086, 95% CI: 1.014-1.164), mean pulmonary arterial pressure (HR: 1.077, 95% CI: 1.024-1.132), right ventricular systolic pressure (HR: 1.039, 95% CI: 1.008-1.070) and diastolic pressure gradient (HR: 1.101, 95% CI: 1.015-1.193) were found to be independent predictors of long-term survival. Furthermore, the addition of mean pulmonary arterial pressure to the established risk models yielded an improvement in model performance (integrated discriminatory index for the GRACE, LIPID and CALIBER model: 4.3%, 4.4% and 4.7%, respectively; all had a P Conclusions: Hemodynamic parameters of pulmonary circulation can be predictive of long-term survival and provide incremental prognostic value to risk assessment in patients with ACS.

Right ventricular (RV) function is considered the major determinant of prognosis in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this meta-analysis was to evaluate RV remodelling and function following balloon pulmonary angioplasty (BPA) in patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension (PH) after pulmonary endarterectomy (PEA). We reviewed all studies evaluating RV function by cardiac magnetic resonance (CMR) and/or echocardiography pre- and post-BPA from PubMed/Medline prior to 15 December 2019. Ten (299 patients) of the 29 studies retrieved met the inclusion criteria: 5 CMR and 5 echocardiography studies. The systematic review and meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Guidelines. Pooled data from CMR studies revealed BPA resulted in a significantly decreased RV end-diastolic volume index (weighted mean difference (WMD) − 28.33 ml/m2, p < 0.00001) and RV end-systolic volume index (WMD − 29.06 ml/m2, p < 0.00001) accompanied by an increased RV ejection fraction (RVEF, WMD 8.97%, p < 0.00001). Data from the echocardiography studies showed BPA resulted in decreased RV basal diameter (WMD − 0.37 cm, p = 0.0009) and an increase of RV fractional area change (WMD 5.97 %, p = 0.003), but improvements of tricuspid annular plane systolic excursion (TAPSE) and S′ were not significant. BPA improves RVEF and decreases RV volumes in patients with inoperable CTEPH or persistent/recurrent PH after PEA. • Balloon pulmonary angioplasty improves RVEF and decreases RV volumes in patients with inoperable CTEPH or persistent/recurrent PH after PEA.

Background The prevalence, characteristics and survival of pulmonary hypertension due to left heart disease (PH-LHD) in heart failure with reduced and preserved ejection fraction (HFrEF, HFpEF) has yet to be explored. Methods Consecutive patients with chronic heart failure undergoing first right heart catheterization (RHC) were prospectively enrolled from October 2012 to August 2016 in 11 participating medical centers. Follow-up was performed every 6 months ± 2 weeks. The primary endpoint was all-cause mortality. Results A total of 500 patients were enrolled. The prevalence of PH was 67.2% in HFrEF and 40.2% in HFpEF, respectively. Predictors of PH differed between PH etiologies, but left ventricular end diastolic diameter (LVEDD) was a consistent both in HFrEF (P=0.031) and HFpEF (P=0.003). During a median follow-up time of 33.39 months, 69 patients (13.8%) met the primary endpoint. The survival of PH patients was significantly worse than that of patients without PH (P=0.001). Diastolic pressure gradient (DPG) was a significant prognostic variable both in HFrEF (HR=1.057, 95% CI=1.007-1.108, P=0.024) and HFpEF (HR=1.094, 95% CI=1.009-1.187, P=0.030). Patients with a DPG ≥ 7 mmHg had a worse survival compared to those whose DPG < 7 mmHg both in HFrEF (log rank test, P=0.047) and in HFpEF (P=0.016). Conclusion Though the prevalence, characteristics and prognosis of PH differ between HFrEF and HFpEF, PH-LHD is a common complication and has an adverse effect on the prognosis. Study registration NCT02164526.

Background and objective The purpose of this study was to report the characteristics and long-term survival of patients with CTEPH treated in three distinct ways: PEA, BPA and medical therapy. Methods Patients diagnosed with CTEPH were included in the registry that was set up in 18 centres from August 2009 to July 2018. The characteristics and survival of patients with CTEPH receiving the different treatments were reported. Prognostic factors were evaluated by Cox regression model. Results A total of 593 patients with CTEPH were included. Eighty-one patients were treated with PEA, 61 with BPA and 451 with drugs. The estimated survival rates at 1, 3, 5 and 8 years were, respectively, 95.2%, 84.6%, 73.4% and 66.6% in all patients; 92.6%, 89.6%, 87.5% and 80.2% in surgical patients; and 95.4%, 88.3%, 71.0% and 64.1% in medically treated patients. The estimated survival rates at 1, 3, 5 and 7 years in patients treated with BPA were 96.7%, 88.1%, 70.0% and 70.0%, respectively. For all patients, PEA was an independent predictor of survival. Other independent risk factors were CHD, cardiac index, PVR, big endothelin-1, APE and 6MWD. Conclusion This is the first multicentre prospective registry reporting baseline characteristics and estimated survival of patients with CTEPH in China. The long-term survival rates are similar to those of patients in the international and Spanish registries. PEA is an independent predictor of survival.

To explore the value of right ventricular (RV) parameters detected by three-dimensional echocardiography (3DE) in risk stratification in pulmonary arterial hypertension (PAH) patients. We prospectively recruited 130 pulmonary hypertension patients from National Center for Cardiovascular Diseases, Fuwai Hospital. Each participant was performed a transthoracic echocardiography and 3DE parameters were measured using an off-line software (4D RV Function 2.0, TomTec). Patients were classified into low, intermediate-high risk group based on 2015 ESC Guidelines. A total of 91 PAH patients (34 ± 12 years old, 25 males) were enrolled, among which, 42 were classified into low risk group, while 49 were intermediate-high risk group. Compared with low-risk patients, those with intermediate-high risk had significantly larger 3DE-RV volumes, worse ejection fraction (EF) and tricuspid annular plane systolic excursion, and decreased longitudinal strain (LS). Receive operating characteristic curves illustrated all the 3DE parameters were able to predict intermediate-high risk stratification, especially 3D-RVEF (area under curve, 0.82, 95% CI 0.73–0.91, P

To investigate and compare the value of right ventricular longitudinal strain detected by two-dimensional and three-dimensional speckle-tracking echocardiography in risk stratification evaluation in pre-capillary pulmonary hypertension. We consecutively screened 66 patients diagnosed with pre-capillary pulmonary hypertension in our center. According to the risk assessment recommended by 2015 European Society of Cardiology Guidelines, all participants were classified into low- and intermediate-high-risk group. Two-dimensional and three-dimensional strains were measured using off-line softwares (GE EchoPAC version 201 and TomTec, 4D RV Function 2.0). Fifty-seven pre-capillary pulmonary hypertension patients (average 35 years old, 18 males and 39 females) were finally enrolled in our study, 32 (56.1%) were classified in low-risk group, while 25 (43.9%) were in the intermediate-high-risk group. Clinical data associated with disease severity, such as N-terminal pro-brain natriuretic peptide ( r = 0.574, P