BACKGROUND:Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is characterized by substantial prognostic and therapeutic heterogeneity. Conventional clinical and anatomical-pathophysiological classifications fail to fully capture these intricacies. Unsupervised clustering approaches such as latent class analysis (LCA) have been used to address this limitation. RESEARCH QUESTION:Can LCA-derived phenogroups refine risk stratification and predict PAH-targeted treatment responses in adult patients with PAH-CHD? STUDY DESIGN AND METHODS:Adult patients with PAH-CHD (N = 889) from a multicenter prospective registry were stratified into post- and pre-tricuspid shunt subsets. LCA was performed in each subset using 28 candidate clinical, laboratory, and hemodynamic variables. Ten-year survival and the effect of PAH-targeted therapy across phenogroups were evaluated. RESULTS:LCA identified 4 phenogroups in each subset with distinct phenotyping patterns. In post-tricuspid shunts, Group 1 ("adaptation with open shunt") exhibited preserved right ventricular function and the best survival despite high afterload. Group 3 ("maladaptation and failure") and Group 4 ("closed shunt and failure") faced 2- to 3-fold higher mortality than Group 1. Notably, Group 2 ("hyperkinetic state") uniquely benefited from combination therapy (Pinteraction = .026). In pre-tricuspid shunts, Group 1 ("high shunt and mild resistance") and Group 3 ("young female and low inflammation") exhibited preserved survival despite varying afterloads. However, Group 2 ("old male and comorbidities") had significantly higher mortality and an attenuated treatment response (Pinteraction = .037) despite sharing mild hemodynamics with Group 1. Group 4 ("high resistance and failure") faced severe hypoxemia and the worst prognosis. INTERPRETATION:LCA successfully stratified patients with PAH-CHD into distinct phenogroups with varied clinical profiles, survival, and treatment responses. This data-driven phenotyping system provides a novel approach for risk stratification and may guide personalized clinical decision-making. CLINICAL TRIAL REGISTRATION:ClinicalTrials.gov; No.: NCT01417338; URL: www. CLINICALTRIALS:gov.

Data on the 10-year survival of incident IPAH in China is lacking. The changing trend of characteristics and survival of IPAH over the past decade has yet been demonstrated. The 4-stratum risk model needs further validation in different cohorts. Incident cases of IPAH identified in the national multicenter prospective registry of PAH from 2009 to 2019 were analyzed. Patients with at least two of the three acquired variables (WHO-FC, 6MWD, NT-pro BNP/BNP) were included for risk stratification. 605 incident patients with IPAH were included (age: 35 ± 12 years; 75.2

Pulmonary arterial compliance (PAC) reflects the pulsatile load and predicts outcome in pulmonary arterial hypertension (PAH). The prognostic role of PAC in the heterogeneous patient population of PAH associated with congenital heart disease (PAH-CHD) is poorly defined. This study aimed to explore the prognostic value of PAC in patients with PAH-CHD. Adult patients diagnosed with PAH-CHD were collected from a PAH multicenter prospective registry between August 2009 and December 2019. The primary endpoint was all-cause mortality. Multivariable Cox regression and restricted cubic spline (RCS) analysis were used to evaluate the association between PAC and the primary endpoint. Subgroup and interaction analysis between PAC and shunts or defect characteristics were explored. Incremental predictive performance was evaluated by calculating the C-index, continuous net reclassification improvement, and integrated discrimination improvement. A total of 434 adult PAH-CHD patients were enrolled. The median follow-up time was 52.2 months. The survival rate of patients in the lower PAC group was significantly worse than those in the higher PAC group (Log-rank P < 0.001). Multivariable Cox regression analysis showed that PAC independently predicted all-cause mortality after adjustment for other prognostic factors, whether as a continuous variable (HR = 0.665, 95

Purpose To investigate the prognostic value of cardiac MRI-derived left ventricular (LV) layer-specific strain in pulmonary arterial hypertension (PAH). Materials and Methods This study analyzed data from a subcohort of participants consecutively enrolled in the prospective PAH registry study between January 2011 and December 2017 who underwent right heart catheterization and cardiac MRI (1.5-T scanner) within 1 week. The deformable registration algorithm software was used to analyze LV layer-specific strain from acquired breath-held cine images. Each participant was followed up at intervals of 6 months plus or minus 2 weeks for all-cause mortality. Cox regression and Kaplan-Meier analyses were performed to assess the association between LV layer-specific strain and all-cause mortality. Results Eighty participants with PAH (mean age ± SD, 30.30 years ± 9.73; 62 female, 18 male) were included, with a median follow-up of 1511 days. At the end, 29 (36%) participants died by any cause. Participants who survived had significantly higher endocardial wall peak systolic radial strain rate (ewSRSR; 72.74% ± 50.64 vs 30.61% ± 67.43; P = .005) and transmural gradient from the epicardial to endocardial walls in peak late diastolic circumferential strain rate (Gepiw-ewDCSRL; -20.05% ± 22.97 vs -33.50% ± 25.71; P = .02) compared with participants who died. Multivariable Cox regression showed that ewSRSR and Gepiw-ewDCSRL were independent predictors of all-cause mortality. Kaplan-Meier analysis showed that participants with PAH with ewSRSR less than 54.48% (log-rank P = .003) or Gepiw-ewDCSRL less than -25.14% (log-rank P = .02) at baseline had a decreased likelihood of long-term survival. In the low- or intermediate-risk PAH groups, participants with LV ewSRSR less than 54.48% had a worse prognosis (log-rank P = .02). Conclusion Cardiac MRI-derived LV ewSRSR and Gepiw-ewDCSRL independently predicted all-cause mortality in participants with PAH. Keywords: Layer-specific Strain, DRA-CMR, Left Ventricle, Pulmonary Arterial Hypertension, Prognosis ClinicalTrials.gov: NCT01417338 Supplemental material is available for this article. © RSNA, 2025.

Background: Identification of acute pulmonary embolism (APE) in patients with acute exacerbation of chronic obstructive pulmonary disease (AECOPD) is challenging. Wells score and Revised Geneva score have been developed to diagnose APE. We aim to investigate the predictive accuracy of two clinical scoring systems combined with D-dimer for APE in patients with AECOPD. Methods: A multicentre cross-sectional study was conducted in 13 China hospitals. A total of 731 patients were enrolled. Computed tomography pulmonary angiography (CTPA) was performed within 48 hours of admission. The performance of the clinical scoring systems was compared by calculating the area under the receiver operating characteristic curves (AUROC), sensitivities, and specificities. Results: 731 patients were included with an average age of 68.9 years, with a male proportion of 585 (80.0 %). 112 (15.3 %) were diagnosed with APE. The optimal D-dimer cut-off value for identifying APE in AECOPD was 690.12 ng/mL. Analysis for assessing the clinical probability of APE using the 3-level Wells and Revised Geneva scores showed the AUC were 0.74 and 0.60, sensitivity were 61.61 % and 77.68 %, and specificity were 85.46 % and 38.29 %, respectively. Analysis using the 3-level Wells and Revised Geneva scores combined with a D-dimer cut-off value of 690.12 ng/mL showed the AUC were 0.909 and 0.869, sensitivity were 73.21 % and 91.96 %, specificity were 92.08 %and 72.70 %. The performance of the 3-level Wells score with D-dimer was significantly better than the performance of the 3-level Revised Geneva score with D-dimer (P = 0.01). Conclusions: The 3-level Wells score combined with a D-dimer cut-off value of 690.12 ng/mL performed better than other clinical scoring algorithms for assessing clinical probability of APE in patients with AECOPD.

Background and ObjectiveOff-label pulmonary arterial hypertension (PAH)-targeted drugs are commonly prescribed for non-operated chronic thromboembolic pulmonary hypertension (CTEPH), but their effect on the long-term prognosis of CTEPH remains unknown. This study investigated the effect of off-label PAH-targeted drugs on the long-term survival of CTEPH patients.MethodsCTEPH patients were enrolled from a prospective multicentre national registry. Except for licensed riociguat and treprostinil, other PAH-targeted drugs were off-label. In the original and propensity score-matched (PSM) samples, five-year survival was compared in two groups: (a) patients not receiving off-label PAH-targeted drugs (control) versus (b) patients receiving off-label PAH-targeted drugs (treatment). The latter group was investigated for the effect of started off-label PAH-targeted drugs at baselines (initial) or during follow-up (subsequent).ResultsOf 347 enrolled patients, 212 were treated with off-label PAH-targeted drugs initially (n = 173) or subsequently (n = 39), and 135 were untreated. The 1-, 2-, 3- and 5-year survival of the treatment group was significantly higher than that of the control group (97.1% vs. 89.4%, 92.3% vs. 82.1%, 83.2% vs. 75.1% and 71.1% vs. 55.3%, respectively, log-rank test, p = 0.005). Initial treatment was correlated with better 5-year survival after excluding patients with subsequent treatment to reduce the immortal-time bias (hazard ratio: 0.611; 95% CI: 0.397-0.940; p = 0.025). In PSM samples, patients given initial treatment showed significantly better 5-year survival than untreated patients (68.9% vs. 49.3%, log-rank test, p = 0.008).ConclusionOff-label targeted drugs contributed to improved long-term survival in CTEPH patients receiving pharmacotherapies. imageConclusionOff-label targeted drugs contributed to improved long-term survival in CTEPH patients receiving pharmacotherapies. image The study demonstrates that off-label use of pulmonary arterial hypertension medication improves long-term survival in patients with chronic thromboembolic pulmonary hypertension. This is a national, multicentre, prospective registry with a large sample size and low rate of loss to follow-up. The efficacy is more transparent after rigorously reducing bias. image

PURPOSE:To investigate the imaging characteristics and prognostic factors for the long-term survival of Behcet's disease (BD) with arterial involvement. METHODS:In this retrospective study, BD patients with arterial involvement were identified from January 2003 to January 2020. Arterial lesions were detected by ultrasonography, traditional arteriography, and/or computed tomography angiography (CTA). Cox proportional hazards regression analyses were performed to identify the prognostic factors. RESULTS:Totally, 84 BD patients with arterial involvement were identified (73.8 % males). The mean age at BD diagnosis was 39.1 ± 13.1 years. Arterial involvement was the initial manifestation in 33.3 % of the patients, and the median time from BD diagnosis to arterial involvement was 6 (IQR 1-15.5) years for the rest of patients. Systemic artery involvement and pulmonary artery involvement (PAI) were found in 64 and 27 patients, respectively. Approximately 94.0 % (79/84) of the patients had more than one artery involved concurrently or successively during the course of BD. Aneurysm/dilation was the most prevalent lesion in the aorta (76.0 %), while stenosis/occlusion was the main lesion of the coronary artery (90.9 %) and other aortic branches (74.5 %). Pulmonary hypertension was found in 70.4 % (19/27) of patients with PAI. The 5- and 10-year survival rates of BD patients with arterial involvement were 87.4 % and 84.1 %, respectively. Cardiac involvement (HR: 4.34) and pulmonary artery aneurysm/dilation (HR: 4.89) were independently associated with mortality. CONCLUSIONS:Arterial lesions associated with BD usually involve multiple arteries and manifest differently in different types of arteries. Cardiac involvement and pulmonary artery aneurysm/dilation are independent prognostic factors of BD patients with arterial involvement.

肺动脉高压（PH）是由多种病因引起的临床和病理生理综合征，因肺动脉压升高和肺血管阻力增加，导致进行性右心室衰竭和过早死亡，其机制尚未被完全阐明。铁死亡是一种铁离子依赖的调节性细胞死亡，对其调控机制的研究包括多不饱和脂肪酸及其衍生物代谢、活性氧物质产生、脂质过氧化等方面。近些年的研究表明铁死亡的促进和抑制在PH发展中共存，通过肺血管内皮细胞功能障碍、肺动脉平滑肌细胞增殖和右心室肥厚等环节参与PH的发病及进展，有望成为PH精准治疗的靶点。

BACKGROUND: Registry-based studies of pediatric pulmonary arterial hypertension (PPAH) are scarce in developing countries, including China. The PPAH risk assessment tool needs further evaluation and improvement.RESEARCH QUESTION: What are the characteristics and long-term survival of PPAH in China and what is the performance of the PPAH risk model in Chinese patients?STUDY DESIGN AND METHODS: Patients with PAH were enrolled in the national prospective multicenter registry from August 2009 through December 2019. Children 3 months to 18 years of age at the time of PAH diagnosis were analyzed.RESULTS: A total of 247 children with PAH were enrolled. The median patient age was 14.8 years, and 58.3% of patients were female. Most patients had a diagnosis of PAH associated with congenital heart disease (CHD; 61.5%) and idiopathic or heritable PAH (37.7%). The median time from symptom onset to PAH diagnosis was 24 months. The mean pulmonary artery pressure and pulmonary vascular resistance index were 70.78 + 19.80 mm Hg and 21.82 + 11.18 Wood Units$m2, respectively. Patients with CHD-associated PAH experienced a longer diagnostic delay and demonstrated higher pulmonary artery pressure, but better cardiac performance, than those with idiopathic or heritable PAH. An increased number of patients received targeted therapy at the last follow-up compared with baseline. The 5-and 10-year survival rates of the entire cohort were 74.9% and 55.7%, respectively, with better survival in patients with CHD-associated PAH than in those with idiopathic or heritable PAH. Patients with low risk had better survival than those with high risk according to the simplified noninvasive risk score model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up.INTERPRETATION: Patients with PPAH in China showed severely compromised hemody-namics with marked diagnostic delay. The long-term survival of PPAH is poor despite the increased usefulness of targeted drugs. The simplified noninvasive risk model demonstrated good performance for predicting survival in Chinese children with PAH.TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01417338; URL: www.clinicaltrials.gov CHEST 2023; 163(6):1531-1542

Objectives To 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. Methods This 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). Results A 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 SaO(2) 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). Conclusion The 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.

Background and Objective The prevalence of venous thrombus embolism (VTE) in patients with chronic obstructive pulmonary disease (COPD) is higher than in patients without COPD. Owing to the similarity of clinical symptoms between PE and acute exacerbation COPD (AECOPD), PE is likely to be overlooked or underdiagnosed in patients with AECOPD. The aim of the study was to investigate the prevalence, risk factor, clinical characteristics, and prognostic impact of VTE in patients with AECOPD. Methods This multicenter, prospective, cohort study was conducted in 11 research centers of China. Data on the baseline characteristics, VTE-related risk factors, clinical symptoms, laboratory examination results, computed tomography pulmonary angiography (CTPA) and lower limb venous ultrasound of AECOPD patients were collected. Patients were followed up for 1 year. Results A total of 1580 AECOPD patients were included in the study. The mean (SD) age was 70.4 (9.9) years and 195 (26%) patients were women. The prevalence of VTE was 24.5% (387/1580) and PE was 16.8% (266/1580). VTE patients were older; had higher BMI; and longer course of COPD than non-VTE patients. The history of VTE, cor pulmonale, less purulent sputum, increased respiratory rate, higher D-dimer, and higher NT-proBNP/BNP were independently associated with VTE in hospitalized patients with AECOPD. The mortality at 1-year was higher in patients with VTE than patients without VTE (12.9% vs 4.5%, p<0.01). There was no significant difference in the prognosis of patients with PE in segmental or subsegmental arteries and in main pulmonary arteries or lobar arteries (P>0.05). Conclusion VTE is common in COPD patients and is associated with poor prognosis. Patients with PE at different locations had poorer prognosis than patients without PE. It is necessary to perform active screening strategy for VTE in AECOPD patients with risk factors.

Background: Pulmonary arterial hypertension (PAH) leads to myocardial remodeling, manifesting as mechanical dyssynchrony (M-dys) and electrical dyssynchrony (E-dys), in both right (RV) and left ventricles (LV). However, the impacts of layer-specific intraventricular M-dys on biventricular functions and its association with E-dys in PAH remain unclear. Methods: Seventy-nine newly diagnosed patients with PAH undergoing cardiac magnetic resonance scanning were consecutively recruited between January 2011 and December 2017. The biventricular volumetric and layer-specific intraventricular M-dys were analyzed. The QRS duration z-scores were calculated after adjusting for age and sex. Results: 77.22 % of patients were female (mean age 30.30 ± 9.79 years; median follow-up 5.53 years). Further, 29 (36.71 %) patients succumbed to all-cause mortality by the end of the study. At the baseline, LV layer–specific intraventricular M-dys had apparent transmural gradients compared with RV in the radial and circumferential directions. However, deceased patients lost the transmural gradients. The LV longitudinal strain rate time to late diastolic peak in the myocardial region (LVmyoLSRTTLDPintra) predicted long-term survival. The Kaplan–Meier curve revealed that patients with PAH with LVmyoLSRTTLDPintra <20.01 milliseconds had a worse prognosis. Larger right ventricle (RV) intraventricular M-dys resulted in worse RV ejection fraction. However, larger LV intraventricular M-dys in the late diastolic phase indicated remarkable exercise capacity and higher LV stroke volume index. E-dys and intraventricular M-dys had no direct correlations. Conclusions: The layer-specific intraventricular M-dys had varying impacts on biventricular functions in PAH. PAH patients with LVmyoLSRTTLDPintra <20.01 milliseconds had a worse prognosis. LV intraventricular M-dys in the late diastolic phase needs more attention to precisely evaluate LV function.

Right heart thrombus (RHT) is a rare but life-threatening condition in acute pulmonary embolism (APE) without clear management guidelines. This study aimed to address the clinical characteristics and outcomes of RHT-APE in Chinese patients. In this study, 17 RHT-APE and 329 non-RHT-APE patients, who were diagnosed between September 2015 and August 2019, were retrospectively recruited with the median follow-up was 360 days. The overall prevalence of RHT was 4.91% in APE. Its prevalence increased along the increase of APE risk stratifications. Comparisons showed that with higher proportion of male gender and younger age, RHT-APE patients also had worse hemodynamic instability and heart function, and higher risk stratification levels than non-RHT-APE patients. After adjusting by age and gender, multivariate logistic regression analysis found high/intermediate-high risk stratification, decreased right ventricular (RV) motion, NT-proBNP >600 pg/mL, and RV dysfunction were risk factors for RHT. Kaplan-Meier analysis showed non-RHT had better prognosis than RHT patients (30-day survival: log-rank: p < 0.001; 90-day survival: log-rank: p = 0.002). The multivariate logistic regression analysis showed RHT was an independent risk factor for 30-day mortality in APE. The subgroup analysis showed RHT would result in worse outcomes in patients who already had higher APE early mortality risk. RHT would increase the risk of 30- and 90-day mortality in APE. More attention should be paid to young male APE patients with decreased RV motion, NT-proBNP >600 pg/mL, RV dysfunction, or high level of risk stratification, to exclude the coexistence of RHT.

Background Our objective was to clarify the characteristics and long-term survival of idiopathic pulmonary arterial hypertension (IPAH) patients with thyroid dysfunction and compare them with IPAH without thyroid dysfunction. Methods A retrospective analysis was conducted using prospectively collected data. IPAH patients with thyroid dysfunction at baseline were included. Patients with other subgroups of PAH and Group 2–5 pulmonary hypertension were excluded. IPAH patients with euthyroid function were matched 1:1 to IPAH patients with thyroid dysfunction by age and sex. Results In total, 148 IPAH patients with thyroid dysfunction were included. Patients with hyperthyroidism, hypothyroidism, subclinical hyperthyroidism and subclinical hypothyroidism accounted for 16.2%, 18.9%, 8.1% and 56.8%, respectively. IPAH patients with hyperthyroidism showed the highest mixed venous oxygen saturation (SvO2) and the lowest pulmonary vascular resistance (PVR) at baseline among subgroups, while patients with subclinical hypothyroidism had the lowest SvO2 and highest PVR (p<0.05). Compared with IPAH without thyroid dysfunction, patients with hyperthyroidism (9.14 versus 13.86 WU; p<0.05) and hypothyroidism (10.70 versus 13.86 WU; p<0.05) showed significantly lower PVR. The haemodynamic profiles of patients with subclinical hypothyroidism were similar to IPAH with euthyroid function except for lower right atrial pressure (6 versus 8 mmHg; p=0.009). The long-term survival of patients with clinical thyroid dysfunction was better than IPAH without thyroid dysfunction, while that of those with subclinical diseases was comparable to the latter, even after adjusting for baseline haemodynamics and treatment. Conclusion IPAH patients with clinical hyper- and hypothyroidism had better haemodynamics and survival than those without thyroid dysfunction, while patients with subclinical hypothyroidism had similar haemodynamics and survival profiles to the latter.

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. 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 cutoff 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 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.

BACKGROUND: The purpose of this registry was to provide insights into the characteristics, treatments and survival of patients with PAH-CHD in China.METHODS: Patients diagnosed with PAH-CHD were enrolled in this national multicenter prospective registry. Baseline and follow-up data on clinical characteristics, PAH-targeted treatments and survival were collected.RESULTS: A total of 1060 PAH-CHD patients (mean age 31 years; 67.9% females) were included, with Eisenmenger syndrome (51.5%) being the most common form and atrial septal defects (37.3%) com-prising the most frequent underlying defect. Approximately 33.0% of the patients were in World Health Organization functional class III to IV. The overall mean pulmonary arterial pressure and pulmonary vascular resistance were 67.1 (20.1) mm Hg and 1112.4 (705.9) dyn/s/cm5, respectively. PAH-targeted therapy was utilized in 826 patients (77.9%), and 203 patients (19.1%) received combination therapy. The estimated 1-, 3-, 5-, and 10-year survival rates of the overall cohort were 96.9%, 92.9%, 87.6% and 73.0%, respectively. Patients received combination therapy had significantly better survival than those with monotherapy (p = 0.016). NT-proBNP >1400 pg/ml, SvO2 & LE; 65% and Borg dyspnea index & GE; 3 and PAH-targeted therapy were independent predictors of mortality. Hemoglobin > 160g/L was a unique predictor for mortality in Eisenmenger syndrome.CONCLUSIONS: Chinese PAH-CHD patients predominantly exhibit Eisenmenger syndrome and have significantly impaired exercise tolerance and right ventricular function at diagnosis, which are closely associated with long-term survival. PAH-targeted therapy including combination therapy showed a favorable effect on survival in PAH-CHD. The long-term survival of Chinese CHD-PAH patients remains to be improved. J Heart Lung Transplant 2023;42:974-984 & COPY; 2023 International Society for Heart and Lung Transplantation. All rights reserved.

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.

Background::Coronary artery disease (CAD) is the commonest cause of heart failure (HF), whereas pulmonary hypertension (PH) has not been established or reported in this patient population. Therefore, we assessed the prevalence, risk factors, and survival in CAD-associated HF (CAD-HF) complicated with PH.Methods::Symptomatic CAD-HF patients were continuously enrolled in this prospective, multicenter registry study. Echocardiography, coronary arteriography, left and right heart catheterization (RHC), and other baseline clinical data were recorded. Patients were followed up and their survival was recorded.Results::One hundred and eighty-two CAD-HF patients were enrolled, including 142 with HF with a preserved ejection fraction (heart failure with preserved ejection fraction [HFpEF]; left ventricular ejection fraction [LVEF] ≥50%) and 40 with a reduced ejection fraction (heart failure with reduced ejection fraction [HFrEF]; LVEF < 50%). PH was diagnosed with RHC in 77.5% of patients. Patients with PH showed worse hemodynamic parameters and higher mortality. HFrEF-PH patients had worse survival than HFpEF-PH patients. CAD-HF patients with an enlarged left ventricular end-diastolic diameter and reduced hemoglobin were at higher risk of PH. Nitrate treatment reduced the risk of PH. Elevated creatinine and mean pulmonary arterial pressure (mPAP), diastolic pressure gradient (DPG) ≥7 mmHg, and previous myocardial infarction (MI) entailed a higher risk of mortality in CAD-HF patients with PH.Conclusions::PH is common in CAD-HF and worsens the hemodynamics and survival in these patients. Left ventricle enlargement and anemia increase the risk of PH in CAD-HF. Patients may benefit from nitrate medications. Renal impairment, elevated mPAP, DPG ≥7 mmHg, and previous MI are strong predictors of mortality in CAD-HF-PH patients.Trial Registration::ClinicalTrials.gov, NCT02164526.

Abstract Risk assessment for pulmonary arterial hypertension (PAH) utilizing noninvasive prognostic variables could be more practical in real‐world scenarios, especially at follow‐up reevaluations. Patients who underwent comprehensive evaluations both at baseline and at follow‐up visits were enrolled. The primary endpoint was all‐cause mortality. Predictive variables identified by Cox analyses were further incorporated with the French noninvasive risk prediction approach. A total of 580 PAH patients were enrolled. During a median follow‐up time of 47.0 months, 112 patients (19.3%) died. By multivariate Cox analyses, tricuspid annular plane systolic excursion (TAPSE), TAPSE/pulmonary arterial systolic pressure (PASP), and cardiopulmonary exercise testing‐derived peak oxygen consumption (VO2) remained independent predictors for survival. Regarding the French noninvasive risk prediction method, substituting N‐terminal pro‐b‐type natriuretic peptide (NT‐proBNP) with the newly derived low‐risk criteria of a TAPSE ≥ 17 mm or a TAPSE/PASP > 0.17 mm/mmHg, or alternating 6‐min walking distance with a peak VO2 ≥ 44 %predicted retained the discrimination power. When recombining the low‐risk criteria, the combination of World Health Organization functional class (WHO FC), TAPSE and peak VO2 at baseline, and the combination of WHO FC, NT‐proBNP, and peak VO2 at follow‐up showed better discriminative ability than the other combinations. In conclusion, Peak VO2, TAPSE, and TAPSE/PASP are significant prognostic predictors for survival in PAH, with incremental prognostic value when incorporated with the French noninvasive risk prediction approach, especially at reevaluations. For better risk prediction, WHO FC, at least one measurement of exercise capacity and one measurement of right ventricular function should be considered.

Background Patients with left heart failure (LHF) are often associated with the development of pulmonary hypertension (PH) which leads to an increased risk of death. Recently, the diagnostic standard for PH has changed from mean pulmonary arterial pressure (mPAP) ≥25 mmHg to >20 mmHg. Nonetheless, the effect of borderline PH (mPAP: 21–24 mmHg) on the prognosis of LHF patients is unclear. This study aimed to investigate the relationship between borderline PH and 3-year clinical outcomes in LHF patients. Methods A retrospective analysis of a prospective cohort study was done for LHF patients who underwent right heart catheterization (RHC) between January 2013 and November 2016. The primary outcome was all-cause mortality; the secondary outcome was rehospitalization. Results Among 344 patients, 62.5% were identified with a proportion of PH (mPAP ≥ 25), 10.8% with borderline PH (21–24), and 26.7% with non-PH (≤20), respectively. Multivariable Cox analysis revealed that borderline PH patients had a higher adjusted mortality risk (HR = 3.822; 95% CI: 1.043–13.999; p = 0.043) than non-PH patients. When mPAP was treated as a continuous variable, the hazard ratio for death increased progressively with increasing mPAP starting at 20 mmHg (HR = 1.006; 95% CI: 1.001–1.012). There was no statistically significant difference in adjusted rehospitalization between borderline PH and non-PH patients (HR = 1.599; 95% CI: 0.833–3.067; p = 0.158). Conclusions Borderline PH is independently related to increased 3-year mortality in LHF patients. Future research is needed to evaluate whether more close monitoring, and managing with an intensifier improves clinical outcomes in borderline PH caused by LHF. Clinical trials registration www.clinicaltrials.gov NCT02164526.