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BACKGROUND: Arrhythmia is not uncommon among pulmonary hypertension (PH) population, and may be associated with disease severity. HYPOTHESIS: To investigate different spectrums and prevalence of arrhythmias in different clinical PH groups in Chinese population. METHODS: Patients diagnosed with PH between April 15, 2019, and August 2, 2021, were enrolled prospectively. The prevalence of different types of arrhythmias in PH patients were calculated. Logistic regression analyses were conducted to determine independent predictors for arrhythmia. RESULTS: One thousand patients were enrolled. The prevalence of any arrhythmia, sinus node dysfunction, sinus tachycardia, atrial fibrillation, atrial flutter, other types of atrial tachycardia, atrioventricular block, and ventricular tachycardia is 44.4%, 12.2%, 15.2%, 8.1%, 4.1%, 10.2%, 7.1%, and 2.5%. Logistic regression analyses revealed that older age and larger right ventricle (odds ratio: 1.111 and 1.095, p

BACKGROUND: In the 6(th) World Symposium on Pulmonary Hypertension (PH), the hemodynamic definition of PH was reduced from a mean pulmonary artery pressure (mPAP) ≥25 to >20 mmHg. This study aimed to evaluate the impact of the revised hemodynamic definition on the diagnosis of precapillary PH. METHODS: We retrospectively enrolled patients who underwent right heart catheterization from January 2012 to December 2018. All included patients were reassessed according to the revised hemodynamic definition. RESULTS: A total of 1,251 patients were included for analysis, of whom 1,044 patients had precapillary PH and 182 patients had mPAP

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).

Dexmedetomidine is an α2 adrenoceptor agonist and has cardioprotective effect,the mechanism of which is being studied.Increasing studies have proved the clinical value of dexmedetomidine in reducing postoperative complications and improving the prognosis of patients.Therefore,this review summarizes the cardiac protection mechanism of dexmedetomidine based on the existing studies and expounds the application of dexmedetomidine in the perioperative period of cardiovascular surgery.

Objectives: The study compared the diagnostic performance of Planar Ventilation/perfusion (V/Q) and V/Q Single-photon computed tomography (SPECT), and determined whether combining perfusion scanning with low-dose computed tomography (Q-LDCT) may be equally effective in a prospective study of patients with chronic thromboembolic pulmonary hypertension (CTEPH) patients. Background: V/Q scanning is recommended for excluding CTEPH during the diagnosis of pulmonary hypertension (PH). However, Planar V/Q and V/Q SPECT techniques have yet to be compared in patients with CTEPH. Methods: Patients with suspected PH were eligible for the study. PH attributable to left heart disease or lung disease was excluded, and patients whose PH was confirmed by right heart catheterization and who completed Planar V/Q, V/Q-SPECT, Q-LDCT, and pulmonary angiography were included. V/Q images were interpreted and patients were diagnosed as instructed by the 2009 EANM guidelines, and pulmonary angiography analyses were used as a reference standard. Results: A total of 208 patients completed the study, including 69 with CTEPH confirmed by pulmonary angiography. Planar V/Q, V/Q-SPECT, and Q-LDCT were all highly effective for diagnosing CTEPH, with no significant differences in sensitivity or specificity observed among the three techniques (Planar V/Q [sensitivity/specificity]: 94.20%/92.81%; V/Q-SPECT: 97.10%/91.37%, Q-LCDT: 95.65%/90.65%). However, V/Q-SPECT was significantly more sensitive (V/Q-SPECT: 79.21%; Planar V/Q: 75.84%, P = 0.012; Q-LDCT: 74.91%, p

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.

MicroRNAs are key molecules involved in the regulation of endothelial function. They are important risk factors and biomarkers for the development of hypertension related to endothelial dysfunction. However, the gene expression patterns associated with hypertension development related to endothelial dysfunction have not been fully elucidated. We conducted a case-control study of 65 patients with essential hypertension (EH) and 61 controls without EH. Plasma levels of miR-122 and its target protein high-affinity cationic amino acid transporter 1 (CAT-1) were measured by qRT-PCR and ELISA, respectively. miR-122 expression in plasma of patients with EH was significantly higher than that of the control group (p = 0.001), while CAT-1 expression in patients with EH was significantly lower than that in the control group (p = 0.018). miR-122 expression in plasma of young patients with EH was significantly higher than that in young people without EH (p = 0.0004), and CAT-1 expression in plasma of young patients with EH was also significantly lower than that of the control group (p = 0.002). CAT-1 expression in the plasma of young participants was significantly higher than that of individuals aged ≥40 years (p = 0.003), whereas miR-122 expression was significantly lower (p = 0.001). We showed that among patients with EH, the high expression of miR-122 contributed to endothelial dysfunction by suppressing the expression of the CAT-1 protein, which led to a decrease in CAT-1 expression in plasma. Therefore, high expression of miR-122 appears to be a risk factor for endothelial dysfunction in EH, especially in younger patients.

Sudden cardiac death(SCD),a serious public health problem facing China and the world,causes heavy social burden.It is reported that SCD accounts for 15%-20% of all the deaths and the proportion of SCD patients with non-cardiac disease is as high as 50%.Finding effective predictors to identify SCD early is particularly important for clinical practice.To date,non-invasive electrocardiologic examination has been the first choice for predicting the risks of fatal ventricular arrhythmias and SCD because of its safety and feasibility.This review summarizes the available relevant studies to expound the value of non-invasive electrocardiologic examination and indicators in predicting SCD.

With the wide application of vascular Doppler ultrasound as well as the improvement of instrument precision and personnel operation practice,calf muscular venous thrombosis(CMVT)has been more and more frequently diagnosed among patients,becoming a common clinical disease.However,it is controversial about the anticoagulant therapy for CMVT patients.In this article,we summarize the current research progress of anticoagulation therapy,aiming to provide reference for the treatment of CMVT.

Resting two-dimensional speckle tracking echocardiography (2D-STE) identified right ventricular (RV) systolic function were reported to predict exercise capacity in pulmonary hypertension (PH) patients, but little attention had been payed to 2D-STE detected RV diastolic function. Therefore, we aim to elucidate and compare the relations between 2D-STE identified RV diastolic/systolic functions and peak oxygen consumption (PVO2) determined by cardiopulmonary exercise testing (CPET) in pre-capillary PH. 2D-STE was performed in 66 pre-capillary PH patients and 28 healthy controls. Linear correlation and multivariate regression analyses were performed to evaluate and compare the relations between RV 2D-STE parameters and PVO2. Receiver operating characteristic curves were used to compare the predictive value of 2D-STE parameters in predicting the cut-off—PVO2 − 0.65/s had a 88.2% sensibility and 82.2% specificity to predict PVO2

Background Pulmonary hypertension (PH) is defined as a mean pulmonary arterial pressure (PAP) ≥25 mm Hg measured by right heart catheterization. However, the upper limit of a normal mean PAP is 20 mm Hg. There is a gap between the upper limit of normal and the threshold for diagnosing PH. Therefore, we aimed to investigate whether individuals with a mildly elevated PAP, defined as 20 mm Hg < mean PAP Methods and Results We reviewed studies evaluating the risk of progression to PH and/or mortality in individuals with a mildly elevated PAP versus those with a normal PAP. The mean PAP value of each participant was confirmed by right heart catheterization. We reviewed 1213 studies and 8 fulfilled our inclusion criteria. Our results indicated that individuals with a mildly elevated PAP were 1.81 to 2.45 times more likely to progress to PH than individuals with a normal PAP. There was a statistically significant difference in mortality between the mildly elevated PAP and normal PAP groups (hazard ratio, 2.48; 95% CI, 1.69–3.64). We also pooled survival probabilities in each arm to obtain a summary survival curve for each group, and the pooled survival rates in the mildly elevated PAP group were numerically lower than those in the normal PAP group. Conclusions Our study revealed that individuals with a mildly elevated PAP were at an increased risk of progression to PH and mortality than those with a normal PAP.

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.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex chronic disease in which pulmonary artery stenosis or obstruction caused by organized thrombus can lead to increased pulmonary artery pressure and pulmonary vascular resistance, ultimately triggering progressive right heart failure and death. Currently, its exact mechanism is not fully understood. Pulmonary endarterectomy (PEA) has immediate effects with low perioperative mortality and satisfactory prognosis in experienced expert centers for CTEPH patients with proximal lesions. Nevertheless, 37% of patients are deemed unsuitable for PEA surgery due to comorbidities and other factors, and nearly half of the operated patients have residual or recurrent pulmonary hypertension. Riociguat is the only approved drug for CTEPH, although its effect is limited. Balloon pulmonary angioplasty (BPA) is a promising alternative treatment for patients with CTEPH. After more than 30 years of development and refinements, emerging evidence has confirmed its role in patients with inoperable CTEPH or residual/recurrent pulmonary hypertension, with acceptable complications and comparable long-term prognosis to PEA. This review summarizes the pathophysiology of CTEPH, BPA history and development, therapeutic principles, indications and contraindications, interventional procedures, imaging modalities, efficacy and prognosis, complications and management, bridging and hybrid therapies, ongoing clinical trials and future prospects.

Background Variants in the gene encoding bone morphogenetic protein receptor type II (BMPR2) are the most common genetic cause of pulmonary arterial hypertension (PAH), whereas biallelic variants in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) are described in pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis (PVOD/PCH). Racial background may influence the clinical characteristics of patients diagnosed with PAH or PVOD/PCH. Here, we compared the clinical characteristics and survival between patients with BMPR2 variants or EIF2AK4 variants in a Chinese population. Methods Heterozygous variants in BMPR2 and homozygous or compound heterozygous biallelic EIF2AK4 variants predicted to be deleterious were identified as potentially causal. Clinical and radiological data were collected and analysed. The primary outcomes were death or lung transplantation. Hazard ratios (HRs) for death or transplantation associated with the presence of BMPR2 or biallelic EIF2AK4 variants were calculated using Cox proportional hazards models to analyse patient survival. Results Two hundred thirty-two patients with PAH were enrolled for genetic testing, and PAH patients with associated conditions were excluded from the study. Forty-five patients with BMPR2 variants and 11 patients with biallelic EIF2AK4 variants were recruited. PAH patients with BMPR2 or biallelic EIF2AK4 variants presented symptoms at the ages of 25.57 ± 10.17 years and 31.6 ± 9.38 years, respectively. The whole group of patients showed female dominance either with BMPR2 variants or biallelic EIF2AK4 variants. Specific radiological abnormalities are more prominent in EIF2AK4 variant carriers but can also be found in some patients with BMPR2 variants. Biallelic EIF2AK4 variant carriers had worse survival than BMPR2 variant carriers (p Conclusions Clinical pictures of PAH patients with BMPR2 and biallelic EIF2AK4 variants in the Chinese population differ from other populations by a younger age at diagnosis and demonstrate female dominance in the whole patient group. High-resolution chest CT can help assist in differentiating PAH with PVOD/PCH. BMPR2 variants and biallelic EIF2AK4 variants are associated with adverse outcomes, but the survival of patients with biallelic EIF2AK4 variants is dismal.

Background Accurate methods for identifying obstructions in both large and small vessels are crucial for diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Purpose To compare the performance of ventilation-perfusion (V/Q) scanning, V/Q SPECT, and CT pulmonary angiography (PA) in CTEPH by using digital subtraction PA as the reference standard. Materials and Methods This prospective study was conducted from January 2016 to January 2018. A total of 229 participants suspected of having CTEPH were evaluated with V/Q SPECT, V/Q planar scintigraphy, CT PA, and digital subtraction PA. Participants underwent all four procedures within 1 week. Differences in the diagnostic performance of V/Q SPECT, V/Q planar scintigraphy, and CT PA were evaluated with areas under the curve receiver operator curve, the McNemar test, and generalized estimating equations analysis. Results A total of 150 participants (mean age, 42 years ± 15 [standard deviation]; 99 women) were enrolled. Digital subtraction PA assessments confirmed CTEPH in 51 participants and indicated that 602 of 1020 lung segments (20 segments per participant) were obstructed. The three imaging methods showed high sensitivity (V/Q SPECT, 98%; V/Q planar scintigraphy, 98%; CT PA, 94%) and specificity (V/Q SPECT, 89%; V/Q planar scintigraphy, 91%; CT PA, 96%) (all P > .05). However, both V/Q scanning techniques were more sensitive (V/Q SPECT: 85%, P .05), for individual analysis, and 0.64, 0.67, and 0.64, respectively, by segment (V/Q planar scintigraphy vs V/Q SPECT, P = .02; V/Q planar scintigraphy vs CT PA, P = .08; V/Q SPECT vs CT PA, P = .94). Conclusion Ventilation-perfusion scanning was more sensitive and less specific than was CT pulmonary angiography for detecting vascular obstructions at the segmental pulmonary arterial level. © RSNA, 2020 See also the editorial by Swift and Rajaram in this issue.

BackgroundHuman epididymis protein 4 (HE4) was proved to be a novel biomarker for left heart failure. The purpose of this exploratory study was to evaluate the role of HE4 in patients with idiopathic pulmonary arterial hypertension (IPAH) who usually have concurrent right heart failure.Methods55 patients with newly diagnosed IPAH were continuously enrolled and serum HE4 levels were assessed at baseline. All patients were followed up from the date of blood sampling, and a composite endpoint of clinical worsening was detailedly recorded.ResultsSerum levels of HE4 were significantly higher in IPAH patients than healthy controls (6.9 ± 2.2 vs 4.4 ± 0.9 ng/ml,p r = 0.331,p r = 0.30,p 6.5 ng/ml discriminated clinical worsening with a sensitivity of 92.31% and a specificity of 59.52% (area under the curve [AUC] = 0.81). Multivariate Cox regression analysis demonstrated that HE4 (χ2: 5.10; hazard ratio [HR] = 1.26; 95% confidence interval: 1.03 to 1.55,p 2: 4.19; HR = 1.14; 95% confidence interval: 1.00–1.29,p 6.5 ng/ml had a worse 2-year survival rate than those with HE4 ≤ 6.5 ng/ml (58.9% vs 96.2%,p ConclusionsSerum levels of HE4 were elevated in IPAH patients and correlated with disease severity. HE4 was an independent predictor of clinical worsening in IPAH patients.

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.

Background The reversibility of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) is of great importance for the operability of CHD. Proteomics analysis found that transgelin was significantly up‐regulated in the lung tissue of CHD‐PAH patients, especially in the irreversible group. However, how exactly it participated in CHD‐PAH development is unknown. Methods Immunohistochemical staining and Western blot were performed for further qualitative and quantitative analysis of transgelin in the lung tissues of CHD‐PAH patients. The mechanism of transgelin in CHD‐PAH development was explored in vitro. Primary human pulmonary arterial smooth muscle cells (hPASMCs) were cultured and infected with TAGLN siRNA or TAGLN lentiviral vector. Cell morphologic change (Coomassie Brilliant Blue staining), proliferation (cell count and EdU assay), apoptosis (terminal deoxyribonucleotidyl transferase mediated dUTP nick end labeling assay and Annexin‐V flow cytometry) and migration (transwell) were evaluated following the cell treatment. The mRNA and protein expression levels were detected in real‐time PCR and Western blot. Results In line with the proteomic findings, transgelin was obviously expressed in PASMC of the middle pulmonary arterioles, especially in the irreversible PAH group. Also, transgelin expression showed positive relation with pathological grading. Experiment in vitro demonstrated that transgelin overexpression promoted PASMC proliferation and migration, strengthened cytoskeleton and was accompanied by increased expression of synthetic phenotype markers (osteopontin, proliferating cell nuclear antigen) and anti‐apoptotic protein (bcl‐2). On the other hand, suppression of transgelin expression activated PASMC apoptosis, reducing cell proliferation and migration. Conclusions Transgelin may be a potential target in the development of irreversible CHD‐PAH through inducing PASMC phenotype change, proliferation, migration and reducing cell apoptosis.

BackgroundPulmonary veno-occlusive disease (PVOD) is a rare condition with poor prognosis, and lung transplantation is recommended as the only curative therapy. The role of pulmonary arterial hypertension targeted therapy in PVOD remains controversial, and long-term effects of targeted therapy have been rarely reported. This study aims to retrospectively evaluate the role of targeted therapy in PVOD patients and the long-term outcome.MethodsPVOD patients with good responses to targeted therapies were analyzed, and data pre- and post- targeted therapies were compared. An overview of the effects of targeted therapies on PVOD patients was also conducted.ResultsFive genetically or histologically confirmed PVOD patients received targeted therapies and showed good responses. Their mean pulmonary arterial pressure by right heart catheterization was 62.0 ± 11.7 mmHg. Two receiving monotherapy got stabilized, and three receiving sequential combination therapy got improved, cardiac function and exercise capacity significantly improved after treatments. No pulmonary edema occurred. The mean time from the first targeted therapy to the last follow up was 39.3 months, and the longest was 9 years. A systematic review regarding the effects of targeted therapies on PVOD patients indicated majorities of patients got hemodynamics or 6-min walk distance improved, and 26.7% patients developed pulmonary edema. The interval from targeted drugs use to death ranged from 71 min to over 4 years.ConclusionsCautious use of targeted therapy could safely and effectively improve or stabilize hemodynamics and exercise capacity of some patients without any complications. PVOD patients could live longer than expected.

BackgroundBalloon pulmonary angioplasty (BPA) has been demonstrated to improve cardiac function and exercise capacity in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but its instant impact on cardiopulmonary function has seldom been evaluated. This study aims to determine the safety and efficacy of BPA and its immediate and lasting effects on cardiopulmonary function among CTEPH patients.MethodsFrom May 2018 to January 2019, patients with inoperable CTEPH who underwent BPA sessions were consecutively enrolled. Hemodynamics were measured by right heart catheterization, selective pulmonary angiography and BPA were successively conducted. Hemodynamic variables, WHO functional class (WHO-FC), 6-min walk distance (6MWD) and serum NT-proBNP were evaluated before and after BPA sessions during hospitalization. Pulmonary function testing (PFT) and cardiopulmonary exercise testing (CPET) were performed within 1–3 days pre and post BPA to evaluate the effect of BPA on cardiopulmonary function.ResultsTwenty-five patients with inoperable CTEPH who underwent a total of forty BPA sessions were consecutively enrolled. A total of 183 segmental or subsegmental vessels (4.6 ± 1.9 vessels per session) in 137 segments (3.4 ± 1.6 segments per session) were dilated. No procedure-related complications occurred. Instant hemodynamics, WHO-FC, 6MWD and NT-proBNP were all significantly improved after a single BPA session. Significant improvement in cardiopulmonary function was also evident as assessed by PFT indexes (forced vital capacity, forced expiratory volume in the first second, maximal voluntary ventilation) and CPET parameters (peak work rate, peak VO2, oxygen uptake efficiency slope). Further analysis among ten CTEPH patients receiving multiple BPA sessions (2–4 sessions) indicated BPA resulted in lasting improvements in hemodynamics and cardiopulmonary function.ConclusionsBPA, a safe and effective approach, can bring instant improvements after a single session and lasting benefits after multiple sessions to hemodynamics and cardiopulmonary function for patients with inoperable CTEPH.

Pulmonary arterial hypertension (PAH) is a severe complication of hereditary hemorrhagic telangiectasia (HHT); however, little is known about its clinical characteristics and prognosis. Nine newly diagnosed HHT-PAH patients were prospectively recruited between October 2007 and January 2016 and were followed up every half-year. Eighteen idiopathic pulmonary arterial hypertension (IPAH) patients, matched with HHT-PAH patients on mean pulmonary arterial pressure, pulmonary capillary wedge pressure, pulmonary vascular resistance, cardiac index, and World Health Organization (WHO) functional class (FC), were recruited. The clinical characteristics of HHT-PAH patients were described and the prognosis of these two cohorts were compared. Of HHT-PAH patients, 55.56% were WHO FC III. Kaplan–Meier survival analysis showed one- and three-year survival rates of HHT-PAH patients were 77.8% and 53.3% respectively, which were worse than matched IPAH patients (log rank: P = 0.047). HHT-PAH patients had higher red cell distribution width (14.88 ± 2.93% versus 13.19 ± 0.83%, P = 0.031), larger right ventricular anteroposterior diameter (34.67 ± 6.67 mm versus 28.56 ± 6.35 mm, P = 0.029), and lower mean corpuscular hemoglobin concentration (317.38 ± 17.71 g/L versus 335.72 ± 14.68 g/L, P = 0.011) than matched IPAH patients. Multivariate Cox proportional hazards regression analyses showed baseline total bilirubin independently predicted the mortality of HHT-PAH after adjusting by age, cardiac index, mixed venous oxygen saturation, or serum uric acid. HHT-PAH patients may have a worse prognosis than matched IPAH patients. Baseline total bilirubin may be a promising predictor for the long-term prognosis in HHT-PAH patients.

Background Studies have shown that peak circulatory power (peak CircP; peak oxygen uptake × peak systolic blood pressure) is a variable predictor of prognosis in patients with left heart failure. It remains unknown whether peak CircP also predicts outcome in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods Patients with newly diagnosed IPAH who underwent symptom-limited cardiopulmonary exercise testing (CPET) from 1 January 2011 to 1 January 2014 in Fuwai Hospital were prospectively enrolled and followed for up to 66 months for cardiac events (mortality and lung transplantation). Results One hundred forty patients with IPAH (104 female, mean age: 33 ± 11 years) were studied. During follow-up (mean: 42 ± 14 months), 24 patients died and 1 patient underwent lung transplantation. In the univariate analysis, peak oxygen uptake(VO2), oxygen uptake at anaerobic threshold, ventilation (VE)/carbon dioxide output (VCO2) slope, end-tidal partial pressure of carbon dioxide at anaerobic threshold, peak systolic blood pressure (SBP), the change of SBP, the change of heart rate, peak work rate, peak CircP, pulmonary vascular resistance, cardiac index and World Health Organization functional class were predictive of cardiac events (all P Conclusions Peak CircP, better than peak VO2 and VE/VCO2slope, was a strong predictor of cardiac events among exercise parameters in patients with IPAH.

Objective: To present our treatment experiences and the follow-up data of patients with paradoxical embolism (PDE). Methods: The clinical characteristics, management, and follow-up data of all included patients who were diagnosed with PDE at Fuwai Hospital from January 1994 to October 2015 were recorded. Results: Twelve patients were included; all had a pulmonary embolism, and 8 had deep venous thrombosis. The artery embolisms involved the cerebral artery (7 patients), renal artery (2 patients), mesentery artery (2 patients), popliteal artery (1 patient), descending aorta thrombus (1 patient), and thrombus-straddled patent foramen ovale (PFO) (1 patient). PFO was found in 3 cases. One patient underwent thrombectomy and PFO closure; Six patients received thrombolysis; and 3 patients were implanted with a vena cava filter. Long-term anticoagulation with warfarin was recommended for each patient. One patient died from ventricular fibrillation despite cardiopulmonary resuscitation. Eleven patients were discharged with improvements. No late mortality occurred in 8 patients with a complete follow-up of 10.6â17.7 years. One had a recurrent deep venous thrombosis. No patient had a recurrent pulmonary or arterial embolism. Two patients changed their treatment from warfarin to aspirin; others remained on warfarin. Only 1 case had an occasional gum bleeding. Conclusions: PDE treatment including thrombolysis, anticoagulation, and embolectomy should be individualized. We recommend long-term anticoagulation therapy to prevent the recurrence of PDE, especially to those with an intracardiac communication or persistent risk factors for re-thrombosis. Keywords: Paradoxical embolism, Pulmonary embolism, Deep venous thrombosis, Patent foramen ovale

Association between electrocardiography (ECG) features and right ventricular anatomy and physiology has been established. This study is aimed to identify the value of 12-lead ECG in evaluating prognosis of patients with idiopathic pulmonary arterial hypertension (IPAH). 194 patients with newly diagnosed IPAH were included in this study. Correlations between electrocardiography variables and hemodynamics were assessed. Univariate and multivariable cox regression analysis were performed to identify ECG variables for predicting all-cause mortality in IPAH. Partial correlation analysis showed that P wave amplitude in lead II correlated with the mean pulmonary arterial pressure (mPAP, r = 0.349, p ≤ 0.001) and cardiac index (CI, r = −0.224, p = 0.002); R wave amplitude in V1 correlated with mPAP (r = 0.359, p ≤ 0.001); S wave amplitude in V6 correlated with mPAP (r = 0.259, p = 0.030) and CI (r = −0.220, p = 0.003). P wave amplitude in lead II (HR 1.555, p = 0.033) and R wave amplitude in lead aVR (HR 5.058, p

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

Idiopathic pulmonary arterial hypertension is a progressive disease with high mortality with an increasing burden of right ventricular. Right ventricular dyssynchrony was observed in idiopathic pulmonary arterial hypertension, but the association with mortality is unclear. This study aimed to investigate the impact of right ventricular dyssynchrony on the survival of idiopathic pulmonary arterial hypertension. A total of 116 patients with idiopathic pulmonary arterial hypertension were enrolled in this study. All these patients underwent comprehensive clinical evaluation. Right ventricular dyssynchrony was assessed by two-dimensional speckle-tracking echocardiography. The time to peak strain (T peak ) of right ventricular segments were obtained. Right ventricular dyssynchrony was quantified by the standard deviation of the heart rate-corrected T peak of right ventricular four segments. All patients were followed up and the primary endpoint was all cause of death. Results found patients with significant right ventricular dyssynchrony present with advanced World Health Organization functional class, worse hemodynamic status and right ventricular function. Right ventricular dyssynchrony was an independent predictive factor for the survival of idiopathic pulmonary arterial hypertension. Kaplan–Meier survival curves showed patients with right ventricular dyssynchrony had worse prognosis. In conclusion, right ventricular dyssynchrony analyzed by speckle-tracking echocardiography provided added value to hemodynamic and echocardiographic parameters in evaluating the survival of patients with idiopathic pulmonary arterial hypertension.

Background Pulmonary hypertension (PH) patients have poor prognosis due to progressive right ventricular (RV) dysfunction. As a low-cost and non-invasive tool, echocardiography is by far the most widely used technique to investigate the RV structure and function in PH patients. Recent studies showed that RV longitudinal strain (RVLS) measured by two- or three-dimensional echocardiography (2DE, 3DE) was correlated with RV function parameters and have the potential to predict the prognosis of PH patients. However, few studies have compared the value of 2DE- and 3DE- RVLS to predict disease severity of pre-capillary PH patients. Therefore, our study aims to compare the capacity of RVLS assessed by 3DE and 2DE in predicting disease severity of pre-capillary PH patients. Methods We consecutively enrolled 57 patients (18 males and 39 females, 35±13 years) with pre-capillary PH diagnosed by right heart catheterization in our center. Standard transthoracic echocardiography was performed in all participants. 2DE- RVLS were obtained from speckle-tracking analyses using GE EchoPAC version 201; while 3DE- RVLS were analyzed by TomTec 4D RV-Function 2.0. On the basis of the risk assessment strategy of 2015 ESC Guidelines for the diagnosis and treatment of pulmonary hypertension, all the participants were classified into low risk or intermediate-high risk groups. Linear regression analyses were performed to evaluate the correlations between RVLS and peak oxygen consumption (PVO2). In addition, receive operating characteristic curves (ROC) were used to compare the predictive values of 2DE- and 3DE-RVLS and identify the optimal cut points for the detection of low risk based on the risk assessment strategy of 2015 ESC Guidelines. Results Linear regression analyses showed a significant correlation between PVO2 and 2DE- RVLS (r=−0.484, P Conclusions Both two- and three-dimensional echocardiography detected RVLS had the potential to evaluate disease severity of pre-capillary PH patients, but the former may have a better predictive capacity.

We investigated and compared the correlations between two-dimensional speckle tracking echocardiography detected left ventricular peak early diastolic strain rates (global: left ventricular global peak early diastolic strain rate; septum: left ventricular peak early diastolic strain rate of septum; free wall: left ventricular peak early diastolic strain rate of free wall) and disease severity in pre-capillary pulmonary hypertension. Seventy-four pre-capillary pulmonary hypertension patients (23 males and 51 females, 35 ± 13 years) and thirty healthy controls were consecutively recruited for two-dimensional speckle tracking echocardiography analyses in our study. Medical records of pre-capillary pulmonary hypertension patients were reviewed to capture clinical data; risk assessments were performed based on the 2015 ESC Guidelines. Compared with healthy controls, left ventricular global peak early diastolic strain rate was lower in pre-capillary pulmonary hypertension patients (1.11 ± 0.60 s −1 versus 1.47 ± 0.45 s −1 , P = 0.001), especially that of the septum (1.13 ± 0.58 s −1 versus 1.68 ± 0.46 s −1 , 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

Both portal hypertension and splenectomy are risk factors for pulmonary hypertension. However, the interactions between portal hypertension and splenectomy in the development of pulmonary hypertension remain unclear. Twelve newly diagnosed pulmonary hypertension patients with a previous history of splenectomy induced by portal hypertension were recruited between November 2008 and May 2017. We compared their clinical features, hemodynamics, and prognosis with idiopathic pulmonary arterial hypertension patients, who were matched by cardiac index, mean pulmonary arterial pressure, and pulmonary vascular resistance. We also compared the clinical characteristics of portal hypertension-post-splenectomy-pulmonary hypertension patients with eight portopulmonary hypertension patients. Compared with the matched idiopathic pulmonary arterial hypertension patients, the portal hypertension-post-splenectomy-pulmonary hypertension patients showed significantly wider red blood cell distribution width (16.7 ± 2.8% versus 13.3 ± 1.7%, p = 0.004), higher total bilirubin concentration (31.0 ± 13.8 µmol/l versus 18.9 ± 10.0 µmol/l, p = 0.010), and higher lactate dehydrogenase concentration (321.5 ± 41.2 IU/l versus 229.2 ± 69.4 IU/l, p = 0.001). Kaplan–Meier survival analyses showed that the portal hypertension-post-splenectomy-pulmonary hypertension patients tended to have poorer prognosis than the matched idiopathic pulmonary arterial hypertension patients (log-rank test: p = 0.010). Compared with the portal hypertension-post-splenectomy-pulmonary hypertension patients, the portopulmonary hypertension cohort appeared to exhibit poorer clinical conditions, including significantly lower mixed venous oxygen saturation (62.9 ± 8.0% versus 73.9 ± 6.5%, p = 0.004) and a significantly higher proportion of pericardial effusion (75.0% versus 8.3%, p = 0.004), even though the two cohorts showed similar hemodynamics. The mean intervals from diagnosis of portal hypertension to pulmonary hypertension in portopulmonary hypertension patients were significantly shorter than the intervals from splenectomy to diagnosis of pulmonary hypertension in portal hypertension-post-splenectomy-pulmonary hypertension patients (5.5 ± 5.2 years versus 13.1 ± 5.9 years, p = 0.008). Splenectomy might be involved in the initiation and development of pulmonary hypertension in patients with portal hypertension, although the precise mechanisms involved remain unknown. Portal hypertension-post-splenectomy-pulmonary hypertension patients might have poorer prognosis even with mild hemodynamics.

The ventilation/perfusion lung scan is recommended to exclude chronic thromboembolic pulmonary hypertension in the diagnostic algorithm of pulmonary hypertension, but its role in pulmonary arterial hypertension (PAH) has not been well explored. We characterized the lung perfusion pattern assessed by lung perfusion scintigraphy in idiopathic PAH (IPAH) patients and evaluate the potential prognostic significance of the patchy pattern perfusion defect. A total of 318 patients with IPAH confirmed by right heart catheterization who performed lung perfusion scintigraphy were included. On lung perfusion scintigraphy, 134 patients had normal lung perfusion and 184 patients showed patchy perfusion defects. In comparison to patients with normal lung perfusion, patients with patchy perfusion defects experienced significantly higher mean pulmonary arterial pressure (58.0 ± 15.4 mmHg vs. 54.1 ± 16.2 mmHg, P = 0.027) and total pulmonary resistance (1192.6 ± 533.7 dyn·s·cm −5 vs. 1067.2 ± 549.3 dyn·s·cm −5 , P = 0.042). During a median follow-up period of 884.0 days, 53 patients reached the primary endpoint of all-cause mortality. On univariate Cox analysis, the patchy pattern of perfusion defect was significantly associated with the all-cause mortality (hazard ratio [HR] = 2.47, 95% confidence interval [CI] = 1.32–4.63, P = 0.005). Patients with patchy perfusion defects had a worse outcome (log-rank = 8.605, P = 0.003). On multivariate analysis, the patchy pattern remained as a significant independent predictor of the endpoint (HR = 2.30, 95% CI = 1.22–4.31, P = 0.010). IPAH patients presented with heterogeneity in lung perfusion and the patchy pattern of lung perfusion defect commonly existed. Patients with patchy pattern identified by lung perfusion scintigraphy were associated with more severe disease and worse outcome.

Background and objective The potential prognostic value of cardiovascular magnetic resonance (CMR)-derived right ventricular (RV) ejection/filling parameters in the assessment of RV function remains to be fully established. The goal of this study was to explore the prognostic value of these parameters in idiopathic pulmonary arterial hypertension (IPAH) patients. Methods In this prospective investigation, newly diagnosed IPAH patients without targeted therapy were recruited. Patients underwent right heart catheterization (RHC), 6-min walk test and CMR imaging within 1 week. Cardiopulmonary haemodynamics derived from RHC, and RV ejection/filling parameters derived from CMR were collected. Results A total of 41 IPAH patients were recruited with the median follow-up time of 824 days. During this period, 13 patients significantly deteriorated and among them, 7 patients died. CMR-derived RV peak ejection rate (PER) correlated significantly with CMR-derived RV stroke volume index (r = 0.574, P