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BACKGROUND Acute vasoreactivity testing (AVT) which reflects the compliance of the pulmonary vascular bed has been proven to be of prognostic value. The purpose of the present study is to explore the sex differences of hemodynamics during the AVT and their impact on event-free survival in patients with chronic thromboembolic pulmonary hypertension (CTEPH). METHODS Eighty-six patients underwent a right heart catheterization and AVT at Shanghai Pulmonary Hospital from February 2009 to February 2018. Univariate and multiple stepwise regression analysis were performed to determine the predictors of independent event-free survival, and receiver operating characteristic curve was plotted to determine the cut-off value of independent parameters in CTEPH. RESULTS There were no significant differences in both demographics and hemodynamics between male and female patients with CTEPH. Except ΔPVR/PVR showed a significantly higher difference in female than male patients (P = 0.034). Male patients had higher mRAP of pre- and post-AVT than female patients in the event-free subgroup, while, female patients showed higher PVR of pre-AVT than male patients in the event subgroup (P

Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). This study aimed to explore the prevalence, clinical features, hemodynamic characteristics and prognosis of different severity of ILD in a cohort of patients with SSc-associated precapillary pulmonary hypertension (SSc-PH) and investigate the differences between SSc-PAH and idiopathic pulmonary arterial hypertension (IPAH) patients.SSc-PH patients and IPAH patients, admitted to Shanghai Pulmonary Hospital (August 1, 2008-January 31, 2020) and diagnosed by right-sided heart catheterization (RHC) or echocardiography, were retrospectively included. SSc-PH patients had a baseline chest high-resolution computed tomography (HRCT), and PH classification was based on the extent of ILD. Clinical, pulmonary function, hemodynamic characteristics and survival data were extracted.The study included 45 SSc-PH patients (60% had coexisting ILD and 77.8% were SSc-Group 1 PH/SSc-PAH [without ILD or with mild ILD], 22.2% were SSc-Group 3 PH/SSc-PH with severe ILD) and 52 IPAH patients. SSc-PH with ILD had lower arterial oxygen partial pressure (PaOILD was common in SSc-PH patients. Careful phenotyping of PH in SSc-PH patients is very important as it is imperative to recognize its impact on clinical course, treatment and survival.• ILD was common in Chinese SSc-PH patients. • SSc-PH patients with ILD had lower PaO

OBJECTIVE This study intended to explore the relation between heart rate recovery at 1 minutes (HRR1) during the recovery phase of cardiopulmonary exercise test (CPET) and exercise capacity in female systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-PAH) patients. METHODS Twenty-one female SLE-PAH patients underwent right heart catheterization (RHC), pulmonary function test (PFT) and CPET. Forty-two healthy subjects matched with SLE-PAH patients in age, sex and BMI were recruited as a control group. The correlations between HRR1 with clinical and CPET parameters were performed. RESULTS Peak HR, ΔHR, HRR1, Peak HR-warm HR1min , Peak HR-warm HR2min and CR were significantly lower in SLE-PAH than in controls (P

BACKGROUND: The aim of the present study was to investigate the role of key cardiopulmonary exercise testing (CPET) parameters in the identification of pre-capillary components in patients with pulmonary hypertension associated with left heart disease (PH-LHD), and to evaluate their correlations with hemodynamic parameters. METHODS: Ninety patients with PH-LHD underwent right-heart catheterization, echocardiography, and CPET. The differences in related indexes between a combined post- and pre-capillary PH (Cpc-PH) group (n=47) and an isolated post-capillary PH (Ipc-PH) group (n=43) were compared. Correlation analysis was performed. Logistic regression and receiver operator characteristic (ROC) analyses were performed to assess the ability of CPET variables to distinguish patients with Cpc-PH from those with Ipc-PH. RESULTS: The hemodynamics, hyperventilation and right ventricular function of Cpc-pH group were worse than those of Ipc-pH group. The parameters related to minute ventilation versus carbon dioxide production (VE/VCO(2)) played a significant role in the differentiation of Cpc-PH and Ipc-PH, and had a moderate positive correlation with pulmonary vascular resistance (PVR). Univariate and multivariate logistic analyses showed that lowest percentage of VE/VCO(2) in predicted value (VE/VCO(2)%pred) was the single best predictor of Cpc-PH, and the area under ROC curve also confirmed that lowest VE/VCO(2)%pred (≥137%) could serve as a novel diagnostic marker for Cpc-PH. On the basis of this lowest VE/VCO(2)%pred threshold, patients were divided into two groups. Most hemodynamic parameters were worse in patients with a lowest VE/VCO(2)%pred ≥137%. CONCLUSIONS: VE/VCO(2)-related parameters are powerful prognosticators for the presence of pre-capillary components in patients with PH-LHD, especially lowest VE/VCO(2)%pred.

BACKGROUND: Acute vasoreactivity testing (AVT) during right heart catheterization (RHC) is performed in certain subsets of patients with pulmonary arterial hypertension (PAH) in order to identify those who benefit from calcium channel blockers. The present study aimed to investigate the prognostic value of pre-AVT, post-AVT, and changes in AVT (ΔAVT) parameters for idiopathic PAH (IPAH) patients with a negative acute response, and to identify sex differences that could be of prognostic value. METHODS: A total of 487 incident IPAH patients (171 males and 316 females) with a negative acute response to AVT were recruited from Shanghai Pulmonary Hospital between 2009 and 2018. Outcomes were predicted using the Kaplan-Meier curve and univariate/multivariate Cox regression analyses. All patients were followed up till January 2020, with outcome specified as all-cause mortality. RESULTS: Inhalation of iloprost aerosol improved the hemodynamic parameters for all patients. Post-PVR was decreased, and post-cardiac output (post-CO) and post-cardiac index (post-CI) were increased compared with pre-AVT parameters among males. Increased post- artery oxygen saturation (post-SaO(2)) and decreased post-mean right atrium pressure (post-mRAP) were also observed in females. For all patients, pre-CO ≥3.25 L/min, post-mPAP

Background: Superoxide dismutases (SODs) are an important family of antioxidant enzymes that modulate reactive oxygen species levels. It is largely unknown which SOD isoform(s) change in vivo in idiopathic pulmonary arterial hypertension (IPAH) patients. Methods: A total of 133 consecutive adult IPAH patients who underwent bone morphogenetic protein receptor type 2 (BMPR2) genetic counseling were enrolled in this prospective study. The plasma activities of three subtypes of SOD [copper–zinc (Cu/Zn-SOD), manganese (Mn-SOD), and extracellular SOD (Ec-SOD)] were examined. Results: The activities of SODs were significantly lower in IPAH patients than in healthy subjects. However, only Ec-SOD activity in BMPR2 mutation patients was significantly decreased compared to those in patients without a mutation. The reduced Ec-SOD activity was markedly associated with mean pulmonary arterial pressure, pulmonary vascular resistance (PVR), and 6-min walking distance (6MWD). The reduction of Mn-SOD activity was only associated with 6MWD. There was no association between Cu/Zn-SOD and hemodynamics. Patients with a lower Ec-SOD level had a worse survival compared to those with a higher baseline. The reduced Ec-SOD activity and the raised PVR increased the mortality risk. Conclusions: Ec-SOD was correlated with BMPR2 mutation, hemodynamic dysfunction, and poor outcomes. Circulating Ec-SOD could be a potentially vital antioxidant enzyme in the pathogenesis of IPAH.

Background: The elevated gamma-glutamyltransferase (GGT) activity is regarded as an indicator of cardiovascular disease, with males having higher values than females. The greater incidence of idiopathic pulmonary arterial hypertension (IPAH) is observed in women, whereas prognosis is poor in men. The present study aims to investigate the potential association of GGT on male patients.Methods: Serum GGT levels were measured in 338 consecutive adult IPAH patients, who underwent bone morphogenetic protein receptor type 2 (BMPR2) genetic counseling, and matched with healthy subjects by sex and age. The followed interval was 48 ± 34 months.Results: Increased serum GGT levels were more common in patients with IPAH than controls (p < 0.001). GGT values were significantly higher in male patients than those of females (p < 0.001). Compared with female patients with BMPR2 mutation, GGT level in male patients with BMPR2 mutation was further increased (p = 0.002). Higher GGT levels were associated with worse hemodynamics and Nterminal pro B-type natriuretic peptide in all patients. However, males with a GGT concentration ≥ 53 U/L had a worse survival than those of females. Contrarily, if GGT concentration

BACKGROUND: Although soluble suppression of tumorigenicity-2 (sST2) has been identified as a clinical biomarker for pulmonary hypertension (PH) by previous studies, the implication of sST2 combined with hemodynamic parameters in PH has not been well studied. This study aimed to evaluate the relationship between sST2 and hemodynamic parameters and to evaluate the predictive value of sST2 for mortality in patients with PH. METHODS: One hundred eighty-four incident patients with PH and 14 healthy controls were retrospectively enrolled by Shanghai Pulmonary Hospital for this retrospective study. After all patients underwent right heart catheterization, blood samples were collected and serum sST2 concentration was assessed by the Presage™ ST2 assay. Kaplan-Meier curve and Cox regression analyses were used to predict survival and the association between survival and different factors such as sST2, SvO(2). RESULTS: During a follow-up of 44.9 (IQR 28.5–64.4) months, 65 patients died. The median concentration of sST2 in PH patients was 33.1 ng/mL, which is higher than that in control group (23.1 ng/mL, P=0.005). Furthermore, for PH group, the level of sST2 was higher in non-survivors than that in survivors. Cox regression analyses demonstrated that sST2 and SvO(2) were independent risk factors for survival. In Kaplan-Meier curve analyses, elevated sST2 level and reduced SvO(2) predicted a poor outcome for patients with PH. CONCLUSIONS: Higher sST2 was independently associated with increased mortality, as well as lower SvO(2) in patients with PH. Especially, the combination of higher sST2 and lower SvO(2) had the strongest predictive value of mortality in patients with PH.

Tian-Xiang Chen,1 Bigyan Pudasaini,1 Jian Guo,2 Su-Gang Gong,1 Rong Jiang,1 Lan Wang,1 Qin-Hua Zhao,1 Wen-Hui Wu,1 Ping Yuan,1 Jin-Ming Liu1 1Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, People’s Republic of China; 2Department of Pulmonary Function Test, Shanghai Pulmonary Hospital, Tongji University, Shanghai, People’s Republic of China Background: Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) have been revealed in few studies. Although right heart catheterization (RHC) is the gold standard for clinical diagnosis and assessment of prognosis in pulmonary hypertension (PH), cardiopulmonary exercise testing (CPET) has been a more widely used assessment of functional capacity, disease severity, prognosis, and treatment response in PH. We hypothesized that the “sex-specific” CPET indices could estimate the severity of inoperable CTEPH.Methods: Data were retrieved for 33 male (age, mean ± standard deviation [SD] =62.5±13.4 years) and 40 female (age, mean ± SD =56.3±11.8 years) patients with stable CTEPH who underwent both RHC and CPET at Shanghai Pulmonary Hospital from February 2010 to February 2016. Univariate and forward/backward multiple stepwise regression analysis was performed to assess the predictive value of CPET indices to hemodynamic parameters. Event-free survival was estimated using the Kaplan–Meier method and analyzed with the log-rank test. Cox proportional hazards models were performed to determine the independent event-free survival predictors.Results: Numerous CPET parameters were different between male and female patients with CTEPH and the control group. There were no significant differences in both clinical variables and RHC parameters between male and female patients with CTEPH. O2 pulse, workload, minute ventilation (VE), and end-tidal partial pressure of O2 (PETO2) at anaerobic threshold, as well as peak O2 pulse, workload, VE, and nadir VE/CO2 were significantly higher in male patients than in female patients (P

Background Abnormal lipid metabolism has been reported in patients with idiopathic pulmonary arterial hypertension (IPAH); however, the prognostic value of plasma free fatty acids (FFAs) for these patients is unclear. The present study aimed to determine whether FFA can play a role in predicting the survival of patients with IPAH. Methods A total of 69 blood samples from patients with IPAH were subjected to liquid chromatography-mass spectrometry (LC-MS). According to the classification criteria for pulmonary hypertension in the European Society of Cardiology (ESC) guidelines, patients were divided into low-risk, intermediate-risk, and high-risk groups. The FFA expression levels of patients in the three groups were compared, and the indicators with significant differences were selected. Cox regression analysis was performed to examine the associations between survival and different factors. Receiver operator characteristic (ROC) curves were used to assess the predictive effect of plasma lipids in assessing patients' risk of morbidity, including area under the curve (AUC), sensitivity, specificity and the best cutoff value. Kaplan-Meier curves were used to predict survival. Results A total of 24 FFA molecules were detected in the patients with IPAH. Among them, FFA (20:4), FFA (20:5), FFA (22:5), FFA (22:6), FFA (24:0) and FFA (30:4) showed significant differences between the low-risk and the intermediate-risk or high-risk patients with IPAH. These six FFAs were significantly correlated with hemodynamic parameters. FFA (22:6), named docosahexaenoic acid (DHA), displayed significant negative correlations with World Health Organization functional classification (WHO FC), mean right atrial pressure (mRAP), and pulmonary vascular resistance (PVR), and significant positive correlations with 6-minute walking distance (6MWD) and cardiac index (CI). Cox regression analyses demonstrated that total bilirubin (TBIL) and DHA were independent risk factors for survival of IPAH. Receiver operating characteristic curve analysis revealed that DHA had a cut-off value of 77.55, which had a sensitivity of 96.7% and a specificity of 62.5% for predicting survival. Kaplan-Meier curve analysis showed that a lower level of DHA predicted a poor outcome in patients with IPAH. Conclusions Our study suggested that FFA levels were correlated with disease severity. Lower levels of DHA predict poor survival in patients with IPAH.

Lan Wang,1,* Yuan-Zhe Jin,2,* Qin-Hua Zhao,1 Rong Jiang,1 Wen-hui Wu,1 Su-Gang Gong,1 Jing He,1 Jin-Ming Liu,1 Zhi-Cheng Jing1,3 1Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China; 2Department of Cardiology, The Fourth Affiliated Hospital of China Medical University, Liaoning, China; 3Thrombosis and Vascular Medicine Center, State Key Laboratory of Cardiovascular Disease, FuWai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China *These authors contributed equally to this work Abstract: Studies have shown that vasodilators such as iloprost can be useful for treating pulmonary hypertension (PH). However, in patients with COPD, vasodilators may inhibit hypoxic pulmonary vasoconstriction and impair gas exchange. The efficacy and safety of iloprost inhalation was assessed in 67 patients with PH associated with COPD (COPD-PH), diagnosed by right heart catheterization. Of these, 37 patients had severe PH (mean pulmonary arterial pressure [mPAP] >35 mmHg or mPAP 25–35 mmHg with low cardiac index [

Background: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD). The aim of the present study was to investigate characteristics and prognosis of ILD associated PH in a predominantly Chinese patient cohort. Methods: Consecutive patients with ILD associated PH, confirmed by right heart catheterization at shanghai pulmonary hospital were included in the study. Characteristics at baseline and during follow-up, as well as survival, were analyzed. Results: This observational study was conducted in 26 patients with a mean age of 60.0 ± 12.2 years old. The six-minutes walking distance was 335.0 ± 190.9 meters. Mean pulmonary arterial pressure (PAP) was 42.9 ± 10.9 mm Hg, cardiac output was 4.4 ± 1.0 L/min, and pulmonary vascular resistance (PVR) was 8.6 ± 4.1 Wood Units. Mean PAP was ≥35 mmHg in 69.2% of cases. There were no difference in lung function parameters between ILD with PH and ILD without PH. The overall survival rate was 92% at 1 year, 82% at 3 years, and 62% at 5 years. PVR was the only variable significantly associated with death. Conclusions: Severe hemodynamic impairment along with PH can occur in patients with ILD. These patients have more severely impaired exercise capacity and cardiac function than ILD patients without PH. PVR was the only prognostic factor identified in this group of patients. This article is protected by copyright. All rights reserved.

Pulmonary hypertension (PH), as a complication of bronchiectasis, is associated with increased mortality. However, hemodynamic characteristics and the efficacy of pulmonary arterial hypertension (PAH) therapies in patients with bronchiectasis and PH remain unknown. Patients with bilateral bronchiectasis and concurrent PH were included in the study. Patient characteristics at baseline and during follow-up, as well as survival, were analyzed. This observational study was conducted in 36 patients with a mean age of 51.5 years (range, 17‒74 years). The 6 min walking distance was 300.8 ± 3.3 m. The mean pulmonary arterial pressure (PAP) was 41.5 ± 1.7 mmHg, cardiac output was 5.2 ± 1.4 L/min, and pulmonary vascular resistance was 561.5 ± 281.5 dyn∙s∙cm(-5). The mean PAP was > 35 mmHg in 75% of the cases. Mean PAP was inversely correlated with arterial oxygen saturation values (r =-0.45, P = 0.02). In 24 patients who received oral PAH therapy, systolic PAP was reduced from 82.4 ± 27.0 mmHg to 65.5 ± 20.9 mmHg (P = 0.025) on echocardiography after a median of 6 months of follow-up. The overall probability of survival was 97.1% at 1 year, 83.4% at 3 years, and 64.5% at 5 years. Given the results, we conclude that PH with severe hemodynamic impairment can occur in patients with bilateral bronchiectasis, and PAH therapy might improve hemodynamics in such patients. Prospective clinical trials focusing on this patient population are warranted.

Background Pulmonary hypertension patients with mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg had impaired cardiopulmonary exercise testing (CPET). Borderline mean pulmonary pressures (boPAP; 21-24 mm Hg) represent early pulmonary vasculopathy. The CPET characteristics of boPAP are a matter of discussion. We aimed to determine the CPET profile of such borderline hemodynamics. Methods A matched case-control study was conducted on consecutive boPAP patients at the Shanghai Pulmonary Hospital between Jan 2012 and Jan 2017. Hemodynamics, echocardiography, the pulmonary function test (PFT) and CPET parameters were compared between boPAP patients and normal mPAP patients which were matched 1:1 by sex and age. Conditional logistic regression analysis was performed to determine the efficacy of CPET in detecting boPAP. Results A total of 48 patients underwent RHC and CPET (24 Normal, 24 boPAP). There were no differences in the demographics, echocardiography and PFT. BoPAP patients had significantly decreased VO2 at the anaerobic threshold and peak VO2 /kg (858.4 ± 246.5 mL/min vs 727.9 ± 228.0 mL/min, P = 0.037; 21.1 ± 6.4 mL/min/kg vs. 15.5 ± 5.6 mL/min/kg, P = 0.001, respectively). Significant differences were not observed in ventilation efficiency. A trend of impaired oxygen pulse and submaximal exercise tolerance were observed in boPAP patients. Conditional logistical regression analysis revealed the risk of boPAP increased by 2.493 (95% confident interval: 1.388 to 4.476, P = 0.002) with every 5 mL/min/kg decrease in peak VO2 /kg. Conclusions Patients with boPAP have a greater prevalence of exercise intolerance, a trend of impaired oxygen pulse and submaximal exercise tolerance.

Background and objective: Two endothelin receptor antagonists (ETRAs), bosentan and ambrisentan, are approved for patients with pulmonary arterial hypertension (PAH). However, there is little information about the transition strategy between these two ETRAs. We aimed to evaluate the safety and efficacy from ambrisentan to bosentan. Methods: Twenty PAH patients were enrolled into the single-center, open-labelled prospective study. Echocardiogram, WHO functional class (WHO-FC), 6-minute walking distance (6MWD), right heart catheterization, and hemotology were collected. After receiving oral 5 mg ambrisentan daily initially for one year, the patients were divided into two arms: eight patients switched to bosentan, while the remaining 12 patients continued ambrisentan. Characteristics at baseline, 1-and 2-year follow-up points were compared. Results: There were no significant differences in echocardiogram, WHO-FC, hemodynamics, demographics and liver function at baseline, 1-and 2-year points in both arms. 6MWD in bosentan group was significantly shorter at baseline. But there were no significant differences of 6MWD at 1- and 2-year points. Conclusions: It is safe for stable PAH patients to transition from ambrisentan to bosentan without hemodynamic or hematologic deterioration.

Background and objective The responses of oxygen uptake efficiency (OUE) during cardiopulmonary exercise training (CPET) have not been reported in patients with pulmonary hypertension. We aimed to investigate the differences in OUE between patients with idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods Forty-four patients with IPAH and 29 patients with CTEPH were retrospectively enrolled into our study. All patients underwent right-heart catheterization, pulmonary function test and performed the 6-min walk test and CPET. Results We found that oxygen uptake efficiency plateau (OUEP) and oxygen uptake efficiency at anaerobic threshold (OUE@AT) was significantly higher in IPAH than that in CTEPH (both P = 0.002). However, patients with CTEPH had lower mean pulmonary artery pressure, pulmonary vascular resistance and transpulmonary gradient (all P 0.05) in patients with IPAH. In patients with CTEPH, both anaerobic threshold (r = 0.307, P > 0.05) and VE_AT (r = −0.709, P

Background: Many studies have highlighted sex preponderance in idiopathic pulmonary arterial hypertension (IPAH). It is well established that there are differences in exercise capacities in the two sexes but how much of that difference reflects on disease severity or correlates to markers of severity in the two sexes is still not clear. Right heart catheterization (RHC) and cardiopulmonary exercise testing (CPET) have been widely used for assessing functional capacity, prognosis and treatment response in IPAH. We aimed to investigate the ‘sex-specific’ CPET parameters in relation to hemodynamics in IPAH. Methods: Data were retrieved from 30 males and 53 females [mean ± standard deviation (SD) age: 39.6 ± 17.2 and 37.5 ± 12.0] stable IPAH patients who underwent both RHC and CPET at Shanghai Pulmonary Hospital from 2010 to 2016. Univariate and forward/backward multiple stepwise regression analysis was performed to assess the prognostic value of CPET and hemodynamic parameters. Results: There were no significant differences in clinical variables between men and women. Peak workload, peak oxygen uptake, anaerobic threshold (AT), peak minute ventilation, carbon dioxide output, O2 pulse and oxygen uptake efficiency slope were significantly higher in men compared with women ( p < 0.05). Several CPET indexes correlated with hemodynamics. Pulmonary vascular resistance (PVR) and cardiac output (CO) were distinctly different between the sexes. Peak end-tidal partial pressure of CO2 (PETCO2) was an independent predictor of PVR elevation in all patients and in men. Peak maximum oxygen consumption (VO2) was independently predictive of CO decline in all patients and in men. Only peak O2 pulse was an independent predictor of increased PVR and decreased CO in women. Conclusions: Even after adjusting for age, body mass index and World Health Organization functional class, different CPET parameters correlated with PVR elevation and CO decline in men and women differently, which could potentially better predict severity in men and women with IPAH.

BACKGROUND: Doppler echocardiography is usually the first diagnostic investigation for patients suspected with pulmonary hypertension (PH), but it is often inaccurate when used alone, especially in mild PH. HYPOTHESIS: Cardiopulmonary exercise testing (CPET) may serve as a complementary tool to improve diagnostic accuracy in echocardiography‐suspected “PH possible” patients. METHODS: Eighty‐eight consecutive patients with suspected PH (referred to as “PH possible” hereafter) based on echocardiography were included in the study. CPET was assessed subsequently and PH was confirmed by right‐heart catheterization in all subjects. We analyzed CPET data from patients and derived a CPET prediction rule to hemodynamically differentiate PH. RESULTS: Eighty‐eight patients (27 patients with confirmed PH, and PH ruled out in 61 patients) were included in the study. Compared with non‐PH patients, the PH subjects had lower peak oxygen uptake (VO(2)), aerobic capacity (AT), peak partial pressure of end‐tidal CO(2) (P(ET)CO(2)), oxygen uptake efficiency plateau (OUEP), and oxygen uptake efficiency slope (OUES), along with higher minute ventilation (VE)/carbon dioxide output (VCO(2)) slope and lowest VE/VCO(2) (P < 0.001). VE/VCO(2) slope and AT were independent predictors of PH derived from multivariate logistic regression adjusted for age and body mass index. A score combining VE/VCO(2) slope and AT reached a high area under the curve value of 0.98. A score ≥0.5 had 95% specificity and 92.6% sensitivity for diagnosis of PH. CONCLUSIONS: A score combining VE/VCO(2) slope and AT provides high specificity in screening out PH from a pool of echocardiography‐suspected PH patients.

Objectives To identify the pulmonary hypertension (PH) patients who develop an exercise induced venous-to-systemic shunt (EIS) by performing the cardiopulmonary exercise test (CPET), analyse the changes of CPET measurements during exercise and compare the exercise physiology and resting pulmonary hemodynamics between shunt-PH and no-shunt-PH patients. Methods Retrospectively, resting pulmonary function test (PFT), right heart catheterization (RHC), and CPET for clinical evaluation of 104 PH patients were studied. Results Considering all 104 PH patients by three investigators, 37 were early EIS+, 61 were EIS-, 3 were late EIS+, and 3 others were placed in the discordant group. PeakVO2, AT and OUES were all reduced in the shunt-PH patients compared with the no-shunt-PH subjects, whereas VE/VCO2 slope and the lowest VE/VCO2 increased. Besides, the changes and the response characteristics of the key CPET parameters at the beginning of exercise in the shunt group were notably different from those of the no shunt one. At cardiac catheterization, the shunt patients had significantly increased mean pulmonary artery pressure (mPAP), mean right atrial pressure (mRAP) and pulmonary vascular resistance (PVR), reduced cardiac output (CO) and cardiac index (CI) compared with the no shunt ones (P