Objectives:To investigate the clinical and cardiopulmonary functional characteristics of cardiopulmonary-phenotype idiopathic pulmonary arterial hypertension(IPAH)patients in comparison with classical IPAH patients and pulmonary hypertension patients associated with chronic lung disease(CLD-PH).Methods:In this retrospective study,data were collected from 30 patients with classical IPAH,20 cardiopulmonary phenotype IPAH patients,and 20 patients with CLD-PH,who were hospitalized in the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital from November 2017 to February 2025.Pulmonary hypertension was diagnosed via right heart catheterization and all patients underwent pulmonary function tests,chest computed tomography(CT),echocardiography,and patients were followed up to 5 years.Results:Compared to classical IPAH patients,both cardiopulmonary phenotype IPAH patients and CLD-PH patients exhibited later onset age,higher proportions of World Health Organization(WHO)functional class Ⅲ-Ⅳ,males,and smokers(all P<0.05).The cardiopulmonary-phenotype IPAH patients also had higher rates of coronary artery disease and diabetes compared to classical IPAH patients(all P<0.05).Physiologically,the cardiopulmonary-phenotype IPAH patients showed reduced diffusing capacity for carbon monoxide(DLCO)and partial pressure of oxygen(PaO2),along with higher rates of emphysema and pulmonary fibrosis compared to classical IPAH(all P<0.05).In contrast,CLD-PH patients had lower mean pulmonary arterial pressure(mPAP),pulmonary vascular resistance(PVR),and pulmonary artery wedge pressure(PAWP),as well as reduced forced expiratory volume in the first second(FEV1%predicted),FEV1/forced vital capacity(FVC)ratio,and DLCO.However,CLD-PH patients demonstrated higher tricuspid annular plane systolic excursion(TAPSE),peak systolic velocity of the tricuspid annulus(S`),and partial pressure of carbon dioxide(PaCO2),along with increased rates of emphysema and pulmonary fibrosis(all P<0.05).Compared with LCD-PH,cardiopulmonary-phenotype IPAH patients had higher mPAP,PVR,FEV1%predicted,FEV1/FVC ratio,PAWP,and systolic pulmonary artery pressure,but lower DLCO,cardiac output,TAPSE,S′,and PaCO2(all P<0.05).No statistical difference was observed in PaO2 between these two groups.All cardiopulmonary-phenotype IPAH patients and classical IPAH patients received targeted medications,55%of CLD-PH patients did not receive targeted therapy,45%received monotherapy only(compared with cardiopulmonary-phenotype IPAH patients and classical IPAH groups,P<0.05).The 1,2,and 5-year survival rates were 79.2%,62.2%,and 46.7%,respectively in patients with cardiopulmonary-phenotype IPAH,100%,94.4%,and 94.4%,respectively in classic IPAH patients,and 92.9%,77.4%and 77.4%,respectively in patients with CLD-PH.Survival rates for cardiopulmonary-phenotype IPAH patients were significantly lower than those of classical IPAH and CLD-PH patients(log-rank P=0.008).Conclusions:IPAH cardiopulmonary phenotype patients are older,predominantly male,and often have a smoking history(median 30 pack-years).They exhibit severe hypoxemia,markedly reduced DLCOc,preserved spirometry,and severe pulmonary hypertension and lower survival rate.

Objectives:To investigate the clinical and cardiopulmonary functional characteristics of cardiopulmonary-phenotype idiopathic pulmonary arterial hypertension(IPAH)patients in comparison with classical IPAH patients and pulmonary hypertension patients associated with chronic lung disease(CLD-PH).Methods:In this retrospective study,data were collected from 30 patients with classical IPAH,20 cardiopulmonary phenotype IPAH patients,and 20 patients with CLD-PH,who were hospitalized in the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital from November 2017 to February 2025.Pulmonary hypertension was diagnosed via right heart catheterization and all patients underwent pulmonary function tests,chest computed tomography(CT),echocardiography,and patients were followed up to 5 years.Results:Compared to classical IPAH patients,both cardiopulmonary phenotype IPAH patients and CLD-PH patients exhibited later onset age,higher proportions of World Health Organization(WHO)functional class Ⅲ-Ⅳ,males,and smokers(all P<0.05).The cardiopulmonary-phenotype IPAH patients also had higher rates of coronary artery disease and diabetes compared to classical IPAH patients(all P<0.05).Physiologically,the cardiopulmonary-phenotype IPAH patients showed reduced diffusing capacity for carbon monoxide(DLCO)and partial pressure of oxygen(PaO2),along with higher rates of emphysema and pulmonary fibrosis compared to classical IPAH(all P<0.05).In contrast,CLD-PH patients had lower mean pulmonary arterial pressure(mPAP),pulmonary vascular resistance(PVR),and pulmonary artery wedge pressure(PAWP),as well as reduced forced expiratory volume in the first second(FEV1%predicted),FEV1/forced vital capacity(FVC)ratio,and DLCO.However,CLD-PH patients demonstrated higher tricuspid annular plane systolic excursion(TAPSE),peak systolic velocity of the tricuspid annulus(S`),and partial pressure of carbon dioxide(PaCO2),along with increased rates of emphysema and pulmonary fibrosis(all P<0.05).Compared with LCD-PH,cardiopulmonary-phenotype IPAH patients had higher mPAP,PVR,FEV1%predicted,FEV1/FVC ratio,PAWP,and systolic pulmonary artery pressure,but lower DLCO,cardiac output,TAPSE,S′,and PaCO2(all P<0.05).No statistical difference was observed in PaO2 between these two groups.All cardiopulmonary-phenotype IPAH patients and classical IPAH patients received targeted medications,55%of CLD-PH patients did not receive targeted therapy,45%received monotherapy only(compared with cardiopulmonary-phenotype IPAH patients and classical IPAH groups,P<0.05).The 1,2,and 5-year survival rates were 79.2%,62.2%,and 46.7%,respectively in patients with cardiopulmonary-phenotype IPAH,100%,94.4%,and 94.4%,respectively in classic IPAH patients,and 92.9%,77.4%and 77.4%,respectively in patients with CLD-PH.Survival rates for cardiopulmonary-phenotype IPAH patients were significantly lower than those of classical IPAH and CLD-PH patients(log-rank P=0.008).Conclusions:IPAH cardiopulmonary phenotype patients are older,predominantly male,and often have a smoking history(median 30 pack-years).They exhibit severe hypoxemia,markedly reduced DLCOc,preserved spirometry,and severe pulmonary hypertension and lower survival rate.

Objective:To investigate whether patients with pulmonary hypertension (PH) develop abnormal fibroblast activation protein (FAP) inhibitor (FAPI) uptake in the left ventricular free wall and to analyze its characteristics and significance.Methods:Al 18F-1, 4, 7-triazacyclononane-1, 4, 7-triacetic acid (NOTA)-FAPI-04 PET/CT images of 51 patients diagnosed with PH (24 males, 27 females; age: (48±21) years) and 10 healthy volunteers (4 males, 6 females; age: (59±12) years) from Beijing Chaoyang Hospital of Capital Medical University between February 2021 and January 2024 were retrospectively analyzed. Higher FAPI uptake in the left ventricular free wall than that in blood pool was defined as abnormal and SUV max, SUV mean, and total lesion FAP activity were quantitatively measured. Associated factors with FAPI uptake in the left ventricle were analyzed, and differences of clinical parameters between patients with and without abnormal left ventricular uptake were compared. Independent-sample t test and Mann-Whitney U test were used to compare differences between groups. Spearman rank correlation analysis was used for correlation analysis. Results:Abnormal FAPI uptake that was diffusely distributed in the left ventricular free wall was observed in 19 patients with PH. Total lesion FAP activity was positively correlated with peak mitral late-diastolic inflow velocity ( A) ( rs=0.696, P=0.001) and negatively correlated with peak mitral early-diastolic inflow velocity ( E)/ A and pulmonary vascular resistance (PVR) ( rs values: -0.629, -0.540, P values: 0.004, 0.017). Significant differences in E/ A (0.8(0.6, 1.1) vs 0.9(0.8, 1.4); z=-2.33, P=0.020), left ventricular end-systolic internal diameter ((25.7±2.6) vs (27.8±4.2) mm; t=-2.22, P=0.031), and left ventricular end-systolic volume ((26.7±7.3) vs (32.5±9.9) ml; t=-2.26, P=0.028) were found between patients with and without abnormal FAPI uptake in the left ventricle. Conclusion:In patients with PH, the left ventricular free wall develops diffuse abnormal uptake of FAPI, the extent of which is related to impaired left ventricular diastolic function.

Objective:To explore the relationship between the imaging features of enhanced MRI in patients with central chronic pulmonary artery thromboembolism (CPTE) and pulmonary vascular resistance (PVR).Methods:Thirty-nine patients with CPTE who had contrast-enhanced MRI examination were retrospectively enrolled this study from January 2018 to December 2020. And 33 patients who received right heart catheterization were divided into two groups based on PVR=1 000 dyn·s·cm -5. The differences of imaging features of CPTE in enhanced MRI between the two groups were compared. The relationship between gender, duration of disease, age, pleural thickening, bilateral bronchial artery dilation, number of the involved vascular segments, number of thrombosis, number of the thrombus-related delayed enhancement of artery wall and PVR was analyzed by binary logistic regression. Results:In 39 patients with central CPTE, the dilated lumen (168, 43.30%) and delayed enhancement of wall (122, 31.52%) were found in most of pulmonary arteries. The rate of the lumen dilatation associated with thrombus was the highest among that of the lumen abnormality (66, 52.80%). There were more thrombi in PVR<1 000 dyn·s·cm -5 group than those in PVR≥1 000 dyn·s·cm -5 group (χ 2=9.55, P=0.002). There was no significant difference in the incidence of wall delayed enhancement associated the thrombus between the two groups (χ 2=0.90, P=0.344). The incidence of bilateral bronchial arterial dilatation in PVR<1 000 dyn·s·cm -5 group was higher than that in PVR≥1 000 dyn·s·cm -5 group ( P=0.019). Logistic regression analysis showed that female, the less number of involved vascular segments and bilateral bronchial artery dilation were correlated with the lower PVR. Conclusions:Enhanced MRI is helpful to accurately evaluate the lumen abnormality of pulmonary artery and wall remodeling in central CPTE, which is of great value for the assessment of patients′ conditions and treatment effect.

Objective:To analyze the late gadolinium enhancement (LGE) manifestations, cardiac function, and myocardial strain by feature tracking (FT) in Takayasu arteritis (TA) with pulmonary artery involvement (PTA) using cardiac MR (CMR), and then to investigate manifestations of the impaired myocardial structure and function.Methods:A retrospective study was performed on 32 patients with PTA and 21 healthy subjects without cardiopulmonary diseases from January 2017 to December 2020. All of them underwent CMR examinations. According to the presence of pulmonary arterial hypertension (PAH),PTA patients were divided into two groups including PAH group (11 cases) and non-PAH group (21 cases). LGE manifestations were observed and Fisher exact test was used for statistical analysis between the two groups. Cardiac function parameters and FT values including global peak strain of the left and right ventricle were calculated separately in PAH, non-PAH group of patients and healthy controls, using One-way ANOVA or non-parametric Kruskal-Wallis test for statistical analysis including a pairwise comparison between groups. The correlations between FT values of the PAH group and parameters measured by right heart catheterization test (RHC) and transthoracic echocardiography were analyzed using Pearson or Spearman correlation analysis.Results:There were 23 PTA patients (71.9%) with LGE. LGE in the interventricular insertion points (IPs)(11/11), and in the mid-wall (11/11) or epicardial (10/11) myocardium was more common ( P values were 0.006,<0.001 and 0.011, respectively) in PAH group, compared with LGE in the IPs (11/21), and in the mid-wall (7/21) or epicardial (9/21) myocardium in non-PAH group. The absolute values of left ventricular global peak circumferential strain (LVGPCS), left ventricular global peak longitudinal strain (LVGPLS) and right ventricular global peak longitudinal strain in PAH group were smaller than those in healthy subjects ( P<0.05). The absolute values of LVGPCS and LVGPLS in non-PAH group were smaller than those in healthy subjects ( P<0.05). In PAH group, mean pulmonary artery pressure of RHC was correlated with several FT parameters ( P<0.05), especially left ventricular global peak radial strain ( r=-0.807, P=0.009) and LVGPCS ( r s=0.817, P=0.007). Conclusions:Myocardial injury can be seen in PTA patients. And LGE in the IPs and LGE in the mid-wall or epicardial myocardium is more common in PTA patients with PAH. LVGPCS and LVGPLS can early indicate left heart dysfunction in PTA patients without PAH.

Objective:To evaluate the efficacy of balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH) using 99Tc m-macroaggregated albumin (MAA) pulmonary perfusion tomography imaging. Methods:Twenty-five patients (4 males, 21 females; age (56.5±12.3) years) with CTEPH who underwent BPA from January 2017 to April 2020 in Beijing Chaoyang Hospital, Capital Medical University were enrolled retrospectively. Effect of BPA on the improvement of pulmonary lobe/pulmonary segment perfusion was analyzed, and the proportions of improved and unimproved pulmonary lobe/pulmonary segment perfusion by BPA were calculated. The percentages of perfusion defect scores (PPDs%) of lung perfusion tomography imaging before BPA and after 4-6 times BPA were compared and analyzed (paired t test). The correlations between PPDs% and mean pulmonary artery pressure (mPAP) before BPA and after BPA were analyzed respectively, and the correlation between decreased percentage of PPDs% and decreased percentage of mPAP after BPA were also analyzed (Pearson correlation analysis). Results:Among 150 lobes of 25 patients, 96.00%(144/150) lobes showed perfusion abnormalities before BPA. After BPA, 11.11%(16/144) showed complete improvement, 57.64%(83/144) showed partial improvement, and 31.25%(45/144) showed no improvement. Among 450 pulmonary segments of 25 patients, 62.44%(281/450) showed perfusion abnormalities before BPA. After BPA, 30.60%(86/281), 37.37%(105/281), 32.03%(90/281) showed complete, partial and no improvement, respectively. The post-BPA PPDs% was significantly lower than that of pre-BPA ((39.08±10.88)% vs (57.88±10.46)%; t=10.40, P<0.001). The post-BPA mPAP was significantly lower than that of pre-BPA ((32.36±10.57) vs (49.08±10.23) mmHg; 1 mmHg=0.133 kPa; t=10.25, P<0.001). There was no significant correlation between PPDs% and mPAP either before BPA ( r=0.01, P=0.953) or after BPA ( r=0.27, P=0.199), but there was a positive correlation between the changes of PPDs% and mPAP ( r=0.40, P=0.045). Conclusions:BPA can significantly improve the pulmonary perfusion and reduce mPAP in CTEPH patients. Pulmonary perfusion tomography imaging can be used to evaluate the efficacy of BPA in CTEPH.

Humans ; Behcet Syndrome/diagnostic imaging*

Objective: To evaluate the safety and efficacy of balloon pulmonary angioplasty (BPA) in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Methods: A study was conducted on 25 patients consisting of 10 males and 15 females with CTEPH who received BPA treatment from February 2017 to May 2018 in Beijing Chaoyang Hospital, Capital Medical University. The changes of brain natriuretic peptide (BNP), mean pulmonary artery pressure (mPAP), tricuspid annulus systolic plane excursion (TAPSE), six-minute walking test (6WMD), right ventricular basement diameter and WHO cardiac function grading were compared before and after treatment with BPA using paired t test. Results: Twenty-five patients received a total of 50 BPA treatments, and among them, 12 patients received two or more times. The mPAP decreased from (50.4±9.9) mmHg (1 mmHg=0.133 kPa) to (39.9±10.6) mmHg before and after operation, and the difference was statistically significant (t=9.7, P<0.001); BNP decreased from (513.5±357.3) pg/ml to (106.3±137.53) pg/ml, and the difference was significant (t=3.2, P=0.006); TAPSE increased from (16.2±2.8) mm to (18.0±2.4) mm, and the difference was statistically significant (t=-5.7, P=0.002); the right ventricular base diameter decreased from (45.9±6.9) mm to (41.2±7.3) mm, and the difference was statistically significant (t=5.6, P<0.001); 6WMD increased from (371.1±86.8) m to (467.7±76.1) m, with statistical significance (t=-6.4, P<0.001); WHO cardiac function grading was improved from (2.4±0.7) to (1.2±0.4) after surgery, and the difference was statistically significant (t=2.8, P=0.021). Pulmonary artery injury occurred in 3 patients and 2 patients had hemoptysis.The hemoptysis was stopped after arterial embolization. No other complications such as pulmonary edema occurred. Conclusion BPA may improve clinical status and hemodynamics of patients with CTEPH, which offers an alternative approach of treatment in patients with inoperable CTEPH.

Objective To evaluate the cardiac hemodynamics and function before and after balloon pulmonary angioplasty ( BPA ) treatment in patients with chronic thromboembolic pulmonary hypertension ( CTEPH) using echocardiography ,and to discuss the clinical value of the treatment . Methods A total of 36 CT EPH patients with medium‐high risk stratification underwent BPA during the period of September 2016 to January 2019 in Beijing Chao‐Yang Hospital ,Capital M edical University were recruited . T he following conventional echocardiographic parameters including right ventricular basal diameter ( RVD ) ,left ventricular basal diameter ( LVD) ,right atrial diameter ( RAD) ,left atrial diameter ( LAD) ,main pulmonary artery diameter ( DM PA ) , left ventricular eccentricity index ( LVEI ) , left ventricular ejection fraction ( LVEF) and pulmonary artery systolic pressure ( PASP ) were recorded .Echocardiographic parameters of right ventricular ( RV ) function including tricuspid annular plane systolic excursion ( T APSE ) , right ventricular fractional area change ( RVFAC ) ,tissue Doppler – derived tricuspid lateral annular systolic velocity ( S′) ,RV index of myocardial performance ( RIM P ) and right ventricular free wall longitudinal strain ( GLS) were measured . T he patients were divided into 2 groups according to the threshold value of postoperative SPAP of 50 mmHg (group Ⅰ :SPAP ≤ 50 mmHg ,group Ⅱ :SPAP＞50 mmHg) . Changes between each parameter before and after BPA were analyzed . Results ① T he frequency of BPA treatment for CT EPH patients ranged from 1 to 6 times . After BPA treatment ,SPAP decreased significantly ,and the measurements of RV function including T APSE ,RVFAC ,RIM P and GLS improved significantly ( all P ＜0 .05) . ②Patients in group Ⅰ showed significantly better RV function including T APSE ,RVFAC and GLS compared with group Ⅱ before BPA ( P ＜0 .05) . ③Univariate logistic regression showed that parameters of preoperative RV function T APSE , RVFAC and GLS had certain effects on the curative effect of interventional surgery . Conclusions Echocardiography can evaluate the hemodynamics and RV function in CT EPH patients with BPA .After BPA ,pulmonary artery pressure decreases and RV function improves to some extent ,suggesting that the treatment of BPA has certain clinical application and popularization value .

Objective: To evaluate the cardiac hemodynamics and function before and after balloon pulmonary angioplasty (BPA) treatment in patients with chronic thromboembolic pulmonary hypertension (CTEPH) using echocardiography, and to discuss the clinical value of the treatment. Methods: A total of 36 CTEPH patients with medium-high risk stratification underwent BPA during the period of September 2016 to January 2019 in Beijing Chao-Yang Hospital, Capital Medical University were recruited. The following conventional echocardiographic parameters including right ventricular basal diameter (RVD), left ventricular basal diameter (LVD), right atrial diameter (RAD), left atrial diameter (LAD), main pulmonary artery diameter (DMPA), left ventricular eccentricity index (LVEI), left ventricular ejection fraction (LVEF) and pulmonary artery systolic pressure (PASP) were recorded.Echocardiographic parameters of right ventricular (RV) function including tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RVFAC), tissue Doppler–derived tricuspid lateral annular systolic velocity (S′), RV index of myocardial performance (RIMP) and right ventricular free wall longitudinal strain (GLS) were measured. The patients were divided into 2 groups according to the threshold value of postoperative SPAP of 50 mmHg (group Ⅰ: SPAP ≤ 50 mmHg, group Ⅱ: SPAP>50 mmHg). Changes between each parameter before and after BPA were analyzed. Results: ①The frequency of BPA treatment for CTEPH patients ranged from 1 to 6 times. After BPA treatment, SPAP decreased significantly, and the measurements of RV function including TAPSE, RVFAC, RIMP and GLS improved significantly (all P<0.05). ②Patients in group Ⅰ showed significantly better RV function including TAPSE, RVFAC and GLS compared with group Ⅱ before BPA (P<0.05). ③Univariate logistic regression showed that parameters of preoperative RV function TAPSE, RVFAC and GLS had certain effects on the curative effect of interventional surgery. Conclusions Echocardiography can evaluate the hemodynamics and RV function in CTEPH patients with BPA.After BPA, pulmonary artery pressure decreases and RV function improves to some extent, suggesting that the treatment of BPA has certain clinical application and popularization value.