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Background: The aim of this study was to investigate the clinical characteristics of patients between active and inactive Takayasu's arteritis with pulmonary artery involvement (PTA) and to identify better markers of disease activity in these patients., Methods: Sixty-four PTA patients in Beijing Chao-yang hospital (2011 to 2021) were included. According to National Institutes of Health criteria, 29 patients were in active stage and 35 were in inactive stage. Their medical records were collected and analyzed., Results: Compared with inactive group, patients in active group were younger. More patients in active stage presented fever (41.38% vs 5.71%), chest pain (55.17% vs 20%), increased C-reactive protein (2.91 vs 0.46 mg/L), erythrocyte sedimentation rate (35.0 vs 9 mm/h), and platelet count (291 vs 221 × 10 9 /L). Pulmonary artery wall thickening was more common in active group (51.72% vs 11.43%). These parameters were restored after treatment. The incidence of pulmonary hypertension was comparable between groups (34.48% vs 51.43%), but patients in active group had lower pulmonary vascular resistance (PVR) (361.0 vs 891.0 dyn·s·cm -5 ) and higher cardiac index (2.76 ± 0.72 vs 2.01 ± 0.58 L/min/m 2 ). On multivariate logistic regression analysis, chest pain [odds ratio (OR) 9.37, 95%CI (1.98-44.38), P = 0.005], increased platelet count (>242.5 × 10 9 /L) [OR 9.03, 95%CI (2.10-38.87), P = 0.003] and pulmonary artery wall thickening [OR 7.08, 95%CI (1.44-34.89), P = 0.016] were independently associated with disease activity., Conclusion: Chest pain, increased platelet count, and pulmonary artery wall thickening are potential new indicators of disease activity in PTA. Patients in active stage may have lower PVR and better right heart function., Competing Interests: Declaration of Competing Interest The authors have declared that no competing interest exists., (Copyright © 2023. Published by Elsevier B.V.)

Background: Pulmonary endarterectomy (PEA) is the preferred treatment for CTEPH patients which can significantly improve symptoms and pulmonary hemodynamics. Therefore, this retrospective study evaluated the long-term outcomes after pulmonary endarterectomy (PEA) and analyze the predictors of long-term outcomes for chronic thromboembolic pulmonary hypertension (CTEPH)., Methods: From 2002-2020, 76 CTEPH patients successfully discharged after PEA in Beijing Chaoyang Hospital were followed-up by scheduled clinical visits or telephone interviews. The follow-up time lasted for 18 years and median time was 7.29 years., Results: The survival rate at 1,3,5,10,15 years postoperatively was 100.00%, 97.10%, 95.40%, 89.80% and 82.90%, respectively. Multivariate logistics regression analysis showed that postoperative mPAP (hazard ratio: 1.144; 95%confidence interval: 1.018-1.285; P  = 0.023) was associated with a higher risk of late death, right atrium right and left diameters (hazard ratio: 1.113; 95%confidence interval, 1.006-1.231; P  = 0.038) were associated with a higher risk of major adverse events., Conclusion: Pulmonary endarterectomy is an effective way to treat CTEPH. Long-term outcome is excellent for patients who undergoing pulmonary endarterectomy who survived from peri-operation time. Postoperative mPAP is a significant prognostic factor for long-term death and right atrium right and left diameters is a significant prognostic factor for major adverse events. That shows patients with high postoperative mPAP and right atrium right and left diameter should be followed up closely.

Objective: To analyze the clinical characteristics and the effect of targeted drug therapy of portopulmonary hypertension (PoPH). Methods: A total of 5 patients with PoPH who were admitted to the Department of Pulmonary and Critical Care Medicine of Beijing Chao-Yang Hospital from January 1, 2017 to December 31, 2018 were included. The clinical information and follow-up data were collected. The patient's medical history, clinical manifestations, right cardiac catheterization (RHC), classification of cardiac and hepatic function, treatment and prognosis were analyzed. Results: Among the 5 patients with PoPH, 3 were male and 2 were female. The median age was 56 years. The underlying diseases of portal hypertension were all cirrhosis, and 1 patient combined with hepatopulmonary syndrome (HPS). Dyspnea was the main respiratory symptom in all the 5 patients, and the median time from symptom onset to diagnosis was 1 year (5 months to 8 years). RHC was used as the diagnostic criteria for pulmonary hypertension in all patients, with a median mean pulmonary arterial pressure of 42 mmHg (1 mmHg=0.133 kPa) and a median pulmonary vascular resistance of 538 dyn·s·cm(-5). 3 cases were in Child-Pugh liver function grade B, and 2 were in grade A. The hepatic reserve function was not matched with the severity of cardiac insufficiency. Liver transplantation was performed in 1 patient, whose right ventricular dysfunction can be alleviated by targeted drug therapy after operation. All the 5 patients received targeted drug therapy of pulmonary hypertension. In the 3 patients who were regularly treated with targeted drugs and followed up on time, the cardiac function was improved during the follow-up period. There was no improvement or even deterioration of cardiac function in 2 patients who were not regularly treated or followed up. One patient died after liver transplantation. The cause of death was severe pneumonia and right ventricular dysfunction. The survival time after transplantation was 1 year. Conclusions: In PoPH patients, the hepatic reserve function is not matched with the heart function classification. PoPH can coexist with HPS. Regular application of pulmonary hypertension targeting drugs may benefit patients with PoPH.

Objective: To investigate the diagnostic value of N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin Ⅰ (TnI) for detecting right ventricular dysfunction (RVD) in patients with non-high-risk acute pulmonary thromboembolism (APE). Methods: A retrospective analysis was performed in 96 adult patients [44 males, 52 females, aged (61±14) years] with non-high-risk APE from January 2015 to June 2016. All patients were divided into RVD group and non-RVD group according to whether there was right ventricular enlargement on echocardiography. The baseline data, serumTnI and NT-proBNP levels were compared between the 2 groups and the diagnostic value of the 2 cardiac markers for RVD was analyzed. Results: There were no significant differences in age, gender, body mass index between the 2 groups ( P> 0.05). The creatinine clearance rate of the RVD group was lower than that of the non-RVD group [96.4 (77.5,99.6) vs 101.7 (95.1,106.5), P= 0.021]. NT-proBNP [2 300 (1 056,3 396) vs 188 (61,535), P< 0.01] and TnI [0.13 (0.09,0.25) vs 0.00 (0.00,0.02), P< 0.01] were significant higher in the RVD group than in the non-RVD group. The univariate logistic regression analyses showed that NT-proBNP (per 100 ng/L, OR 1.199, 95% CI 1.117-1.287), TnI (per 0.01 μg/L, OR 1.164, 95% CI 1.079-1.256) and creatinine clearance rate ( OR 0.968, 95% CI 0.938-0.998) were significantly associated with RVD. Multivariate regression analysis showed that NT-proBNP (per 100 ng/L, OR 1.155, 95% CI 1.074-1.241) and TnI (per 0.01 μg/L, OR 1.079, 95% CI 1.011-1.151) were independently associated with RVD. The areas under the ROC curve (AUC) of NT-proBNP, TnI, and the combination of them were 0.908 (95% CI 0.841-0.976), 0.896 (95% CI 0.826-0.966) and 0.925 (95% CI 0.862-0.988), respectively. The cut-off value of NT-proBNP was 503.5 ng/L, with a sensitivity of 85.7%, specificity of 75.4%, positive predictive value (PPV) of 66.7% and negative predictive value (NPV) of 90.2%.The cut-off value of TnI was 0.05 μg/L, and the sensitivity, specificity, PPV and NPV was 80.0%, 86.9%, 77.8% and 88.3%, respectively. The optimal probability derived from the logistic regression model in which the 2 biomarkers were the independent variables was 0.779, with a sensitivity, specificity, PPV and NPV of 65.7%, 96.7%, 92.0%, 83.1%, respectively. Conclusion: Both NT-proBNP and TnI had preferably good diagnostic value for RVD in patients with non-high-risk APE, but their clinical application needed comprehensive evaluation combined with the overall manifestations of the patients and experimental methods. The diagnostic value was higher when the 2 biomarkers were evaluated together.

Objective: To analyze the clinical features of 3 cases of Takayasu arteritis(TA) with pulmonary cavities on chest computed tomography(CT). Methods: The clinical data of 3 TA patients with cavities on the chest CT who were admitted into Beijing Chaoyang Hospital were retrospectively analyzed. A literature search was performed with "Takayasu arteritis" and "pulmonary" as the key words in China Knowledge Resource Intergrated Database (CNKI) and Pubmed Database for publications from Jan 1, 2000 to Dec. 31,2017. The relevant literatures were reviewed. Results: Among the 3 patients, 2 were males and 1 was female, aging 49, 28 and 28 years, respectively. They presented with cough, fever and chest pain, and chest CT showed cavities, single or multiple, either with thick or thin wall, or wedge-shaped consolidation, residual stripes after being absorbed, and one case had pulmonary biopsy results which showed hemorrhagic infarction. They were all misdiagnosed before as pneumonia, pulmonary tuberculosis, pulmonary thromboembolism. After being treated by combination therapy of glucocorticoids and immunosuppressive agents, the disease improved significantly. A total of 777 cases with TA involving pulmonary arteries were reported, from which 13 cases with involvement of pulmonary parenchyma were described. Therefore total 16 cases including the 3 cases in this article were included for analysis. Twelve cases showed patchy or wedge-shaped ground-glass opacity and consolidation, and peripheral lung stripes remained after being absorbed. Two cases showed pleural effusion, and 4 cases showed cavities, 3 cases were misdiagnosed as pulmonary tuberculosis, 7 as pulmonary infection, and 5 as pulmonary thromboembolism. Conclusions: TA with pulmonary arteries involved is susceptible to be misdiagnosed and missed, and therefore, in patients with cough, hemoptysis, chest pain and cavities in pulmonary parenchyma, TA should be suspected. Early diagnosis and appropriate treatment can lead to a better prognosis.

Background: Pulmonary hypertension (PH) can be caused by a fistula between the systemic and pulmonary arteries. Here, we report a case of PH due to multiple fistulas between systemic arteries and the right pulmonary artery where the ventilation/perfusion scan showed no perfusion in the right lung., Methods: A 32-year-old male patient was hospitalised for community-acquired pneumonia. After treatment with antibiotics, the pneumonia was alleviated but dyspnoea persisted. Pulmonary hypertension was diagnosed using right heart catheterisation, which detected the mean pulmonary artery pressure as 37mmHg. The anomalies were confirmed by contrast-enhanced CT scan (CT pulmonary angiography), systemic arterial angiography and pulmonary angiography., Results: Following embolisation of the largest fistula, the haemodynamics and oxygen dynamics did not improve, and even worsened to some extent. After supportive therapy including diuretics and oxygen, the patient's dyspnoea, WHO function class and right heart function by transthoracic echocardiography all improved during follow-up., Conclusions: Pulmonary hypertension can be present even when the right lung perfusion is lost. Closure of fistulas by embolisation, when those fistulas act as the proliferating vessels, may be harmful., (Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: To enhance the understanding of Takayasu's arteritis with pulmonary vascular involvement through the analyses on clinical features, imaging findings, misdiagnoses and treatments., Methods: A retrospective study was conducted to analyze the clinical records of patients diagnosed as Takayasu's arteritis with pulmonary vascular involvement admitted to Beijing Chaoyang Hospital from January 2004 to December 2014., Results: In recent 10 years, there were 90 patients diagnosed as Takayasu's arteritis in Beijing Chaoyang Hospital, 33 of them were involved with pulmonary arterial, which account for 36.7%, while 12 cases were involved with pulmonary arteries alone, which account for 13.3% of all. Among the 33 patients, dyspnea (23 cases, 69.7%) was the most common symptom, followed by hemoptysis (19 cases, 57.6%) and Vascular murmur (23 cases, 69.7%), pleural effusion (8 cases, 24.2%), unequal blood pressure of upper limbs (4 cases, 12.1%) were the main signs. The diagnostic time varied significantly, from 1 month to 10 years. 21 patients were misdiagnosed and the misdiagnosis rate was 63.6%., Conclusion: Takayasu's arteritis with pulmonary vascular involvement is not rare. The clinical manifestation of Takayasu's arteritis is unspecific and misdiagnosis rate is relatively high. Therefore, we should raise awareness of Takayasu's arteritis and detailed clinical examination will be helpful to reduce the misdiagnosis rate.

Background: Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is a severe clinical syndrome characterized by right cardiac failure and possibly subsequent liver dysfunction. However, whether serum markers of liver dysfunction can predict prognosis in inoperable CTEPH patients has not been determined. Our study aimed to evaluate the potential role of liver function markers (such as serum levels of transaminase, bilirubin, and gamma-glutamyl transpeptidase [GGT]) combined with 6-min walk test in the prediction of prognosis in patients with inoperable CTEPH., Methods: From June 2005 to May 2013, 77 consecutive patients with inoperable CTEPH without confounding co-morbidities were recruited for this prospective cohort study. Baseline clinical characteristics and 6-min walk distance (6MWD) results were collected. Serum biomarkers of liver function, including levels of aspartate aminotransferase, alanine aminotransferase, GGT, uric acid, and serum bilirubin, were also determined at enrollment. All-cause mortality was recorded during the follow-up period., Results: During the follow-up, 22 patients (29%) died. Cox regression analyses demonstrated that increased serum concentration of total bilirubin (hazard ratio [HR] = 7.755, P < 0.001), elevated N-terminal of the prohormone brain natriuretic peptide (HR = 1.001, P = 0.001), decreased 6MWD (HR = 0.990, P < 0.001), increased central venous pressure (HR = 1.074, P = 0.040), and higher pulmonary vascular resistance (HR = 1.001, P = 0.018) were associated with an increased risk of mortality. Serum concentrations of total bilirubin (HR = 4.755, P = 0.007) and 6MWD (HR = 0.994, P = 0.017) were independent prognostic predictors for CTEPH patients. Patients with hyperbilirubinemia (≥23.7 μmol/L) had markedly worse survival than those with normobilirubinemia., Conclusion: Elevated serum bilirubin and decreased 6MWD are potential predictors for poor prognosis in inoperable CTEPH.

Background: Acute exacerbation of chronic obstructive pulmonary disease (COPD) is always associated with a high incidence and mortality. Because of the presence of some concomitant risk factors such as immobilization, bronchial superinfection, patients who are admitted for acute exacerbations of COPD are generally considered to be at moderate risk for the development of venous thromboembolism. In this study, we investigated the prevalence and the clinical manifestations of deep venous thrombosis (DVT) in patients with acute exacerbation of COPD., Methods: From March 2007 to March 2009, 520 consecutive patients were included in this study. On admission, color Doppler ultrasound of lower extremities in all cases was performed for diagnosing DVT. Patients with DVT were compared with those without DVT from such aspects as demographics, symptoms, physical signs and risk factors., Results: Among the 520 patients, DVT was found in 46 cases (9.7%). In patients with DVT, the duration of hospitalization was longer (P = 0.01), and the mechanical ventilation requirement increased (P < 0.001). Other indicators for patients with more possibility of DVT were immobility exceeding 3 days (P < 0.001); pneumonia as concomitance (P = 0.01); respiratory failure type II (P = 0.013); current smoking (P = 0.001). Lower extremity pain was more common in DVT cases in comparison to those without DVT (34.8% vs. 15.2%, P = 0.01)., Conclusions: The acute exacerbation of COPD patients, who were immobilized for over 3 days, complicated by pneumonia and had respiratory failure type II, had a higher risk of DVT. In addition, DVT detection awareness should be increased in cases that had a lower extremity pain.

Objective: To explore the association between the erythrocyte sedimentation rate, serum C-reactive protein (CRP) and the risk of lung cancer., Methods: One hundred and three patients with newly diagnosed lung cancer and 85 homochronous hospitalized patients with chronic respiratory diseases (including chronic obstructive pulmonary disease, asthma, bronchiectasis and pulmonary fibrosis) were included in this study. ESR, serum levels of CRP, CEA, CA19-9 and CA125 were analyzed in the two groups before the initiation of any therapy after hospitalization. The association with clinicopathological characteristics of lung cancer and the risk of lung cancer were estimated by logistic regression., Results: Both the ESR and CRP levels were significantly higher in the lung cancer group, as compared with that in the chronic respiratory diseases group (P < 0.001). There was no significant association of ESR and CRP with lung cancer stage and type. Spearman correlation analysis showed a positive correlation between ESR and CRP (r = 0.56, P < 0.001), ESR and CA125 (r = 0.33, P < 0.001), and CRP and CA125 (r = 0.32, P < 0.001). The results of multivariate logistic analysis showed that the level of CRP was associated with an increased risk of lung cancer. Adjusting the confounding factors such as age, gender and smoking condition, the risk increased along with the elevation of CRP. Compared with the first quantile patients, the risk of the second quantile patients increased twice (OR: 2.46, 95%CI: 1.16 - 5.20), the risk of the third quantile patients increased ten-fold (OR: 10.52, 95%CI: 4.40 - 25.18)., Conclusion: The level of CRP is associated with an increased risk of lung cancer. The results of this study suggest that early detection of CRP may have a potential predicting value for high risk group of lung cancer.

Objective: To evaluate the short-term efficacy and safety of sildenafil in treatment of pulmonary artery hypertension (PAH)., Methods: Literatures in regard to sildenafil treatment of PAH were searched in PubMed (from 01/01/1968 to 01/05/2006), Embase (from 1980 to 2006) and China National Knowledge Infrastructure (CNKI) (from 1994 to 2006). Randomized-controlled trials (RCTs) of sildenafil versus placebo in the treatment of PAH were conducted. The quality of the included trials was evaluated by two reviewers independently. Meta-analysis was done by using the Cochrane Collaboration's RevMan 4.2.8., Results: Ten literatures were retrieved. Four RCTs, including 328 patients, were included and were graded in terms of the quality of randomization, allocation, concealment and blinding. One study was graded as level A and the other three were graded as level B. The meta-analysis showed that compared with placebo treatment, sildenafil therapy can (1) improve the exercise capacity of the PAH patients measured as distance covered in a 6 minute walk test with an increase of 55.76 meters on average in 6 minutes' walk (95% confidence interval, 41.26 to 70.25; P < 0.01), (2) increase the exercise time of the PAH patients by 221.13s on average (95% confidence interval, 146.13 to 296.14; P < 0.01), (3) decrease the pulmonary artery systolic pressure by 11.51 mmHg on average (95% confidence interval, 1.41 to 21.60, P = 0.03), and (4) alleviate the exacerbation of clinical conditions with a total odds ratio (OR) of 0.36 (95% confidence interval, 0.16 to 0.78, P = 0.01); however, it failed to alleviate the headache and hypotension in comparison with the placebo group. No sexual disturbance was seen in the patients treated with sildenafil., Conclusion: Sildenafil therapy improves the clinical symptoms and exercise capacity, and decreases the pulmonary artery systolic pressure of the PAH patients; and causes no serious side effects.