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Background: Pulmonary arterial hypertension (PAH) is characterized by structural alterations of pulmonary vessels. Few studies have explored the clinical significance of quantitative assessment of pulmonary small vessels by chest computed tomography (CT). Our aim was to assess whether the prognosis of connective tissue diseases (CTD)-PAH patients could be assessed through pulmonary small vessels measured by chest CT., Methods: In all, 42 CTD-PAH patients diagnosed based on right heart catheterization were retrospectively investigated. All patients underwent a chest CT within 1 month before and after right heart catheterization examination. Main pulmonary artery (MPA) and the cross-sectional area of small pulmonary vessels <5 mm2 as a percentage of total lung area (%CSA <5) were measured. The primary endpoint was a composite clinical worsening endpoint., Results: After a median follow-up time of 30.5 (interquartile range, 8.5 to 45.25) months, endpoint events occurred in 16 (38.1%) patients after 19.5 (interquartile range, 10.0 to 45.5) months. Cox univariate proportional hazard analysis showed that pulmonary vascular resistance, MPA diameter, and %CSA <5 were associated with the end point. A combination of MPA diameter and %CSA < 5 was the independent risk factor for the prognosis (hazard ratio, 2.180 [95% confidence interval, 1.405-3.383], P=0.001). Kaplan-Meier analysis showed that CTD-PAH patients satisfying %CSA < 5 of <0.382 and MPA >36.75 mm had the highest risk of experiencing the endpoint., Conclusion: Among the pulmonary vascular indicators measured by chest CT, in addition to MPA, %CSA < 5 may be a potential independent risk factor for poor long-term prognosis in Chinese CTD-PAH patients., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Previous studies have shown that serum uric acid (UA) levels are correlated with the severity of idiopathic pulmonary arterial hypertension (IPAH) and are predictors of disease prognosis. Still, few studies have explored the value of serum UA in pulmonary arterial hypertension secondary to connective tissue disease (CTD-PAH). This retrospective study aimed to investigate the clinical value of serum UA levels in patients with CTD-PAH., Methods: Fifty CTD-PAH patients were enrolled in our study, from which baseline UA levels, respective variations, and additional clinical data were collected. The potential association between baseline UA level and severity of CTD-PAH was investigated. Furthermore, the relationship between baseline UA and survival rate of CTD-PAH patients, as well as between UA variations and survival rate of pulmonary hypertension secondary to connective tissue disease (CTD-PH) patients was discussed., Results: Baseline serum UA levels were positively correlated with pulmonary vascular resistance (PVR). During the follow-up period, 3 CTD-PAH and 12 CTD-PH patients died. Kaplan-Meier survival curves showed lower survival rate in patients with hyperuricemia than in patients with normouricemia, in both groups (CTD-PAH group p = 0.041, CTD-PH group p = 0.013). Concerning serum UA variations, patients with persistent hyperuricemia showed the lowest survival rate when compared with patients with steady normouricemia (p = 0.01) or patients with decresing serum UA levels, i.e. undergoing from a status of hyperuricemia to a status of normouricemia (p = 0.023)., Conclusion: Baseline serum UA levels might predict severity of CTD-PAH. Together with baseline values, changes of uric acid level may predict the clinical prognosis of the disease.

Objective: To explore the long-term prognostic value of a simplified risk assessment strategy based on the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines in Chinese patients with connective tissue disease (CTD) associated with pulmonary arterial hypertension (PAH)., Methods: We identified 50 CTD-PAH patients diagnosed by right heart catheterization. A retrospective chart review was completed to assess their clinical presentation and laboratory test results. A simplified version of the risk stratification model proposed by the 2015 ESC/ERS PH guidelines was applied, which included the WHO functional class, the 6-minute walking distance test, N-terminal pro-B-type natriuretic peptide plasma levels, pericardial effusion, right atrial pressure (RAP), cardiac index (CI), and mixed venous oxygen saturation (SvO 2 ). The risk grades were defined as follows: low risk = at least 3 low-risk variables and no high-risk variables; high risk = at least 2 high-risk variables, including SvO 2 or CI; and intermediate risk = when the above definitions of low or high risk were not fulfilled. The study endpoint was 3-year all-cause mortality., Results: Twenty patients were defined as a low-risk group, while 30 were classified into a combined intermediate-high-risk group at the baseline assessment. All 20 patients in the low-risk group remained in the low-risk group at follow-up, 20 patients in the intermediate-high-risk group were downgraded to the low-risk group, and eight patients remained in the intermediate-high-risk group at the follow-up assessment. Patients in the intermediate-high-risk group exhibited higher 3-year mortality than the low-risk group at baseline (26% vs 14%, P = 0.0384). Compared with patients who remained in the intermediate-high-risk group, patients who were downgraded to the low-risk group showed lower 3-year mortality (P = 0.0281)., Conclusion: A simplified risk stratification model based on the 2015 ESC/ERS PH guidelines helped to identify CTD-PAH patients with poor long-term  prognosis , which was useful in evaluating the severity and treatment response of patients with CTD-PAH.Key Point•This study showed that the simplified version of the 2015 ESC/ERS risk stratification model could help identify Chinese CTD-PAH patients with poor prognosis at diagnosis and after treatment initiation.

Group-based trajectory modeling (GBTM) allows the trajectory analyses of repeated N-terminal pro-brain natriuretic peptide (NT-proBNP) measurements during follow-up visits of pulmonary artery hypertension associated with connective tissue disease (CTD-PAH) patients. This study aimed to (1) identify trajectories of NT-proBNP changing over time, (2) explore the association between NT-proBNP trajectories and prognosis, and (3) explore the effects of baseline clinical characteristics on NT-proBNP trajectories. A retrospective, single-centred, observational study was performed on 52 CTD-PAH patients who had undergone at least three follow-up visits within 1 year from baseline. Four NT-proBNP trajectories were identified using GBTM: low stability (n = 15, 28.85%), early remission (remission within 3 months) (n = 20, 38.46%), delayed remission (remission after 6 or 9 months) (n = 11, 21.15%), and high stability (n = 6, 11.54%). The low-stability and early-remission trajectories were related to a similar positive prognosis, while the delayed-remission and high-stability trajectories were associated with a gradually worsening prognosis (p = 0.000). Intensive CTD immunotherapy (corticosteroids plus immunosuppressants) was the only factor that remained significant after least absolute shrinkage and selection operator regression and multivariate logistic regression, and was independently associated with a lower risk NT-proBNP trajectory (p = 0.048, odds ratio = 0.027, 95% confidence interval: 0.001–0.963), which preliminarily indicated a benefit of CTD-PAH patients undergoing intensive CTD immunotherapy. [ABSTRACT FROM AUTHOR]

BACKGROUND: The morphological alterations of small pulmonary vessels measured by computed tomography (CT) is increasingly used in evaluation of suspected pulmonary hypertension (PH). OBJECTIVE: To investigate the significance alterations of quantitative assessment of small pulmonary vessels on chest CT in distinguishing different types of PH and their severity. METHODS: We retrospectively analyzed a dataset of 120 healthy controls (HCs) and 91 PH patients, including 34 patients with connective tissue diseases-related PH (CTD-PH), 26 patients with idiopathic pulmonary arterial hypertension (iPAH), and 31 patients with chronic obstructive pulmonary disease-related PH (COPD-PH). The CTD-PH patients were divided into mild to moderate PH (CTD-LM-PH) group (n = 17) and severe PH (CTD-S-PH) group (n = 17). A total of 53 CTD patients without PH (CTD-nPH) were enrolled for comparison with the CTD-PH. We measured the cross-sectional area of small pulmonary vessels < 5 mm2 (%CSA <5) and between 5–10 mm2 (%CSA5–10) as a percentage of total lung area among the populations included above and compared %CSA in different types of PH groups and HCs group. The mean pulmonary arterial pressure (mPAP) was measured by right heart catheterization. RESULTS: The %CSA5–10 of COPD-PH, CTD-PH, and iPAH patients increased (0.21±0.09, 0.49±0.20 and 0.61±0.20, p < 0.02) sequentially, while the %CSA <5 of CTD-PH, iPAH, and COPD-PH patients decreased (0.79±0.65, 0.65±0.38 and 0.52±0.27, p < 0.05) sequentially. The %CSA5–10 was significantly higher in CTD-S-PH patients than CTD-LM-PH patients and CTD-nPH patients (0.51±0.21, 0.31±0.15 and 0.28±0.12, p < 0.01). The %CSA5–10 was positively correlated with mPAP in the CTD-PH group. CONCLUSIONS: The quantitative parameters %CSA <5 and %CSA5–10 assessed by chest CT are useful for distinguishing different types of PH. In addition, the %CSA5–10 can provide information for identification of CTD-PH severity. [ABSTRACT FROM AUTHOR]