scholarly journals Hipertensión pulmonar: Correlación de los marcadores plasmáticos con la supervivencia de los pacientes

2021 ◽  
pp. 1-2
Author(s):  
Marco Hugo Sánchez-Bustillos

<b>Background:</b> Recent studies have provided evidence for an important contribution of the immune system in the pathophysiology of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). In this report, we investigated whether the inflammatory profile of pulmonary hypertension patients changes over time and correlates with patient WHO subgroups or survival. <b>Methods:</b> 50 PAH patients (16 idiopathic (I)PAH, 24 Connective Tissue Disease (CTD)-PAH and 10 Congenital Heart Disease (CHD)-PAH), 37 CTEPH patients and 18 healthy controls (HCs) were included in the study. Plasma inflammatory markers at baseline and after 1-year follow-up were measured using ELISAs. Subsequently, correlations with hemodynamic parameters and survival were explored and data sets were subjected to unbiased multivariate analyses. <b>Results:</b> At diagnosis, we found that plasma levels of interleukin-6 (IL-6) and the chemokines (C-X3-C) motif legend CXCL9 and CXCL13 in CTD-PAH patients were significantly increased, compared with HCs. In idiopathic PAH patients the levels of tumor growth factor-β (TGFβ), IL-10 and CXCL9 were elevated, compared with HCs. The increased CXCL9 and IL-8 concentrations in CETPH patients correlated significantly with decreased survival, suggesting that CXCL9 and IL-8 may be prognostic markers. After one year of treatment, IL-10, CXCL13 and TGFβ levels changed significantly in the PAH subgroups and CTEPH patients. Unbiased multivariate analysis revealed clustering of PH patients based on inflammatory mediators and clinical parameters, but did not separate the WHO subgroups. Importantly, these multivariate analyses separated patients with &#x3c;3 years and &#x3e;3 years survival, in particular when inflammatory mediators were combined with clinical parameters. <b>Discussion:</b> Our study revealed elevated plasma levels of inflammatory mediators in different PAH subgroups and CTEPH at baseline and at 1-year follow-up, whereby CXCL9 and IL-8 may prove to be prognostic markers for CTEPH patients. While this study is exploratory and hypothesis generating, our data indicate an important role for IL-8 and CXCL9 in CHD and CTEPH patients considering the increased plasma levels and the observed correlation with survival. <b>Conclusion:</b> In conclusion, our studies identified an inflammatory signature that clustered PH patients into WHO classification-independent subgroups that correlated with patient survival.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4006-4006
Author(s):  
Franco Piovella ◽  
Andrea M. D’Armini ◽  
Marisa Barone ◽  
Vincenzo Emmi ◽  
Chiara Beltrametti ◽  
...  

Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which results from obstruction of the major pulmonary arteries by incompletely resolved or organized pulmonary emboli which have become incorporated into the pulmonary artery wall, eventually causing an increase in pulmonary vascular resistances. Pulmonary endarterectomy (PEA) is the treatment of choice. Careful pre- and post-operative management is essential for a successful outcome following PEA. In 1994, we started in Pavia a program in which members of a multidisciplinary team work in close interaction with the aim of increase experience in the challenging problems these patients present in the evaluative, surgical, and post-operative phases of their care. So far, 134 PEAs have been performed. Preoperatively, New York Heart Association (NYHA) class distribution was respectively 3-II, 56-III, and 75-IV; mean pulmonary artery pressure and pulmonary vascular resistances were 47 ± 13 mmHg and 1149 ± 535 dynes/sec/cm−5 respectively. The overall operative mortality has been 9.7% (in 2005 mortality rate was 4.5%). At present, 92% of the PEA patients are actively participating in the follow-up study. Follow-up visits are at 3 months after PEA, yearly for the following 5 years, and then at 7, 10, and 15 years postoperatively. Both early and late survivals were excellent. Survival rate at 3 months, 1 year, and 3 years were respectively of 89.5±2.7%, 87.3±3.0%, and 82.7±3.6%. Survival rates had not changed at 5, 7, and 10 years postoperative. Three months after PEA, 29 (58%) subjects were within NYHA class I, 18 (36%) in class II, and 3 (6%) in class III. At 1-year follow-up, 40 (80%) patients were within NYHA class I, 10 (20%) in class II. A statistically significant difference exists not only between the preoperative and the postoperative data (p <0.0001), but also between the functional status at 3 months and the other two postoperative controls (p <0.001). Table summarizes the results of hemodynamic tests collected at three months, one year and three years on the first 35 patients who completed the follow-up program. Hemodynamic data from 35 patients participating to the Pavia Pulmonary Endarterectomy Program with complete 3-year follow-up. CVP mPAP CO CI PVR PVRI CVP (mmHg) central venous pressure; mPAP (mmHg) mean pulmonary artery pressure; CO (L/min) cardiac output; CI (L/min/m2) cardiac index; PVR (dynes/sec/cm-5) pulmonary vascular resistances; PVRI (dynes/sec/cm-5/m2) pulmonary vascular resistances index; RV-EF (%) right ventricle ejection fraction. RV-EF A: Before-PEA 7±6 48±12 3.3±0.9 1.8±0.5 1125±412 2027±731 15±8 B:Before discharge 5±4 25±10 5.2±1.1 2.9±0.5 289±142 505±234 32±8 C: 3 months 2±2 24±11 5.1±1.4 2.8±0.6 231±198 542±271 32±7 D: 1 year 1±2 23±12 5.0±1.1 2.7±0.6 290±191 531±343 35±8 E: 3 years 2±2 24±12 4.9±1.1 2.6±0.5 317±226 579±393 34±8 p value A vs. B: nsA vs. C, D, and E: <0.0001B vs. C, D and E: <0.05 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E:


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Juanni Gong ◽  
Yuanhua Yang ◽  
Jianfeng Wang ◽  
Yidan Li ◽  
Xiaojuan Guo ◽  
...  

Background. MicroRNAs (miRNAs) play an important role in the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH). However, the potential correlation between miRNA expression and the severity of CTEPH remains unclear. Our previous study indicated that miRNAs hsa-let-7b-3p, hsa-miR-17-5p, hsa-miR-106b-5p, hsa-miR-3202, hsa-miR-665, and hsa-miR-93-5p are closely involved in CTEPH. This study assessed the associations between the expression levels of these miRNAs and clinical parameters in CTEPH patients. Methods. A total of eight CTEPH patients and eight healthy adults as a reference group were included, and clinical data including total protein (TP), albumin (Alb), lactate dehydrogenase (LDH), hydroxybutyrate dehydrogenase (HBDH), uric acid (UA), and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels were collected. Right heart catheterization was conducted to obtain hemodynamic data including cardiac index (CI). The expression levels of let-7b-3p, miR-17-5p, miR-106b-5p, miR-3202, miR-665, and miR-93-5p were measured by quantitative real-time PCR (qPCR). Correlation analysis was applied to estimate the associations between miRNA expression levels and clinical parameters in CTEPH patients. Results. Serum TP and Alb levels were decreased, while LDH, HBDH, and UA levels were increased in CTEPH patients compared with the reference group ( P < 0.05 ). miR-3202 and miR-665 were upregulated, whereas let-7b-3p, miR-17-5p, miR-106b-5p, and miR-93-5p were downregulated in CTEPH patients relative to the reference group ( P < 0.05 ). miR-93-5p expression was positively correlated with NT-proBNP level and negatively correlated with CI ( P < 0.05 ). Moreover, let-7b-3p tended to be positively correlated with mean pulmonary arterial pressure. Conclusions. miR-93-5p expression was associated with the severity of CTEPH and could act as a potential predictor of high-risk CTEPH.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A R Pereira ◽  
R Cale ◽  
F Ferreira ◽  
S Alegria ◽  
S Sebaiti ◽  
...  

Abstract Introduction Balloon pulmonary angioplasty (BPA) has emerged as a therapeutic option for chronic thromboembolic pulmonary hypertension (CTEPH) considered ineligible for pulmonary endarterectomy (PEA). The initial publications showed very good short-term outcomes for the technique, but there are limited data regarding medium-term outcomes and its comparison with optimal medical treatment (OMT). Objectives To evaluate and compare the medium-term outcomes of OMT versus (vs) BPA in inoperable CTEPH. Methods Retrospective study of consecutive patients (pts) with CTEPH followed in a referral centre for Pulmonary Hypertension. Selected those pts considered ineligible for PEA and with at least 2 years of follow-up. Comparison between two treatment strategies: OMT alone [maximum tolerated doses of pulmonary vasodilator drugs (PVD), as indicated] vs BPA (pts who completed the program with or without OMT). Endpoint was a composite of all-cause death and unplanned right heart failure admission at 2-year. Results From 62 pts, 19 pts were included (11 pts were excluded due to recent diagnosis; 32 were submitted to EAP): mean age 65.0±15.3 years, 89.5% female. At diagnosis, all pts had functional capacity limitation and elevated serum NTproBNP levels (median value 1255.0 pg/mL). Mean pulmonary arterial pressure (mPAP) was 46.2±9.3 mmHg and pulmonary vascular resistance (PVR) 15.3±8.3 Wood units (WU). Concerning treatment, 12 pts (63.2%) underwent OMT alone. These pts had higher NTproBNP levels (2670.0 vs 538.0 pg/mL, p&lt;0.01) and PVR values (19.7±7.6 vs 9.7±5.4 WU, p=0.01) and lower CI (1.6±0.3 vs 2.4±0.5 L/min/m2, p&lt;0.01), at baseline; the remaining basal features didn't differ among groups (Fig.A). At 2-year follow-up, pts submitted to BPA were under PVD in 71.4% of cases with a mean of 1±0.8 drugs per patient and no difference compared to OMT group (83.3%, 1.7±0.9 drugs per patient), although oxygen therapy was higher in medical group (50% vs 0%, p=0.04). A significant overall improvement was observed in BPA group (Table – A): all pts were in functional class I (p&lt;0.01), no one had right ventricular dysfunction (p&lt;0.01) and mPAP decreased to 25.1±6.7 mmHg (p=0.01) and RVP to 2.9±0.8 WU (p=0.01). Inversely, no change was observed in pts under OMT alone (p&gt;0.05 in all, Table – A). Endpoint rate was 31.6% with all adverse events occurring in the OMT group (50% vs 0%, p=0.04). After adjustment by Cox regression, no difference in baseline or follow-up features besides treatment influenced the outcome. Kaplan-Meier analysis (Graphic – B) confirmed significant benefit of BPA in 2-year outcome occurrence (long rank 4.6, p=0.03). Conclusions BPA strategy seems to improve medium-term functional capacity, right ventricular function and haemodynamics and decrease oxygen therapy dependence in inoperable CTEPH. Pts under OMT alone have a poor prognosis. These data encourage the development and implementation of the technique for inoperable CTEPH. FUNDunding Acknowledgement Type of funding sources: None.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Barco ◽  
A Mavromanoli ◽  
F A Klok ◽  
S V Konstantinides

Abstract Background Up to one-third of patients report persisting hemodynamic abnormalities and functional limitation over long-term follow-up after acute pulmonary embolism (PE). Purpose We tested whether a validated algorithm designed to rule-out chronic thromboembolic pulmonary hypertension (CTEPH) after acute PE can be used for identifying patients at lower risk of presenting with persisting symptoms and echocardiographic abnormalities. Methods The multicentre Follow-up of Acute Pulmonary Embolism (FOCUS) cohort study prospectively enrolled 1,100 consecutive patients diagnosed with acute symptomatic PE; two-year follow-up is ongoing. We focused on the scheduled visits for 3- and 12-month follow-up. The rule-out criteria are based on: the absence of ECG signs of right ventricular dysfunction and normal NT-proBNP/BNP values. Echocardiographic abnormalities were defined according to the presence of abnormal parameters indicating an intermediate/high probability of pulmonary hypertension as recommended by the 2015 ESC/ERS Society Guidelines on Pulmonary Hypertension. The presence of functional limitation was defined based on a World Health Organization classification grade ≥3, a Borg dyspnoea index ≥4, or a 6-minute walking distance <300 m. Results We included 323 patients (mean age 61 years, 58% men), of whom 255 have meanwhile completed a one-year follow-up. At 3- and 12-month follow-up, 194 (60%) and 155 (61%) of patients exhibited no abnormal echocardiographic findings or natriuretic peptide levels. The percentage of patients with echocardiographic abnormalities was 20.4% and 18.0%, respectively. The negative predictive value of the score for ruling out the combination of functional limitation and intermediate/high probability of pulmonary hypertension as recommended by the 2015 ESC/ERS Guidelines on Pulmonary Hypertension was 0.96 (95% CI 0.92–0.98) at 3 and 0.97 (0.92–0.99) at 12 months. The corresponding positive predictive values were 0.10 (0.06–0.17) and 0.09 (0.05–0.17), respectively. Conclusions The CTEPH rule-out criteria are capable of excluding functional limitation and evidence of (chronic) pulmonary hypertension 3 and 12 months after the diagnosis of acute PE. Acknowledgement/Funding The sponsor (University Medical Center of the Johannes Gutenberg University, Mainz) has obtained grants from Bayer Vital GmbH and Bayer Pharma AG


2020 ◽  
Vol 6 (2) ◽  
pp. 00218-2019
Author(s):  
Abdullah M. Aldalaan ◽  
Sarfraz A. Saleemi ◽  
Ihab Weheba ◽  
Abeer Abdelsayed ◽  
Pekka Hämmäinen ◽  
...  

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.MethodsThis study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.ResultsAt the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).ConclusionPatients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.


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