scholarly journals Plasma exosomal miR-596: a novel biomarker predicts survival in patients with idiopathic pulmonary artery hypertension

2021 ◽  
Vol 49 (3) ◽  
pp. 030006052110023
Author(s):  
Yang Huang ◽  
Zuo-Gang Wang ◽  
Liang Tang ◽  
Su-Gang Gong ◽  
Yuan-Yuan Sun ◽  
...  
Keyword(s):  
Right Heart Catheterization ◽  
Pulmonary Artery Hypertension ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Distribution Analysis ◽  
Poor Overall Survival ◽  
Transmission Electron ◽  
Exosomal Mirnas ◽  
Pulmonary Arterial

Objective To determine if plasma exosomal microRNAs (miRNAs) can predict survival in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods The study enrolled patients with IPAH that underwent right heart catheterization. Plasma was collected and exosomal miRNAs were extracted. Exosomes were evaluated using transmission electron microscopy, Western blot analysis and particle size distribution analysis. MiRNAs were evaluated using a miRNA microarray and validated using real-time polymerase chain reaction. Results This study included 12 patients with IPAH in the study group and 48 patients with IPAH in the validation group. The mean ± SD follow-up duration was 60.3 ± 35.4 months in the overall cohort. The levels of miR-596 were higher in the nonsurvivors compared with the survivors. The levels of miR-596 significantly correlated with survival time, mean right atrial pressure, pulmonary vascular resistance (PVR) and cardiac index. High levels of miR-596 and PVR were significantly associated with poor overall survival. Multivariate analysis demonstrated that exosomal miR-596 (hazard ratio [HR] = 2.119; 95% confidence interval [CI] 1.402, 3.203) and PVR (HR = 1.146; 95% CI 1.010, 1.300) were independent predictors of survival. Conclusions High levels of plasma exosomal miR-596 were significantly associated with disease severity and poor prognosis of patients with IPAH.

Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

scholarly journals Impact of Three Different Algorithms for the Screening of SSc-PAH and Comparison with the Decisions of a Multidisciplinary Team

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1738
Author(s):  
Valentin Coirier ◽  
Céline Chabanne ◽  
Stéphane Jouneau ◽  
Nicolas Belhomme ◽  
Alice Ballerie ◽  
...  
Keyword(s):  
Multidisciplinary Team ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Tertiary Center ◽  
Previous Definition ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

Background: to compare three existing screening algorithms of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) with the results of a multidisciplinary team (MDT) meeting from a tertiary center. Methods: we conducted a monocentric longitudinal study from 2015 to 2018. All patients with SSc according to LeRoy’s classification were eligible. Patients were excluded in the case of missing data required by any of the three screening algorithms. The algorithms were applied for each patient at inclusion. Right heart catheterization (RHC) was performed based on the MDT decision. MDT members were all blinded from the results of the three algorithms regarding RHC recommendations. The RHC recommendations of each algorithm were compared with the MDT decision, and the impact on diagnosis and management was evaluated. Results: 117 SSc patients were consecutively included in the study, and 99 had follow-up data over the three-year duration of the study (10 deaths). Among the 117 patients, the MDT suggested RHC for 16 patients (14%), DETECT algorithm for 28 (24%), ASIG for 48 (41%) and ESC/ERS 2015 for 20 (17%). Among the 16 patients who had RHC, SSc-PAH was diagnosed in seven. Among patients with an initial recommendation of RHC based on at least one algorithm but not according to the MDT meeting, no SSc-PAH was diagnosed during the three-year follow-up. Results were unchanged when the new 2018 definition of PAH was applied instead of the previous definition. Conclusion: a MDT approach appears interesting for the screening of SSc-PAH, with a significant reduction of RHC performed in comparison with dedicated algorithms. The specific relevance of a MDT for the management and follow-up of patients with RHC recommended by existing algorithms but with no PAH warrants further studies.

scholarly journals Accuracy of Echocardiographic Estimates of Pulmonary Artery Pressures in Pulmonary Hypertension: Insights From the KARUM Hemodynamic Database

2021 ◽  
Author(s):  
Ashwin Venkateshvaran ◽  
Natavan Seidova ◽  
Hande Oktay Tureli ◽  
Barbro Kjellström ◽  
Lars H Lund ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Population Studies ◽  
Right Heart Catheterization ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Limits Of Agreement ◽  
Right Heart ◽  
Minimal Bias

Abstract BACKGROUND. Accurate assessment of pulmonary artery (PA) pressures is integral to diagnosis, follow-up and therapy selection in pulmonary hypertension (PH). Despite wide utilization, the accuracy of echocardiography to estimate PA pressures has been debated. We aimed to evaluate echocardiographic accuracy to estimate right heart catheterization (RHC) based PA pressures in a large, dual-centre hemodynamic database. METHODS. Consecutive PH referrals that underwent comprehensive echocardiography within 3 hours of clinically indicated right heart catheterization were enrolled. Subjects with absent or severe, free-flowing tricuspid regurgitation (TR) were excluded. Accuracy was defined as mean bias between echocardiographic and invasive measurements on Bland-Altman analysis for the cohort and estimate difference within ±10mmHg of invasive measurements for individual diagnosis. RESULTS. In 419 subjects, echocardiographic PA systolic and mean pressures demonstrated minimal bias with invasive measurements (+2.4 and +1.9mmHg respectively) but displayed wide limits of agreement (-20 to +25 and -14 to +18mmHg respectively) and frequently misclassified subjects. Recommendation-based right atrial pressure (RAP) demonstrated poor precision and was falsely elevated in 32% of individual cases. Applying a fixed, median RAP to echocardiographic estimates resulted in relatively lower bias between modalities when assessing PA systolic (+1.4mmHg; 95% limits of agreement +25 to –22mmHg) and PA mean pressures (+1.4mmHg; 95% limits of agreement +19 to -16mmHg).CONCLUSIONS. Echocardiography accurately represents invasive PA pressures for population studies but may be misleading for individual diagnosis owing to modest precision and frequent misclassification. Recommendation-based estimates of RAPmean may not necessarily contribute to greater accuracy of PA pressure estimates.

scholarly journals Right ventricular dimension index by cardiac magnetic resonance for prognostication in connective tissue diseases and pulmonary hypertension

Rheumatology ◽  
2019 ◽  
Author(s):  
Nobuya Abe ◽  
Masaru Kato ◽  
Michihito Kono ◽  
Yuichiro Fujieda ◽  
Hiroshi Ohira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Dimension Index

Abstract Objectives Pulmonary hypertension (PH) in patients with CTD is a heterogeneous condition affected by left heart disease, chronic lung disease and thromboembolism as well as pulmonary vascular disease. Recent studies using cardiac magnetic resonance (CMR) have shown that right ventricular dysfunction is predictive for mortality in patients with PH, but limited to pulmonary arterial hypertension. This study aimed to analyse prognostic factors in PH-CTD. Methods This retrospective analysis comprised 84 CTD patients, including SSc, who underwent both CMR and right heart catheterization from 2008 to 2018. Demographics, laboratory findings, and haemodynamic and morphological parameters were extracted. The prognostic value of each parameter was evaluated by multivariate analysis using covariables derived from propensity score to control confounding factors. Results Of 84 patients, 65 had right heart catheterization-confirmed PH (54 pulmonary arterial hypertension, 11 non-pulmonary arterial hypertension). Nine out of these PH patients died during a median follow-up period of 25 months. In 65 patients with PH, right ventricular end-diastolic dimension index (RVEDDI) evaluated by CMR was independently associated with mortality (hazard ratio 1.24; 95% CI: 1.08–1.46; P = 0.003). In a receiver operating characteristic analysis, RVEDDI highly predicted mortality, with area under the curve of 0.87. The 0.5–2-year follow-up data revealed that RVEDDI in both survivors and non-survivors did not significantly change over the clinical course, leading to the possibility that an early determination of RVEDDI could predict the prognosis. Conclusion RVEDDI simply evaluated by CMR could serve as a significant predictor of mortality in PH-CTD. A further validation cohort study is needed to confirm its usability.

scholarly journals Seizures in Idiopathic Pulmonary Arterial Hypertension

2018 ◽  
Vol 05 (02) ◽  
pp. 107-109
Author(s):  
Jamir Pitton Rissardo ◽  
Ana Letícia Fornari Caprara
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Supplemental Oxygen ◽  
Right Heart Catheterization ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Heart Catheterization ◽  
Right Heart ◽  
Systemic Disorder ◽  
Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a progressive pulmonary vasculopathy. A 29-year-old female patient presenting with dyspnea and syncope within 6 hours of onset was admitted to our hospital. The patient stated that she looked for a neurologist months ago because she experienced abrupt shaking limbs occurring during physical activity. She was diagnosed with focal seizure, and carbamazepine (CBZ) was started. On admission, she reported that the dyspnea had started in the last week and recurrent episodes of syncope in the last few hours. A right heart catheterization was diagnostic of PAH. She was started on spironolactone, furosemide, sildenafil, warfarin, and supplemental oxygen. On 10th admission day, the patient was seizure free and the dose of CBZ was tapered. In the follow-up, the patient remained seizure free. An investigation to search for a chronic lung disease or hypoxemia, systemic disorder, hematological disorder, and metabolic disorder was negative.

scholarly journals Assessment of pulmonary artery hypertension by Doppler echocardiography and its correlation with right heart catheterization

2021 ◽  
Vol 8 (4) ◽  
pp. 523
Author(s):  
Vikrant B. Khese ◽  
Chandrakant B. Chavan ◽  
Ravi Kalra ◽  
Anirudh K. Allam ◽  
Abhinav Mohabey
Keyword(s):  
Pulmonary Artery ◽  
Systolic Pressure ◽  
Maximum Velocity ◽  
Right Heart Catheterization ◽  
Pulmonary Artery Hypertension ◽  
Pulmonary Artery Systolic Pressure ◽  
Heart Catheterization ◽  
Right Heart ◽  
Number Of Patients ◽  
Pulmonary Arterial

Background: Definitive diagnosis of pulmonary artery hypertension (PH) requires an elevated mean pulmonary arterial pressure (MPAP) of 25 mmHg at rest measured by right heart catheterization (RHC). As it is invasive mode of investigation, it is declined by many patients, echocardiography was thought to be an acceptable substitute to assess pulmonary arterial pressures. Whether there is a correlation between these measurements is controversial. The aim of this study was to assess PH by echocardiography and its correlation with RHC.Methods: Twenty-six patients aged ≥18 years with pulmonary artery hypertension with or without tricuspid regurgitation (TR) were included in this cross-sectional study. All the patients underwent a transthoracic echocardiography evaluation and were taken for RHC study within an hour.Results: The correlation between pulmonary artery acceleration time (PAAT) and pulmonary artery systolic pressure (PASP) and PAAT and MPAP was significant in all degrees of PH. In contrast, correlation between TR jet maximum velocity (TR Vmax) derived estimated pulmonary artery systolic pressure (EPASP) and PASP was significant in moderate and severe PH, while it did not correlate in mild PH.Conclusions: PAAT is easily measurable parameter and strongly correlates with the values of PASP and the MPAP obtained by right heart catheterization. Implementation of a novel method of determining EPASP from PAAT shall increase significantly the number of patients in whom TTE can be used for the assessment of pulmonary hemodynamic non-invasively.

Abstract 15161: Increased Right Atrial Stiffness in Patients With Heart Failure With Preserved Ejection Fraction and Pulmonary Hypertension

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Jessie van Wezenbeek ◽  
Arno van der Bovenkamp ◽  
Jeroen N Wessels ◽  
Sophia-Anastasia Mouratoglou ◽  
Marie Jose Goumans ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Ejection Fraction ◽  
Right Heart Catheterization ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Preserved Ejection Fraction ◽  
Chamber View ◽  
Patients With Heart Failure ◽  
Pulmonary Arterial

Background: Patients with Heart Failure with preserved Ejection Fraction (HFpEF) and Pulmonary Hypertension (PH) have increased right atrial (RA) pressures. Whether the higher RA pressures are related to increased afterload or overall stiffening of the heart is unknown. The aim of this study is to gain further insight into the right atrium in HFpEF-PH. Methods: This is a retrospective analysis of patients with HFpEF (no PH), HFpEF-PH and Pulmonary Arterial Hypertension (PAH) that underwent right heart catheterization and cardiac magnetic resonance (CMR) imaging. CMR was used to determine RA function by quantifying volume and strain on the 4-chamber view. Total, passive and active RA emptying fraction (RAEF) were calculated. RA stiffness was calculated by determining the slope of maximum and minimum pressure during v-wave and minimal and maximal RA volumes. Groups were compared with ANOVA and post-hoc comparison with Bonferroni correction. Results: 176 patients were included: 13 HFpEF, 33 HFpEF-PH and 130 PAH patients. Although afterload was lower in PAH and higher in HFpEF patients, as shown by mean pulmonary arterial pressure (mPAP) (41 ± 2 mmHg in HFpEF-PH vs 53 ± 21 mmHg in PAH vs 19 ± 1 mmHg in HFpEF, p<0.001) and pulmonary vascular resistance (PVR) (2.3 ± 0.3 wu/m 2 in HFpEF-PH vs 5.7 ± 0.2 wu/m 2 in PAH vs 0.4 ± 0.06 wu/m 2 in HFpEF, p<0.001), mean RA pressure was significantly higher in HFpEF-PH patients compared to both groups (Figure 1A). HFpEF-PH patients had significantly increased RA stiffness compared to HFpEF and PAH patients (Figure 1B). Total RAEF was reduced in HFpEF-PH compared to PAH and HFpEF patients: passive RAEF was similar, but active RAEF was slightly reduced in HFpEF-PH (Figure 1C). This was in line with measurements of RA longitudinal strain (Figure 1D). Conclusions: Despite lower afterload, HFpEF-PH patients have worse RA function and increased RA stiffness compared to PAH. Higher RA pressures in HFpEF-PH may reflect additional stiffening of the heart.

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

scholarly journals Incremental Experience in In Vitro Primary Culture of Human Pulmonary Arterial Endothelial Cells Harvested from Swan-Ganz Pulmonary Arterial Catheters

Cells ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 3229
Author(s):  
Birger Tielemans ◽  
Leanda Stoian ◽  
Allard Wagenaar ◽  
Mathias Leys ◽  
Catharina Belge ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Vascular Diseases ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Endothelial Phenotype ◽  
Pulmonary Arterial ◽  
Arterial Endothelial Cells ◽  
Pulmonary Vascular Diseases

Pulmonary arterial hypertension (PAH) is a devastating condition affecting the pulmonary microvascular wall and endothelium, resulting in their partial or total obstruction. Despite a combination of expensive vasodilatory therapies, mortality remains high. Personalized therapeutic approaches, based on access to patient material to unravel patient specificities, could move the field forward. An innovative technique involving harvesting pulmonary arterial endothelial cells (PAECs) at the time of diagnosis was recently described. The aim of the present study was to fine-tune the initial technique and to phenotype the evolution of PAECs in vitro subcultures. PAECs were harvested from Swan-Ganz pulmonary arterial catheters during routine diagnostic or follow up right heart catheterization. Collected PAECs were phenotyped by flow cytometry and immunofluorescence focusing on endothelial-specific markers. We highlight the ability to harvest patients’ PAECs and to maintain them for up to 7–12 subcultures. By tracking the endothelial phenotype, we observed that PAECs could maintain an endothelial phenotype for several weeks in culture. The present study highlights the unique opportunity to obtain homogeneous subcultures of primary PAECs from patients at diagnosis and follow-up. In addition, it opens promising perspectives regarding tailored precision medicine for patients suffering from rare pulmonary vascular diseases.

4972Upfront triple combination therapy with ambrisentan, tadalafil and subcutaneous treprostinil in incident patients with severe pulmonary arterial hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M D'Alto ◽  
E Romeo ◽  
P Argiento ◽  
R Badagliacca ◽  
S Papa ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Arterial Compliance ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Triple Combination ◽  
Triple Combination Therapy ◽  
Pulmonary Arterial

Abstract Aim Current treatments strategies for high-risk patients with pulmonary arterial hypertension (PAH) are based on the use of parenteral prostanoids. The evidence to support triple upfront combination therapy remains largely based on expert consensus or small studies. Aim of this study was to evaluate the efficacy and safety of an upfront triple combination therapy with ambrisentan, tadalafil and subcutaneous (sc) treprostinil in patients with severe PAH. Methods This is a multi-center retrospective analysis of patients with newly diagnosed severe PAH treated with upfront triple combination therapy with ambrisentan, tadalafil and sc treprostinil between 2014 and 2018. Clinical evaluations, WHO functional class (FC), 6-min walk distance, biomarkers and right heart catheterization were collected from the patients' medical records at baseline and during the follow-up. Results Overall, 20 patients (mean age 44±15 years, 15 female) were included. Over a median follow-up of 12 months, all patients were still alive on triple combination therapy. At baseline 11 patients were in WHO-FC 3 and 9 patients in WHO-FC 4. At follow-up, WHO-FC (2.0±0.5 vs 3.5±0.5, p<0.001, improved in all: 2, 16, and 2 patients in FC 1, 2 and 3, respectively), exercise capacity (431±67 vs 152±130 m, p<0.001), NT-proBNP (423±260 vs 3492±1864 pg/ml; p<0.001), and haemodynamics (right atrial pressure 5±2 vs 13±3 mmHg, p<0.001; mean pulmonary artery pressure 42±5 vs 60±9 mmHg, p<0.001; cardiac index 3.5±0.8 vs 1.8±0.3 l/min/m2, p<0.001; pulmonary vascular resistance 5.5±1.3 vs 16.4±4.4 Wood units, p<0.001; pulmonary arterial compliance 2.5±0.9 vs 0.8±0.3 ml/mmHg, p<0.001) significantly improved compared with baseline. No patient discontinued the therapy due to serious adverse events. Conclusions Triple upfront combination therapy with ambrisentan, tadalafil and sc treprostinil is safe and offers clinical and heamodynamics benefits in incident patients with severe PAH.

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