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 ◽  
...  

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.

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza

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.


Rheumatology ◽  
2019 ◽  
Author(s):  
Nobuya Abe ◽  
Masaru Kato ◽  
Michihito Kono ◽  
Yuichiro Fujieda ◽  
Hiroshi Ohira ◽  
...  

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.


2018 ◽  
Vol 05 (02) ◽  
pp. 107-109
Author(s):  
Jamir Pitton Rissardo ◽  
Ana Letícia Fornari Caprara

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.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Koell ◽  
S Ludwig ◽  
O Bhadra ◽  
A Gossling ◽  
N Schofer ◽  
...  

Abstract Background Pulmonary hypertension (PH) due to left heart disease is the most common form of PH. Published literature suggests increased perisurgical mortality in patients undergoing surgical repair in the setting of preexisting PH. The data on the impact of preexisting PH on clinical outcomes after percutaneous Mitral Valve Edge-to-Edge Repair (pMVR) is limited to observational studies and rely mostly on echocardiographic data. Purpose The aim of the current study is to evaluate the influence of preexisting PH in patients undergoing pMVR analyzing periprocedural invasive right heart catheterization data. Methods Between September 2008 and July 2018, a total of 911 patients with moderate-to-severe or severe mitral regurgitation (MR) underwent pMVR at our center. This analysis includes 331 patients with a complete data set for pre- and postprocedural right heart catheterization and echocardiographic assessment as well as available follow-up information after the implantation. Patients are divided according to the etiology of PH. The combined primary endpoint consists of all-cause mortality and rehospitalization for heart failure. Furthermore, a sub-analysis is performed for all patients with preexisting post-capillary PH. Patients with post-capillary PH are divided into two groups based on a postprocedural decrease of pulmonary artery wedge pressure (mPAWP) below the threshold of 15mmHg. Univariate and multivariate Cox regression analyses are performed to assess the influence on long-term outcome. Results Of all 331 patients (57.7% [n= 191] male) undergoing pMVR, 195 (62.1%) had functional MR. Median ejection fraction was 40.5% (29.3, 54.0). Patients were followed-up for a maximum of 4.41 years and the median follow-up time was 1.98 years. Preexisting PH (mean pulmonary artery pressure ≥25 mmHg) was found in 236 (71.1%) patients: 49 patients had pre-capillary PH (≤15 mmHg), 187 had post-capillary PH (pcPH; n=183; mPAWP >15 mmHg). In Kaplan-Meier analysis, no statistically significant difference could be found in overall mortality in patients without or with PH, irrespective of etiology (p=0.43). However, in patients suffering from post-capillary PH, patients with a postprocedural reduction of mPAWP below the threshold of 15mmHg showed a significantly lower risk for overall long-term mortality compared to patients without a relevant mPAWP reduction (p=0.018). Multivariate analysis revealed acute postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH to have a significant influence on mortality (HR 2.81 [1.35, 5.86]; p=0.006; Figure 1). Conclusion In contrast to previously published findings, the present results were not able to show a significant impact of PH, disregarding its etiology, on outcome. Nevertheless, a postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH is associated with a favorable outcome. Figure 1 Funding Acknowledgement Type of funding source: None


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Mário Santos ◽  
Aaron B Waxman ◽  
Julie Tracy ◽  
Fariha Khalid ◽  
Alexander R Opotowsky ◽  
...  

Introduction: Supine resting right heart catheterization (srRHC) is the standard method to differentiate pulmonary arterial hypertension (PAH) and heart failure with preserved ejection fraction (HFpEF), but most such patients complain of symptoms during exercise. We hypothesized that the upright invasive exercise phenotype of patients with unexplained exertional intolerance provides a distinct and additive perspective compared with supine resting RHC in the diagnosis of PAH and HFpEF. Methods: We reviewed results of consecutive patients with unexplained effort intolerance who underwent same day sequential srRHC and invasive cardiopulmonary exercise testing (iCPET) between March 2011 and October 2013. At rest, patients were classified with PAH if mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg; as HFpEF if PAWP > 15 mmHg; and as normal if none of the above hemodynamic criteria were met. At peak exercise, patients were categorized as exercise-induced PAH (eiPAH), exercise HFpEF (eHFpEF), normal (eNormal), or peripheral limitation according to the criteria displayed in the table. Results: Of 255 patients, 212 (83%) had normal srRHC. Of these, 46 (22%) had an abnormal iCPET result: eiPAH (n=24), eHFpEF (n=22). A resting mPAP > 18 mmHg discriminated eiPAH reasonably well (ROC AUC: 0.75; 95%CI: 0.67-0.83). Of those with abnormal srRHC, iCPET reclassified diagnosis for 16/43 (37%). Of the 30 patients who had HFpEF by srRHC, 12 (40%) had a normal cardiac hemodynamic profile during upright maximum exercise. 4 (31%) of the 13 patients with PAH at rest had no suggestion of intrinsic pulmonary vascular disease during exercise (3 eHFpEF and 1 non-cardiac limitation) with iCPET. Conclusions: In patients with exertional intolerance, iCPET reveals hemodynamic abnormalities which are overlooked with resting RHC and reclassifies a significant subset of apparent PAH and HFpEF patients by srRHC.


2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Stefano Ghio

[No abstract. Showing first paragraph of article]Right heart catheterization (RHC) has a crucial role in the management of patients with pulmonary hypertension (PH). Firstly, it is mandatory in subjects with the suspicion of PH to confirm the diagnosis. Secondly, it is strongly recommended in the follow-up of patients with confirmed pulmonary arterial hypertension (PAH) to evaluate drug efficacy and in case of clinical deterioration.In order to always perform a complete and correct RHC, clinicians carrying out the examination must be guided not only by technical recommendations, but also by the accurate knowledge of the different clinical issues which have to be addressed in these different situations.


2021 ◽  
Vol 49 (3) ◽  
pp. 030006052110023
Author(s):  
Yang Huang ◽  
Zuo-Gang Wang ◽  
Liang Tang ◽  
Su-Gang Gong ◽  
Yuan-Yuan Sun ◽  
...  

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.


2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877305 ◽  
Author(s):  
Batool AbuHalimeh ◽  
Milind Y. Desai ◽  
Adriano R. Tonelli

The diagnosis of pulmonary hypertension (PH) requires a right heart catheterization (RHC) that reveals a mean pulmonary artery pressure ≥ 25 mmHg. The pulmonary artery catheter traverse the right atrium and ventricle on its way to the pulmonary artery. The presence of abnormal right heart structures, i.e. thrombus, vegetation, benign or malignant cardiac lesions, can lead to complications during this procedure. On the other hand, avoidance of RHC delays the diagnosis and treatment of PH, an approach that might be associated with worse outcomes. This paper discusses the impact of right heart lesions on the diagnosis of PH and suggests an approach on how to manage this association.


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