[Pulmonary hypertension associated with chronic lung diseas].

PULMONARY HYPERTENSION ASSOCIATED WITH CHRONIC LUNG DISEASES. Pulmonary hypertension is commonly observed in chronic respiratory diseases. It is responsible for a worsening of quality of life and a decrease in patient survival in the short and medium term.

Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities.

Background: European guidelines recommend initial monotherapy in pulmonary arterial hypertension patients with cardiovascular comorbidities based on the limited evidence for combination therapy in this growing population.

Methods: A retrospective analysis was conducted on incident pulmonary arterial hypertension patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy oral monotherapy in patients with at least one cardiovascular comorbidity ( hypertension, obesity, diabetes and coronary artery disease).

Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients.

Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional, and imaging characteristics of interstitial lung disease in patients with anti-Ku antibodies. : This single-center, retrospective observational study was conducted at a tertiary referral institution.

Evidence and unresolved questions in pulmonary hypertension: Insights from the 5th French Pulmonary Hypertension Network Meeting.

Pulmonary hypertension (PH) continues to present significant challenges to the medical community, both in terms of diagnosis and treatment. The advent of the updated 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines has introduced pivotal changes that reflect the rapidly advancing understanding of this complex disease. These changes include a revised definition of PH, updates to the classification system, and treatment algorithm.

Bioreactance assessment of cardiac output lacks reliability for the follow-up of patients with pulmonary hypertension.

Cardiac output (CO) is one of the primary prognostic factors evaluated during the follow-up of patients treated for pulmonary hypertension (PH). It is recommended that it be measured using the thermodilution technique during right heart catheterization. The difficulty to perform iterative invasive measurements on the same individual led us to consider a non-invasive option.

Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension.

Background: Haemodynamic variables are prognostic factors in pulmonary arterial hypertension (PAH). However, right heart catheterisation (RHC) is not systematically recommended to assess the risk status during follow-up. This study aimed to assess the added value of haemodynamic variables in prevalent patients to predict the risk of death or lung transplantation according to their risk status assessed by the non-invasive four-strata model as recommended by the European guidelines.

Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis.

Introduction: Asthma associated with eosinophilic granulomatosis with polyangiitis (EGPA) is often severe and corticosteroid-dependent, leading to significant morbidity. Mepolizumab and benralizumab are humanized monoclonal antibodies targeting interleukin 5 (IL-5) and its receptor, respectively. They have been shown to be effective in steroid-sparing in patients with severe eosinophilic asthma.

Pulmonary function test results are correlated with 6-minute walk distance, distance-saturation product, and 6-minute walk work in patients with lymphangioleiomyomatosis.

Background And Objective: Lymphangioleiomyomatosis (LAM) is a rare multicystic lung disease. Although a correlation between pulmonary function test (PFT) results and exercise capacity appears probable, it has not yet been demonstrated. The aim of this study was to assess whether PFT results correlate with 6-minute walk test (6MWT) results in patients with LAM.

Interstitial lung disease-associated pulmonary hypertension - what the future holds.

Purpose Of Review: Pulmonary hypertension associated with interstitial lung disease (ILD-PH) is associated with significant alteration of quality of life, exercise capacity, and survival. Over the past 2 years, there were changes in the guideline definition and classification of ILD-PH, and positive randomized controlled trials were published.

Recent Findings: Pulmonary hypertension associated with chronic lung disease is now hemodynamically defined as a mean pulmonary artery pressure more than 20 mmHg, with pulmonary artery wedge pressure 15 mmHg or less, and pulmonary vascular resistance (PVR) at least 2 Wood units.

Changes in the management of chronic thromboembolic pulmonary hypertension over a 10-year period, in a French expert regional competence centre.

Over the last few years, the advent of balloon pulmonary angioplasty (BPA) had led to changes in the management of chronic thromboembolic pulmonary hypertension (CTEPH). We reviewed data from 98 CTEPH patients diagnosed during the last decade in a pulmonary hypertension (PH) expert centre. The management modalities of 2 periods (Period A: 2011-15 and Period B: 2016-20) were compared.

French recommendations for the diagnosis and management of lymphangioleiomyomatosis.

Background: The present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France.

Methods: Practical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases.

Lung Dual-Energy CT Perfusion Blood Volume as a Marker of Severity in Chronic Thromboembolic Pulmonary Hypertension.

In chronic thromboembolic pulmonary hypertension (CTEPH), assessment of severity requires right heart catheterization (RHC) through cardiac index (CI). Previous studies have shown that dual-energy CT allows a quantitative assessment of the lung perfusion blood volume (PBV). Therefore, the objective was to evaluate the quantitative PBV as a marker of severity in CTEPH.

Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis.

Background: Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in patients with idiopathic and secondary PPFE. Patients admitted to a tertiary care center (2010-2019) were included into this retrospective analysis if they had a multidisciplinary diagnosis of PPFE, had been followed-up for 3 months or more, and had lung function tests and chest CTs available for review.

Pulmonary hypertension in hereditary haemorrhagic telangiectasia is associated with multiple clinical conditions.

https://bit.ly/34V7HPy.

Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis.

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10-30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF).

Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF.

Methods: This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center.

demonstration of pulmonary microvascular involvement in COVID-19 using dual-energy computed tomography.

https://bit.ly/2Zh0XcI

Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease.

Objective: Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc-PH-ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc-PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc-PH-ILD. We undertook this study to compare survival of and response to treatment in patients with SSc-PH-ILD and those with SSc-PAH.

Impact of comorbidities and delay in diagnosis in elderly patients with pulmonary hypertension.

Patient age at diagnosis of pulmonary hypertension is steadily increasing. The present study sought to analyse clinical characteristics, time to diagnosis and prognosis of pulmonary hypertension in elderly and very elderly patients. A study was conducted in a French regional referral centre for pulmonary hypertension.

Safety of right heart catheterization for pulmonary hypertension in very elderly patients.

Right heart catheterization (RHC) is the reference test in diagnosing pulmonary hypertension (PH). The increasing age of patients at the time of diagnosis raises the issue of the morbidity of this invasive test in elderly individuals. We hypothesized that the morbidity associated with RHC would be increased in elderly patients and highlight differences in hemodynamic characteristics compared to younger patients.

Pulmonary hypertension in chronic lung diseases: comparison to other pulmonary hypertension groups.

Group 3 pulmonary hypertension (PH) is a common complication of advanced chronic lung disease. Our hypothesis was that group 3 PH is associated with a more severe baseline presentation and a more severe prognosis compared to group 1 pulmonary arterial hypertension (PAH), chronic thromboembolic PH (group 4), and group 5 PH. We retrospectively analyzed consecutive incident PH patients in a single center between January 2006 and November 2014.

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension.

Background: Pulmonary arterial hypertension (PAH) may lead to right heart failure and subsequently alter glomerular filtration rates (GFR). Chronic kidney disease (CKD, GFR <60 mL/min/1.73 m2) may also adversely affect PAH prognosis.

Severe pulmonary hypertension associated with COPD: hemodynamic improvement with specific therapy.

Background: There is no recommendation for treating pulmonary hypertension (PH) when associated with chronic obstructive pulmonary disease (COPD).

Objective: To evaluate the effect of PH-specific therapy in patients with COPD.

Methods: All successive patients with severe PH [mean pulmonary arterial pressure (mPAP) ≥35 mm Hg] and COPD, who received specific PH medication and who underwent right heart catheterization at baseline and after 3-12 months of treatment, were analyzed from a prospective database.