ERS International Congress 2023: highlights from the Pulmonary Vascular Diseases Assembly.

Pulmonary vascular diseases such as pulmonary embolism and pulmonary hypertension are important and frequently under-recognised conditions. This article provides an overview of key highlights in pulmonary vascular diseases from the European Respiratory Society International Congress 2023. This includes insights into disease modification in pulmonary arterial hypertension and novel therapies such as sotatercept and seralutinib. Exciting developments in our understanding of the mechanisms underpinning pulmonary hypertension associated with interstitial lung disease are also explored. A comprehensive overview of the complex relationship between acute pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH) is provided along with our current understanding of the molecular determinants of CTEPH. The importance of multidisciplinary and holistic care cannot be understated, and this article also addresses advances beyond medication, with a special focus on exercise training and rehabilitation.

Post-COVID central hypersomnia, a treatable trait in long COVID: 4 case reports.

Introduction: Fatigue is the most commonly reported post-COVID symptom. A minority of patients also report excessive daytime sleepiness, which could be a target for treatment. Methods: Among 530 patients with a post-COVID condition, those with excessive daytime sleepiness were systematically assessed for objective central hypersomnia, with exclusion of all cases not clearly attributable to SARS-CoV-2 infection. Results: Four cases of post-COVID central hypersomnia were identified, three fulfilling the criteria of the 3rd International Classification of Sleep Disorders for idiopathic hypersomnia, and one for type II narcolepsy. We report here their clinical history, sleep examination data and treatment, with a favorable response to methylphenidate in three cases and spontaneous resolution in one case. Conclusion: We highlight the importance of identifying cases of post-COVID central hypersomnia, as it may be a treatable trait of a post-COVID condition.

[Pneumology: what's new in 2023]. / Pneumologie : ce qui a changé en 2023.

In this review of new developments in pulmonology for the year 2023, we look at two contributions in the diagnostic field: the optimal way of comparing a spirometry measurement with the expected normal values, and a new tool for identifying dysfunctional breathing. On the therapeutic front, a new molecule, ensifentrine, has been shown to be effective in a phase 3 study involving COPD patients. Finally, and still for patients with severe COPD, volume reduction, either surgically or endoscopically, can lead to an improvement in function and severity scores.

Dans cet article des nouveautés en pneumologie pour l'année 2023, nous abordons deux apports dans le domaine diagnostique : la manière optimale de comparer une mesure de spirométrie aux valeurs normales attendues, ainsi qu'un nouvel outil pour identifier la respiration dysfonctionnelle. Au niveau thérapeutique, une nouvelle molécule, l'ensifentrine, s'est révélée probante dans une étude de phase 3 pour les patients atteints de BPCO. Enfin, et toujours pour les patients atteints de BPCO sévère, une réduction de volume, soit chirurgicale, soit par voie endoscopique, peut amener une amélioration fonctionnelle et des scores de sévérité.

[Pulmonary hypertension: how to differentiate rare from frequent etiologies?] / Hypertension pulmonaire : comment différencier les causes fréquentes et rares?

Pulmonary hypertension (PH) is a frequent finding. PH secondary to left heart diseases is the most prevalent form of PH. PH caused by lung diseases and/or hypoxia is the second most frequent cause. The patient should be addressed to an expert center if the PH does not seem to be secondary to a left heart disease or a lung disease, if the PH seems too severe for the underlying cardiac or pulmonary diseases or in the presence of risk factors for PH caused by rare etiologies (group 1, 4 and 5).

L'hypertension pulmonaire (HTP) est une manifestation clinique fréquente. L'HTP secondaire aux cardiopathies gauches est la forme la plus prévalente. La deuxième forme la plus fréquente est l'HTP associée à une pneumopathie et/ou à une hypoxie chronique. Le patient devrait être adressé à un centre expert si l'HTP n'est pas facilement attribuable à une cardiopathie gauche ou à une pneumopathie, si l'HTP semble trop sévère pour la cardiopathie ou la pneumopathie sous-jacente ou en cas de présence de facteurs de risque pour une HTP causée par une étiologie rare (groupes 1, 4 et 5).

Dysfunctional breathing symptoms, functional impact and quality of life in patients with long COVID-19: a prospective case series.

BACKGROUND: Dysfunctional breathing is increasingly recognised after SARS-CoV-2 infection, but the associated symptoms, functional impact and quality of life have not been systematically studied. METHODS: This study describes a prospective case series of 48 patients with dysfunctional breathing based on compatible symptoms and an abnormal breathing pattern during cardiopulmonary exercise testing. Patients with underlying disease that could explain these symptoms were excluded. Median time from COVID-19 to evaluation was 212 (IQR 121) days. Self-administered questionnaires, including the Nijmegen questionnaire, Short-Form (36) Health Survey (SF-36), Hospital Anxiety and Depression Scale, modified Medical Research Council scale, post-COVID-19 Functional Scale, and specific long COVID symptoms, were the outcome measures. RESULTS: On average, mean V'O2 was preserved. Pulmonary function tests were within limits of normality. Hyperventilation, periodic deep sighs/erratic breathing and mixed types of dysfunctional breathing were diagnosed in 20.8%, 47.1% and 33.3% of patients, respectively. After dyspnoea, the five most frequent symptoms using the Nijmegen scale with a cut-off of ≥3 were faster/deeper breathing (75.6%), palpitations (63.8%), sighs (48.7%), unable to breathe deeply (46.3%) and yawning (46.2%). Median Nijmegen and Hospital Anxiety and Depression Scale scores were 28 (IQR 20) and 16.5 (IQR 11), respectively. SF-36 scores were lower than the reference value. CONCLUSIONS: Long COVID patients with dysfunctional breathing have a high burden of symptoms, functional impact and a low quality of life, despite no or negligible organic damage.

Pulmonary vascular disease as a complication of pediatric congenital heart diseases.

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review.

The Influence of Methods for Cardiac Output Determination on the Diagnosis of Precapillary Pulmonary Hypertension: A Mathematical Model.

Background: precapillary pulmonary hypertension (PH, PcPH) is now defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg, a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 2 WU. For PVR calculation, the measurement of cardiac output (CO) is necessary. It is generally measured using thermodilution. However, recent data showed that the agreement with direct Fick method, historically the gold standard, is less than previously reported. We aimed to create a mathematical model that calculated the probability of being classified differently (PcPH or unclassified PH) if CO measured by direct Fick was used instead of thermodilution for any individual patients with a mPAP > 20 mmHg and a PAWP ≤ 15 mmHg. Methods: The model is based on Bland and Altman analysis with a normally distributed difference of cardiac output, fixed 1.96 standard deviation of bias, bias and physiological cardiac output limits. Results: Following a literature review of the studies comparing CO measured with direct Fick and thermodilution, we fixed the 1.96 standard deviation of bias at 2 L/min, bias at 0 L/min and physiological resting CO limits between 1.3 L/min and 10.2 L/min. Conclusions: This model can help the clinician to evaluate the potential benefit of measuring CO using direct Fick during the diagnostic work-up and its utility in confirming or ruling out a diagnosis of PcPH in any given patient with a mPAP > 20 mmHg and a PAWP ≤ 15 mmHg.

High-flow nasal oxygen in acute hypoxemic respiratory failure: A narrative review of the evidence before and after the COVID-19 pandemic.

High-flow nasal oxygen (HFNO) is a type of non-invasive advanced respiratory support that allows the delivery of high-flow and humidified air through a nasal cannula. It can deliver a higher inspired oxygen fraction than conventional oxygen therapy (COT), improves secretion clearance, has a small positive end-expiratory pressure, and exhibits a washout effect on the upper air space that diminishes dead space ventilation. HFNO has been shown to reduce the work of breathing in acute hypoxemic respiratory failure (AHRF) and has become an interesting option for non-invasive respiratory support. Evidence published before the COVID-19 pandemic suggested a possible reduction of the need for invasive mechanical ventilation compared to COT. The COVID-19 pandemic has resulted in a substantial increase in AHRF worldwide, overwhelming both acute and intensive care unit capacity in most countries. This triggered new trials, adding to the body of evidence on HFNO in AHRF and its possible benefits compared to COT or non-invasive ventilation. We have summarized and discussed this recent evidence to inform the best supportive strategy in AHRF both related and unrelated to COVID-19.

Correlation between Pulmonary Artery Pressure and Vortex Duration Determined by 4D Flow MRI in Main Pulmonary Artery in Patients with Suspicion of Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the causes of pulmonary hypertension (PH) and requires invasive measurement of the mean pulmonary artery pressure (mPAP) during right heart catheterisation (RHC) for the diagnosis. 4D flow MRI could provide non-invasive parameters to estimate the mPAP. Twenty-five patients with suspected CTEPH underwent cardiac MRI. Mean vortex duration (%), pulmonary distensibility, right ventricular volumes and function were measured using 4D flow MRI and cine sequences, and compared with the mPAP measured by RHC. The mPAP measured during RHC was 33 ± 16 mmHg (10−66 mmHg). PH (defined as mPAP > 20 mmHg) was present in 19 of 25 patients (76%). A vortical flow was observed in all but two patients (92%) on 4D flow images, and vortex duration showed good correlation with the mPAP (r = 0.805; p < 0.0001). Youden index analysis showed that a vortex duration of 8.6% of the cardiac cycle provided a 95% sensitivity and an 83% specificity to detect PH. Reliability for the measurement of vortex duration was excellent for both intra-observer ICC = 0.823 and inter-observer ICC = 0.788. Vortex duration could be a useful parameter to non-invasively estimate mPAP in patients with suspected CTEPH.

Non-Invasive Cardiac Output Determination Using Magnetic Resonance Imaging and Thermodilution in Pulmonary Hypertension.

Magnetic resonance imaging (MRI) can be used to measure cardiac output (CO) non-invasively, which is a paramount parameter in pulmonary hypertension (PH) patients. We retrospectively compared stroke volume (SV) obtained with MRI (SVMRI) in six localisations against SV measured with thermodilution (TD) (SVTD) and against each other in 24 patients evaluated in our PH centre using Bland and Altman (BA) agreement analyses, linear correlation, and intraclass correlation (ICC). None of the six tested localisations for SVMRI reached the predetermined criteria for interchangeability with SVTD, with two standard deviations (2SD) of bias between 24.1 mL/beat and 31.1 mL/beat. The SVMRI methods yielded better agreement when compared against each other than the comparison between SVMRI and SVTD, with the best 2SD of bias being 13.8 mL/beat. The inter-observer and intra-observer ICCs for COMRI were excellent (inter-observer ICC between 0.889 and 0.983 and intra-observer ICC between 0.991 and 0.999). We could not confirm the interchangeability of SVMRI with SVTD based on the predetermined interchangeability criteria. The lack of agreement between MRI and TD might be explained because TD is less precise than previously thought. We evaluated a new method to estimate CO through the pulmonary circulation (COp) in PH patients that may be more precise than the previously tested methods.

Dysfunctional breathing diagnosed by cardiopulmonary exercise testing in 'long COVID' patients with persistent dyspnoea.

BACKGROUND: 'Long COVID'-associated dyspnoea may persist for months after SARS-CoV-2 infection. Among the causes of persistent dyspnoea, dysfunctional breathing (DB), defined as an erratic or inappropriate ventilation at rest or exercise, has been observed, but little is known about its occurrence and pathophysiology among individuals with 'long COVID'. We aimed to describe the occurrence and identify clinical predictors of DB among patients following SARS-CoV-2 infection. METHODS: Cardiopulmonary exercise testing (CPET) was performed in 51 SARS-CoV-2 patients (median age, 64 years (IQR, 15)); male, 66.7%) living with 'long COVID' and persistent dyspnoea. CPET was classified into three dominant patterns: respiratory limitation with gas exchange abnormalities (RL); normal CPET or O2 delivery/utilisation impairment (D); and DB. Non-parametric and χ2 tests were applied to analyse the association between CPET dominant patterns and demographics, pulmonary function tests and SARS-CoV-2 severity. RESULTS: Among 51 patients, DB mostly without hyperventilation was found in 29.4% (n=15), RL in 54.9% (n=28) and D in 15.7% (n=8). When compared with RL individuals, patients with DB were younger, had significantly less severe initial infection, a better transfer capacity for carbon monoxide (median 85% (IQR, 28)), higher oxygen consumption (22.9 mL/min/kg (IQR, 5.5)), a better ventilatory efficiency slope (31.6 (IQR, 12.8)), and a higher SpO2 (95% (IQR, 3)). CONCLUSIONS: Our findings suggest that DB without hyperventilation could be an important pathophysiological mechanism of disabling dyspnoea in younger outpatients following SARS-CoV-2 infection, which appears to be a feature of COVID-19 not described in other viral diseases.

Cardiac Output Determination in Precapillary Pulmonary Hypertension: A Systematic Review.

BACKGROUND: Cardiac output determination is essential in precapillary pulmonary hypertension. While direct Fick is the gold standard, thermodilution is commonly used as the reference method. Moving to noninvasive methods would be highly beneficial for patients, avoiding repetitive invasive assessments. This systematic review followed 3 objectives: (1) assessing the validity of indirect Fick and thermodilution in precapillary pulmonary hypertension, (2) assessing the interchangeability of noninvasive cardiac output measurement methods against reference methods in precapillary pulmonary hypertension, and (3) detecting methodological heterogeneity in the included studies. METHODS: We systematically reviewed the literature using medical databases and following PRISMA guidelines. We included articles comparing an invasive or noninvasive cardiac output measurement method with thermodilution or direct Fick in precapillary pulmonary hypertension patients. Cutoffs of limits of agreement and percentage error derived from the Bland and Altman graph were used to accept interchangeability. To study methodological heterogeneity, we extracted 9 quality criteria from all studies. RESULTS: Eleven studies were included. None reached the suggested interchangeability criteria. The median number of the 9 assessed quality criteria was 2 with interquartile range (0-4). CONCLUSIONS: Further studies evaluating the reliability of thermodilution and the consequences of its use in precapillary pulmonary hypertension patients are necessary. No evidence supports the use of indirect Fick in precapillary pulmonary hypertension. The studied noninvasive methods could not be considered interchangeable with invasive methods. A robust methodology should be used to draw sensible conclusions.

[Diagnostic and therapeutic management of medium and long-term sequelae of SARS-CoV-2 infection]. / Prise en charge diagnostique et thérapeutique des séquelles à moyen et long termes liées à l'infection à SARS-CoV-2.

Somatic or psychological sequelae after a SARS-CoV-2 infection are common. Specific organ damage should be investigated to explain persistent symptomatology and propose a treatment. A specialized consultation for the follow-up of patients after a SARS-CoV-2 infection is useful to clinically assess the patient, organized further investigations, offer treatment options and refer the patient to other specialists or to a rehabilitation program. Such a consultation is also intended to reduce the public health burden of long Covid and to collect data that can improve our management in the future.

Les séquelles somatiques ou psychologiques après une infection à SARS-CoV-2 sont fréquentes. Des atteintes d'organes spécifiques doivent être recherchées pour expliquer une symptomatologie persistante et proposer un traitement. Une consultation spécialisée pour le suivi des patients après une infection à SARS-CoV-2 est utile pour évaluer cliniquement le patient, organiser les examens complémentaires, offrir des options de traitements et orienter le patient vers d'autres spécialistes ou un programme de réhabilitation. Une telle consultation a également pour objectif de diminuer le fardeau du Covid long sur la santé publique et de collecter les données qui pourront améliorer notre prise en charge dans le futur.

Selexipag for the treatment of pulmonary arterial hypertension.

INTRODUCTION: : Pulmonary arterial hypertension (PAH) is a rare pulmonary vasculopathy. This review focuses on selexipag, a prostacyclin receptor agonist validated for the treatment of PAH. AREAS COVERED: We review the structure, mechanisms of action, pharmacokinetics, and pharmacodynamics of selexipag. Clinical efficacy and tolerability are discussed using the main clinical trial published for selexipag (GRIPHON) and its post-hoc analysis. EXPERT OPINION: Selexipag should be added as a triple oral combination therapy in case of insufficient response to oral combination therapy with endothelin receptor antagonist and phosphodiesterase 5 inhibitor. However, selexipag should not replace parenteral prostacyclin in high-risk patients.

[E-Cigarette associated lung injury]. / Pneumopathies associées au vapotage.

Vaping associated lung injury is defined by a compatible clinical and radiological picture in a patient who smoked an electronic cigarette in the previous 90 days and after exclusion of other conditions, -notably infections. The severity varies from mild symptoms to -hypoxemic respiratory failure eventually leading to death. The clinical presentation includes general, respiratory and gastrointestinal symptoms. Laboratory findings show leukocytosis with elevated -inflammatory markers. Radiological features consist of bilateral ground-glass opacities with or without consolidation. Broncho-alveolar lavage can be used to refine the diagnosis and exclude an infection. Corticosteroids are at the center of therapy. Antibiotics are often given because of the initial suspicion of infection.

La pneumopathie liée au vapotage est définie par un tableau ­clinique et radiologique compatible chez un sujet ayant inhalé de la vapeur de cigarette électronique dans les 90 jours, après exclusion des diagnostics différentiels. La sévérité est variable allant d'une atteinte discrète à l'insuffisance respiratoire hypoxémique pouvant conduire au décès. La clinique associe des symptômes généraux, respiratoires et gastro-intestinaux. Le laboratoire montre une leucocytose avec une élévation des marqueurs ­inflammatoires. L'imagerie thoracique révèle des infiltrats bila­téraux en verre dépoli avec ou sans condensation. Le lavage ­bronchoalvéolaire peut être utilisé pour préciser le diagnostic et exclure une infection. Les corticostéroïdes constituent le centre du traitement. Des antibiotiques sont souvent administrés en suspectant initialement une atteinte infectieuse.