Corrigendum to "Expert management of congenital portosystemic shunts and their complications" [JHEP Reports 6 (2024)].

[This corrects the article DOI: 10.1016/j.jhepr.2023.100933.].

Expert management of congenital portosystemic shunts and their complications.

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

[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).

Exercise ventilatory response after COVID-19: comparison between ambulatory and hospitalized patients.

Inefficient ventilatory response during cardiopulmonary exercise testing (CPET) has been suggested as a cause of post-COVID-19 dyspnea. It has been described in hospitalized patients (HOSP) with lung parenchymal sequelae but also after mild infection in ambulatory patients (AMBU). We hypothesize that AMBU and HOSP have different ventilatory responses to exercise, due to different etiologies. We analyzed CPET realized between July 2020 and May 2022 of patients with persisting respiratory symptoms 3 mo after COVID-19. Chest computed tomography (CT) scan, pulmonary function tests, quality of life, and respiratory questionnaires were collected. CPET data were specifically explored as a function of ventilation (V̇e) and time. Seventy-nine consecutive patients were included (42 AMBU and 37 HOSP, median: 54 [44-60] yr old, 57% female). Patients were hospitalized for a median of 20 [8-34] days, with pneumonia (41%) or acute respiratory distress syndrome (ARDS; 30%). Among HOSP, 12(32%) patients had abnormal values for spirometry and 18(51%) for carbon monoxide diffusing capacity (P < 0.001). CPET showed no differences between AMBU and HOSP in peak absolute O2 uptake (V̇o2) (1.59 [1.22-2.11] mL·min-1; P = 0.65). Tidal volume (VT) as a function of V̇e, was lower in AMBU than in HOSP (P < 0.01) toward the end of exercise. The slope of the V̇e-CO2 production was higher than normal in both groups (30.9 [26.1-34.3]; P = 0.96). In conclusion, the severity of COVID-19 did not influence the exercise capacity, but AMBU demonstrated a less efficient ventilatory response to exercise as compared with HOSP. CPET with exploration of data as a function of V̇e and throughout the exercise better unveil ventilatory inefficiency.NEW & NOTEWORTHY We evaluated the exercise ventilatory response in patients with persisting dyspnea after severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. We found that despite similar peak power and peak absolute O2 uptake, tidal volume as a function of ventilation was lower in ambulatory than in hospitalized patients toward the end of exercise, reflecting ventilatory inefficiency. We call for evaluation of minute ventilation with the exploration of data throughout the exercise and not only peak data to better unveil ventilatory inefficiency.

Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.

Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD. Methods: This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT. Results: The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO2 slope during CPET and an increased TVR of >2.5 m/s on TTE. Conclusion: DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.

Long-term outcome of patients with combined post- and pre-capillary pulmonary hypertension.

Aims: Pulmonary hypertension (PH) is a complex clinical condition, and left heart disease is the leading cause. Little is known about the epidemiology and prognosis of combined post- and pre-capillary PH (CpcPH). Methods and results: This retrospective analysis of the Swiss PH Registry included incident patients with CpcPH registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient baseline characteristics [age, sex, mean pulmonary artery pressure (mPAP), pulmonary artery wedge pressure (PAWP), pulmonary vascular resistance (PVR), and risk factors, including World Health Organization (WHO)-functional class (FC), 6 min walk distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP), treatment, days of follow-up, and events (death or loss to follow-up) at last visit] were analysed by Kaplan-Meier and Cox regression analyses. Two hundred and thirty-one patients (59.3% women, age 65 ± 12 years, mPAP 48 ± 11 mmHg, PAWP 21 ± 5 mmHg, PVR 7.2 ± 4.8 WU) were included. Survival analyses showed a significantly longer survival for women [hazard ratio (HR) 0.58 (0.38-0.89); P = 0.01] and a higher mortality risk for mPAP > 46 mmHg [HR 1.58 (1.03-2.43); P = 0.04] but no association with age or PVR. Patients stratified to high risk according to four-strata risk assessment had an increased mortality risk compared with patients stratified to low-intermediate risk [HR 2.44 (1.23-4.84); P = 0.01]. A total of 46.8% of CpcPH patients received PH-targeted pharmacotherapy; however, PH-targeted medication was not associated with longer survival. Conclusion: Among patients with CpcPH, women and patients with an mPAP ≤46 mmHg survived longer. Furthermore, risk stratification by using non-invasively assessed risk factors, such as WHO-FC, 6MWD, and NT-proBNP, as proposed for pulmonary arterial hypertension, stratified survival in CpcPH, and might be helpful in the management of these patients.

Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives.

Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. It serves a prominent role in everyday patient care and can be determined using several validated risk assessment scores worldwide. The recently published 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines underline the importance of risk stratification not only at baseline but also during follow-up. Achieving a low-risk status has now become the therapeutic goal, emphasising the importance of personalised therapy. The application of these guidelines is also important in determining the timing for lung transplantation referral. In this review, we summarise the most relevant prognostic factors of PAH as well as the parameters used in PAH risk scores and their evolution in the guidelines over the last decade. Finally, we describe the central role that risk stratification plays in the current guidelines not only in European countries but also in Asian countries.

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.

Pulmonary Recovery 12 Months after Non-Severe and Severe COVID-19: The Prospective Swiss COVID-19 Lung Study.

BACKGROUND: Lung function impairment persists in some patients for months after acute coronavirus disease 2019 (COVID-19). Long-term lung function, radiological features, and their association remain to be clarified. OBJECTIVES: We aimed to prospectively investigate lung function and radiological abnormalities over 12 months after severe and non-severe COVID-19. METHODS: 584 patients were included in the Swiss COVID-19 lung study. We assessed lung function at 3, 6, and 12 months after acute COVID-19 and compared chest computed tomography (CT) imaging to lung functional abnormalities. RESULTS: At 12 months, diffusion capacity for carbon monoxide (DLCOcorr) was lower after severe COVID-19 compared to non-severe COVID-19 (74.9% vs. 85.2% predicted, p < 0.001). Similarly, minimal oxygen saturation on 6-min walk test and total lung capacity were lower after severe COVID-19 (89.6% vs. 92.2%, p = 0.004, respectively, 88.2% vs. 95.1% predicted, p = 0.011). The difference for forced vital capacity (91.6% vs. 96.3% predicted, p = 0.082) was not statistically significant. Between 3 and 12 months, lung function improved in both groups and differences in DLCO between non-severe and severe COVID-19 patients decreased. In patients with chest CT scans at 12 months, we observed a correlation between radiological abnormalities and reduced lung function. While the overall extent of radiological abnormalities diminished over time, the frequency of mosaic attenuation and curvilinear patterns increased. CONCLUSIONS: In this prospective cohort study, patients who had severe COVID-19 had diminished lung function over the first year compared to those after non-severe COVID-19, albeit with a greater extent of recovery in the severe disease group.

[Hypoxemia: from pathophysiology to diagnosis]. / Hypoxémie : de la physiopathologie au diagnostic.

Hypoxemia is defined as a decreased oxygen partial pressure in arterial blood. This frequent clinical phenomenon can lead to tissue hypoxia and requires a prompt diagnostic approach to guide its management. Five pathophysiological mechanisms should be assessed in the presence of hypoxemia: alveolar hypoventilation, ventilation/perfusion mismatches, diffusion disorders, true shunts and a decrease in the partial pressure of inspired oxygen. In this article, we synthesize the main etiologies of hypoxemia based on respiratory pathophysiology and suggest a diagnostic approach for its evaluation.

L'hypoxémie est définie comme la diminution de la pression partielle en oxygène dans le sang artériel. Ce phénomène fréquent en clinique peut amener à une hypoxie tissulaire et nécessite une approche diagnostique rapide afin d'orienter sa prise en charge. Cinq mécanismes physiopathologiques doivent être évoqués devant une hypoxémie : l'hypoventilation alvéolaire, les inégalités de ventilation/perfusion, les troubles de la diffusion, les shunts vrais et la diminution de la pression partielle d'oxygène inspiré. Dans cet article, nous résumons les étiologies principales d'hypoxémie en se basant sur la physiopathologie respiratoire et proposons une démarche diagnostique pour son évaluation.

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.

[COPD: Guidelines for primary care physicians]. / BPCO: recommandations pour les médecins de premiers recours.

Chronic obstructive pulmonary disease (COPD) is common and should be suspected in any patient with chronic dyspnea, cough, or sputum with a history of exposure to tobacco or harmful particles. Spirometry is used for diagnosis. Full evaluation includes the severity of obstruction and clinical data, following the Global Initiative for Chronic Obstructive Lung Disease guidelines. Although the only treatments that have an impact on mortality are tobacco cessation, pulmonary rehabilitation and, for advanced disease, oxygen therapy, new symptomatic treatment have recently been made available. The duration of antibiotic and corticosteroid treatment for exacerbations has been shortened. The new diagnostic and management recommendations are summarized in this article.

La bronchopneumopathie chronique obstructive (BPCO) est fréquente et doit être suspectée chez tout patient présentant une dyspnée, une toux ou des expectorations chroniques avec une anamnèse d'exposition au tabac ou à des particules nuisibles. La spirométrie permet son diagnostic. Son évaluation se base sur la sévérité de l'obstruction et la clinique et tient compte des recommandations du guide de la Global Initiative for Chronic Obstructive Lung Disease. Bien que les seuls traitements impactant la survie soient l'arrêt du tabac, la réhabilitation respiratoire et tardivement l'oxygénothérapie, des nouveautés sont apparues dans l'arsenal thérapeutique symptomatique. La durée du traitement antibiotique et corticostéroïde des exacerbations est raccourcie. Les nouvelles recommandations diagnostiques et de prise en charge sont résumées dans cet article.

Prognostic Implications of the Novel Pulmonary Hypertension Definition in Patients with Aortic Stenosis after Transcatheter Valve Replacement.

Introduction: Pulmonary hypertension (PH), traditionally defined as a mean pulmonary artery pressure (PAP) ≥ 25 mmHg, is associated with poor outcomes in patients undergoing a transcatheter aortic valve replacement (TAVR) for severe aortic stenosis (AS). Recently, a novel definition for PH has been proposed, placing the cut-off value of mean PAP at 20 mmHg, and introducing pulmonary vascular resistance as an exclusive indicator for the pre-capillary involvement. In light of the novel criteria, whether PH still preserves its prognostic significance remains unknown. Methods: The study population consisted of 380 patients with AS, who underwent a right heart catheterization before TAVR. The cohort was divided according to the presence of PH (n = 174, 45.7%) or not. Patients with PH were further divided into the following groups: (1) Pre-capillary PH ((Pre-capPH), n = 46, 12.1%); (2) Isolated post-capillary PH ((IpcPH), n = 78, 20.5%); (3) Combined pre and post-capillary PH ((CpcPH), n = 82, 21.6%). The primary endpoint was all-cause mortality at 1 year. Results: A total of 246 patients (64.7%) exhibited mean PAP > 20 mmHg. Overall, the presence of PH was associated with higher 1-year mortality rates (hazard ratio (HR) 2.8, 95% CI: 1.4−5.8, p = 0.004). Compared to patients with no PH, Pre-capPH and CpcPH (but not IpcPH) were related to higher 1-year mortality (HR 2.7, 95% CI: 1.0−7.2, p = 0.041 and HR 3.9, 95% CI: 1.8−8.5, p = 0.001, respectively). This remained significant even after the adjustment for baseline comorbidities. Conclusions: Pre-interventional PH according to the novel hemodynamic criteria, is linked with poor outcomes in patients undergoing TAVR for severe AS. However, this is mainly driven by patients with mean PAP ≥ 25 mmHg. Patients with a pre-capillary PH component as defined by increased PVR present an even worse prognosis as compared to patients with isolated post-capillary or no PH who present comparable 1-year mortality rates.

The pandemic toll and post-acute sequelae of SARS-CoV-2 in healthcare workers at a Swiss University Hospital.

Healthcare workers have potentially been among the most exposed to SARS-CoV-2 infection as well as the deleterious toll of the pandemic. This study has the objective to differentiate the pandemic toll from post-acute sequelae of SARS-CoV-2 infection in healthcare workers compared to the general population. The study was conducted between April and July 2021 at the Geneva University Hospitals, Switzerland. Eligible participants were all tested staff, and outpatient individuals tested for SARS-CoV-2 at the same hospital. The primary outcome was the prevalence of symptoms in healthcare workers compared to the general population, with measures of COVID-related symptoms and functional impairment, using prevalence estimates and multivariable logistic regression models. Healthcare workers (n = 3083) suffered mostly from fatigue (25.5 %), headache (10.0 %), difficulty concentrating (7.9 %), exhaustion/burnout (7.1 %), insomnia (6.2 %), myalgia (6.7 %) and arthralgia (6.3 %). Regardless of SARS-CoV-2 infection, all symptoms were significantly higher in healthcare workers than the general population (n = 3556). SARS-CoV-2 infection in healthcare workers was associated with loss or change in smell, loss or change in taste, palpitations, dyspnea, difficulty concentrating, fatigue, and headache. Functional impairment was more significant in healthcare workers compared to the general population (aOR 2.28; 1.76-2.96), with a positive association with SARS-CoV-2 infection (aOR 3.81; 2.59-5.60). Symptoms and functional impairment in healthcare workers were increased compared to the general population, and potentially related to the pandemic toll as well as post-acute sequelae of SARS-CoV-2 infection. These findings are of concern, considering the essential role of healthcare workers in caring for all patients including and beyond COVID-19.

One-year persistent symptoms and functional impairment in SARS-CoV-2 positive and negative individuals.

BACKGROUND: Persistent symptoms of SARS-CoV-2 are prevalent weeks to months following the infection. To date, it is difficult to disentangle the direct from the indirect effects of SARS-CoV-2, including lockdown, social, and economic factors. OBJECTIVE: The study aims to characterize the prevalence of symptoms, functional capacity, and quality of life at 12 months in outpatient symptomatic individuals tested positive for SARS-CoV-2 compared to individuals tested negative. METHODS: From 23 April to 27 July 2021, outpatient symptomatic individuals tested for SARS-CoV-2 at the Geneva University Hospitals were followed up 12 months after their test date. RESULTS: At 12 months, out of the 1447 participants (mean age 45.2 years, 61.2% women), 33.4% reported residual mild to moderate symptoms following SARS-CoV-2 infection compared to 6.5% in the control group. Symptoms included fatigue (16% vs. 3.1%), dyspnea (8.9% vs. 1.1%), headache (9.8% vs. 1.7%), insomnia (8.9% vs. 2.7%), and difficulty concentrating (7.4% vs. 2.5%). When compared to the control group, 30.5% of SARS-CoV-2 positive individuals reported functional impairment at 12 months versus 6.6%. SARS-CoV-2 infection was associated with the persistence of symptoms (adjusted odds ratio [aOR] 4.1; 2.60-6.83) and functional impairment (aOR 3.54; 2.16-5.80) overall, and in subgroups of women, men, individuals younger than 40 years, those between 40-59 years, and in individuals with no past medical or psychiatric history. CONCLUSION: SARS-CoV-2 infection leads to persistent symptoms over several months, including in young healthy individuals, in addition to the pandemic effects, and potentially more than other common respiratory infections. Symptoms impact functional capacity up to 12 months post infection.

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.

Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long-term PH-registry data provide information on the changing PH-epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients newly diagnosed with PH (mainly PAH/CTEPH) registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient characteristics (age, body mass index, gender, diagnosis), hemodynamics at baseline, treatment, days of follow-up, and events (death, transplantation, pulmonary endarterectomy, or loss to follow-up) at last visit were analyzed. Patients were stratified into four time periods according to their date of diagnosis. Survival was analyzed overall and separately for PAH/CTEPH and time periods. 1427 PH patients were included (thereof 560 PAH, 383 CTEPH). Over the years, age at baseline (mean ± SD) significantly increased from 59 ± 14 years in 2001-2005 to 66 ± 14 years in 2016-2019 (p < 0.001) while the gender distribution tended toward equality. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased over time (from 46 ± 15 to 41 ± 11 mmHg, respectively, 9 ± 5 to 7 ± 4 WU, p < 0.001). Three-year survival substantially increased over consecutive periods from 69% to 91% (for PAH 63%-95%, for CTEPH 86%-93%) and was poorer in PAH than CTEPH independently of time period (p < 0.001). Most patients were treated with mono- or combination therapy and an increasing number of CTEPH underwent pulmonary endarterectomy (40% 2016-2019 vs. 15% 2001-2005). This long-term PH registry reveals that over two decades of observation, newly diagnosed patients are older, less predominantly female, have less impaired hemodynamics and a better survival.

[Pulmonology 2021: year in review]. / Pneumologie.

Among the new therapeutic developments in pulmonology during 2021, we have identified three topics of interest. A new biotherapy is now available for the management of severe uncontrolled non-Th2 asthma. In the field of pulmonary arterial hypertension, upfront triple therapy at the time of diagnosis is associated with a survival benefit in high-risk patients. Riociguat is a therapeutic option for patients that remain at intermediate risk despite treatment with iPDE5. Sotatercept, a promising new class of drug for treatment of group 1 PAH will soon be available. Finally, the use of transbronchial cryobiopsies as a valid alternative to surgical lung biopsy for the diagnosis of diffuse interstitial lung diseases will also be discussed in this review.

Parmi les nouveautés thérapeutiques en pneumologie au cours de l'année 2021, nous aborderons trois sujets. Une nouvelle biothérapie est désormais disponible pour la prise en charge de l'asthme sévère non contrôlé non T-Helper 2. Concernant l'hypertension artérielle pulmonaire (HTAP), une triple thérapie d'emblée au moment du diagnostic est associée à un bénéfice sur la survie chez les patients à haut risque. Le riociguat est une option thérapeutique lors d'HTAP restant à risque intermédiaire malgré un traitement par inhibiteur de la phosphodiestérase de type 5, et le sotatercept vise une nouvelle cible thérapeutique prometteuse pour l'HTAP du groupe 1. Enfin, la place des cryobiopsies transbronchiques comme alternative valable à la biopsie chirurgicale pour le diagnostic des pneumopathies interstitielles diffuses selon des recommandations récentes se précise.