Athénaïs Boucly

Beyond Heritable PAH: Pulmonary Hypertension in Genetic Syndromes

Julien Grynblat, Florence Coulet, Thomas Lacoste-Palasset, Xavier Jaïs et autres

Pulmonary hypertension (PH) may complicate a broad range of genetic syndromes beyond the established spectrum of heritable pulmonary arterial hypertension. Although these conditions are individually rare, together they represent an emerging field at the crossroads of developmental biology, vascular medicine, and precision …

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European Respiratory Society clinical practice guidelines update for the treatment of pulmonary arterial hypertension

Gábor Kovács, Katarina Zeder, Marc Humbert, Robin Condliffe et autres

BACKGROUND: Pulmonary arterial hypertension (PAH) is a severe, progressive disease of the small pulmonary arteries. These guidelines provide updated evidence-based recommendations for the treatment of PAH focusing on the latest scientific evidence on sotatercept, an activin signalling inhibitor targeting a novel pathway …

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Renal function and outcomes in pulmonary arterial hypertension: a French Pulmonary Hypertension Registry study

Andrei Neagu, A. Seferian, Xavier Jaïs, Mitja Jevnikar et autres

Background Renal impairment is frequent in pulmonary arterial hypertension (PAH), yet renal function is not currently accounted for in the European Society of Cardiology (ESC)/European Respiratory Society (ERS) risk stratification tools. We investigated whether renal function provides independent prognostic information in PAH …

Placental growth factor promotes endothelial activation and inflammatory remodelling in pulmonary hypertension

My Ngoc Ha, Raphaël Thuillet, Mina Ottaviani, Corinne Normand et autres

AIMS: Pulmonary arterial hypertension (PAH) is a progressive cardiopulmonary disorder marked by pulmonary vascular remodelling and vessel loss, paradoxically occurring despite high VEGF signaling. While VEGF/VEGFR pathways are implicated in disease pathogenesis, their role in endothelial and immune cell crosstalk remains poorly …

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ERS Congress 2025: highlights from the Pulmonary Vascular Diseases Assembly

Brandon Budhram, Léon Genecand, Katarina Zeder, Andrea Baccelli et autres

https://bit.ly/425cVX6.

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Is reduced right ventricular power after sotatercept beneficial?

Léon Genecand, Athénaïs Boucly, Olivier Sitbon, D. S. Chemla et autres

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Effective Arterial Elastance Relates More Closely to Pulsatile Than Resistive Indices During Routine Hemodynamic Assessment of Inoperable CTEPH

D. S. Chemla, Xavier Jaïs, Mitja Jevnikar, Philippe Brenot et autres

Extract Increased pulmonary arterial (PA) load is a central determinant of outcome in pulmonary hypertension (PH) because of its impact on right ventricular (RV) function and RV–PA coupling [1–3]. In clinical studies, effective arterial elastance (Ea), estimated as systolic pulmonary arterial pressure …

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Prognostic factors in interstitial lung disease-associated pulmonary hypertension: data from the HYPID cohort and the French Pulmonary Hypertension Registry

Rémi Diesler, Ségolène Turquier, Martine Reynaud-Gaubert, François Lestelle et autres

BACKGROUND: Pulmonary hypertension (PH) frequently complicates interstitial lung diseases (ILDs), adversely affecting outcome. Identifying prognostic factors of patients with PH associated with ILD (ILD-PH) could facilitate early identification of patients who may benefit from PH therapy. METHODS: We included patients with ILD-PH …

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Does the method matter? Direct fick vs thermodilution in the haemodynamic assessment of pulmonary hypertension

Léon Genecand, Athénaïs Boucly, Olivier Sitbon, David Montani

Léon Genecand, MD, PhD, Athénaïs Boucly, MD, PhD, Olivier Sitbon, MD, PhD, David Montani, MD, PhD; Does the Method Matter? Direct Fick Versus Thermodilutio

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Pulmonary hypertension associated with interstitial lung disease: a state-of-the-art review

Ganesh Raghu, Sandeep Sahay, Laura C. Price, Jason Weatherald et autres

Pulmonary hypertension (PH) is a common sequela of interstitial lung diseases (ILDs) and is associated with poor prognosis and quality of life. The diagnosis and management of PH associated with ILD (ILD-PH) are challenging, due in part to the heterogeneity of ILD …

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Work–life balance as early career respiratory experts and ERS representatives: personal experiences, reflections and potential solutions for improvement

Mathieu Marillier, Athénaïs Boucly, Matteo Siciliano, Marjolein A. Heuvelmans et autres

Work-life balance is a real challenge for early career respiratory professionals striving to manage clinical responsibilities, research, teaching and professional commitments. In this collective reflection by members of the European Respiratory Society Early Career Member Committee, we openly explore our own experiences, …

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Pulmonary hypertension in patients with Noonan syndrome

Julien Grynblat, Mathieu Farges, Pascal Magro, Laurent Savale et autres

Background Noonan syndrome is a RASopathy inherited in an autosomal dominant manner, mainly caused by gain-of-function variants activating the RAS/mitogen-activated protein kinase signalling pathway. Pulmonary hypertension (PH) may occur in Noonan syndrome, but its mechanisms, clinical characteristics and outcomes remain poorly defined. …

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