System Maintenance Notice

Please note that maintenance work is scheduled between 4 AM and 5 AM - CET. During this time, you may experience reduced performance. Thanks for your patience.

Publications

Journal Article Review Epublish

PubMed: 42128486 DOI: 10.1183/16000617.0337-2025

Diagnostic and management dilemmas in pulmonary hypertension.

Eur Respir Rev 35(180)

Authors: Robin Condliffe, Jurjan Aman, Athénaïs Boucly, Ellis Cerrone, Etienne-Marie Jutant, Gabor Kovacs, Lilian J Meijboom, Karen M Olsson, Da-Hee Park, Andrei Seferian, Olivier Sitbon, Jean-Luc Vachiery, Anton Vonk Noordegraaf, David Montani, Esther J Nossent

Affiliations

  • Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.
  • Amsterdam UMC location Vrije Universiteit Amsterdam, Center of Expertise for Pulmonary Hypertension, Pulmonary Medicine, Amsterdam Cardiovascular Sciences Pulmonary Hypertension and Thrombosis, Amsterdam, The Netherlands.
  • Université Paris-Saclay, Inserm UMR_S 1358 (HPPIT), Department of Respiratory and Intensive Care Medicine (FHU André Cournand, ERN-LUNG), Hôpital Bicêtre (AP-HP), Le Kremlin Bicêtre, France.
  • Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.
  • Université de Poitiers, CIC-INSERM 1402, IS-ALIVE Research Group, CHU de Poitiers, Poitiers, France.
  • Department of Internal Medicine, Division of Respiratory Medicine, Lung Research Cluster, Medical University of Graz, Graz, Austria.
  • Department of Radiology and Nuclear Medicine, Amsterdam UMC location Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
  • Department for Respiratory Medicine and Infectious Diseases and German Centre of Lung Research (DZL), Hannover Medical School, Hannover, Germany.
  • Department of Cardiology, Hôpital Universitaire de Bruxelles (H.U.B.) Erasme, Brussels, Belgium.

Abstract

INTRODUCTION

Although current international guidelines provide comprehensive evidence-based guidance for the management of pulmonary hypertension, clinicians are still regularly faced with uncertainty regarding correct diagnosis and optimal treatment.

METHODS

Ten diagnostic and management dilemmas were formulated by a panel of experts in the field of pulmonary hypertension. Current evidence was reviewed and a practical approach suggested for each dilemma.

RESULTS

Diagnostic and management dilemmas discussed include pulmonary hypertension with high cardiac output, idiopathic pulmonary arterial hypertension in the presence of cardiopulmonary comorbidities, pulmonary hypertension associated with left heart disease, pulmonary hypertension and obesity, chronic thromboembolic pulmonary hypertension mimics and Group 5 pulmonary hypertension associated with sarcoidosis, fibrosing mediastinitis and neurofibromatosis type 1.

CONCLUSIONS

The suggested approaches are informed by a review of existing evidence and guidelines, as well as clinical experience.

MeSH

Hypertension, Hypertension, Pulmonary, Neurofibromatosis 1