Introduction: Management of acute decompensated pulmonary arterial hypertension (PAH) remains challenging and may require mechanical circulatory support as bridge to urgent transplantation in selected patients. If possible, such patients should be treated at expert centres capable of providing all treatment options.

Aims: To make an inventory of acute decompensated PAH management in the European Reference Network on rare respiratory diseases (ERN-Lung)

Methods: This study was conducted by the ERS Clinical Research Collaboration PHAROS. A survey on the organization of care to manage acute decompensated PAH was sent to 49 PHAROS investigators.

Results: Thirty-nine (80%) centres from 19 countries responded to the survey. Two were paediatric centres. Thirty-five (90%) had an official designation of pulmonary hypertension expert centre. Number of admissions per year in intensive care unit (ICU) for acute decompensated PAH was <5 in 16 centres (42%), between 5 and 10 in 11 (29%), between 11 and 20 in 6 (16%) and >20 in 5 (13%). Twenty-one centres (55%) had a lung transplant program on site and 31 (84%) the possibility to use extracorporeal life support (V-A ECMO being the most widely used approach). Specific graft allocation rules to prioritize patients admitted in ICU were available in 24/37 (65%) centres. In 80% of cases, ECMO was used as a bridge to lung transplantation.

Conclusion: The number of patients admitted per year in ICU for acute decompensated PAH remains low in most of the expert centres. More than half of centres are able to offer all therapeutic options on site, including circulatory assistance and transplantation.