TY - JOUR T1 - Optimising the management of pulmonary arterial hypertension patients: emergency treatments JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 204 LP - 211 DO - 10.1183/09059180.00004910 VL - 19 IS - 117 AU - M. Delcroix AU - R. Naeije Y1 - 2010/09/01 UR - http://err.ersjournals.com/content/19/117/204.abstract N2 - Pulmonary arterial hypertension (PAH) is a rare and potentially fatal disease whose management is usually restricted to a few specialised centres. As patients do not necessarily live in the neighbourhood of these centres, daily care and emergencies have to be delegated to first and second lines. Treatment guidelines do not usually provide recommendations for acute emergency situations as evidence is scarce. This short review provides a description of our therapeutic protocols based on available data. A model of transmural organisation of care for PAH patients, currently applied in Belgium, is described. Thereafter, based on an analysis of the reasons of death in the PAH population, a review of the main emergencies is provided. Cardiac arrest and resuscitation, decompensated right heart failure, respiratory failure, arrhythmia, pericardial effusion, haemoptysis, surgery and drug-related adverse events will be discussed successively. Case reports showing the precariousness of PAH patients will enforce our thesis of the need for optimal patient management organisation. ER -