Abstract
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.
Footnotes
Provenance
Publication of this peer-reviewed article was supported by Pfizer, Ltd (principal sponsor, European Respiratory Review issue 117).
Statement of Interest
M. Delcroix has received fees for serving as investigator, speaker, consultant, or steering committee member from Actelion, Aventis Pharmaceuticals, Bayer, Eli Lilly, Encysive, Gilead (Myogen), GlaxoSmithKline, Nippon Shyniaku, Novartis, Pfizer, Schering, ?and United Therapeutics; educational grants from Actelion, GlaxoSmithKline, Pfizer, and Therabel; and research grants from Actelion, Encysive, Pfizer and GlaxoSmithKline. M. Delcroix is holder of the Actelion Chair for Pulmonary Hypertension and of the GSK Chair for Research and Education in Pulmonary Vascular Pathology at the Catholic University of Leuven. R. Naeije has served on advisory boards for Actelion, United Therapeutics, Mondobiotech and LungRX, and received speaker fees from Actelion, Pfizer, Bayer HealthCare, GSK and United Therapeutics, and research grants from Actelion and Pfizer.
- Received May 19, 2010.
- Accepted July 6, 2010.
- ©ERS 2010