Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2010 Sep;19(117):204-11.
doi: 10.1183/09059180.00004910.

Optimising the management of pulmonary arterial hypertension patients: emergency treatments

M Delcroix  1 , R Naeije
Affiliations
Review

Optimising the management of pulmonary arterial hypertension patients: emergency treatments

M Delcroix et al. Eur Respir Rev. 2010 Sep.

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.

PubMed Disclaimer

Conflict of interest statement

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.

Figures

Figure 1.
Figure 1.
Model of transmural care programme for patients with pulmonary arterial hypertension. GP: general practitioner.
Figure 2.
Figure 2.
Global heart failure in pulmonary arterial hypertension.
Figure 3.
Figure 3.
Management algorithm for patients with pulmonary hypertension in the intensive care unit (ICU). WHO: World Health Organization; RV: right ventricular; LV: left ventricular; BNP: brain natriuretic peptide; iNO: inhaled nitric oxide; PEEP: positive end-expiratory pressure; CVP: central venous pressure; Sv,O2: mixed venous oxygen saturation; PAH: pulmonary arterial hypertension; CO: cardiac output; SVR: systemic vascular resistance. Reproduced from [9] with permission from the publisher.
Figure 4.
Figure 4.
Effect of intravenous furosemide in a pulmonary arterial hypertension patient admitted with overt right heart failure documented by echo-Doppler of the heart. At base line (left panels), right ventricle is hugely dilated inducing left ventricle collapse and a restrictive mitral flow pattern with decreased E/A ratio. After acute diuresis (right panels), left ventricle can fill better and E/A ratio is restored. Reproduced from [10] with permission from the publisher.
See this image and copyright information in PMC

Comment in

References

    1. Simonneau G, Robbins IM, Beghetti M, et al. . Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–S54. - PubMed
    1. Humbert M, Sitbon O, Chaouat A, et al. . Pulmonary arterial hypertension in France: results from a National Registry. Am J Respir Crit Care Med 2006; 173: 1023–1030. - PubMed
    1. Peacock AJ, Murphy NF, McMurray JJ, et al. . An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30: 104–109. - PubMed
    1. McLaughlin VV, Archer SL, Badesch DB, et al. . ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009; 53: 1573–1619. - PubMed
    1. Galie N, Hoeper MM, Humbert M, et al. . Guidelines for the diagnosis and treatment of pulmonary hypertension. The task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Respir J 2009; 34: 1219–1263. - PubMed

MeSH terms