Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the ‘most common’ emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis, haemoptysis (‘CRASH'), as well as considerations relevant to surgery, anaesthesia and pregnancy. Emergencies are not uncommon in PAH. While expertise in PAH management is essential, all physicians involved in acute care should be aware of the principles of acute management of PAH emergencies. A multidisciplinary approach is necessary, with physicians from tertiary PH centres supporting care locally and planning safe transfer of patients to PH centres when appropriate.
- pulmonary hypertension
- right ventricular failure
- pulmonary hypertensive crisis
- myocardial ischaemia
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Contributors LP, KD, PM, RA, LS, JW wrote sections, and LP and KD compiled the initial draft, acquisitions, analysis, interpretation of all data. All authors revised it critically for important intellectual content. All authors oversaw final approval of the version to be published. All authors had agreement to be accountable for all aspects of the work.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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