National Pulmonary Hypertension Service, Royal Brompton Hospital, London, UK.
Adult Congenital Heart Disease Service, Royal Brompton Hospital, London, UK.
Thorax. 2017 Nov;72(11):1035-1045. doi: 10.1136/thoraxjnl-2016-209725. Epub 2017 Sep 12.
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.
治疗肺动脉高压(PAH)患者的急性急症具有挑战性。在英国和爱尔兰,成人 PAH 患者的管理集中在 8 个全国指定的肺动脉高压(PH)中心。然而,许多患者居住在远离这些中心的地方,当地医院的医生通常需要管理 PAH 急症。来自全国指定 PH 中心的医生委员会确定了 PAH 患者中遇到的“最常见”紧急临床情况。此后,针对这些特定主题进行了文献回顾,并根据最佳现有证据和专家共识制定了管理方法。制定了以下 PAH 急症的管理方案:胸痛(包括心肌缺血)、右心衰竭、心律失常、败血症、咯血(“CRASH”),以及与手术、麻醉和妊娠相关的考虑因素。PAH 急症并不少见。虽然 PAH 管理方面的专业知识至关重要,但所有参与急性护理的医生都应了解 PAH 急症急性管理的原则。需要采取多学科方法,来自三级 PH 中心的医生支持当地的护理,并在适当的时候计划将患者安全转移到 PH 中心。