The management of acute medical emergencies in patients with pulmonary hypertension (PH) can be challenging. Patients with preexisting PH can rapidly deteriorate due to right ventricular decompensation when faced with acute physiological challenges that would usually be considered low-risk scenarios. This review considers the assessment and management of acute medical emergencies in patients with PH, encompassing both pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), acknowledging these comprise the more severe groups of PH. Management protocols are described in a systems-based approach. Respiratory emergencies include pulmonary embolism, airways disease, and pneumonia; cardiac emergencies including arrhythmia and chest pain with acute myocardial infarction are discussed, alongside PH-specific emergencies such as pulmonary artery dissection and extrinsic coronary artery compression by a dilated proximal pulmonary artery. Other emergencies including sepsis, severe gastroenteritis with dehydration, syncope, and liver failure are also considered. We propose management recommendations for medical emergencies based on available evidence, international guidelines, and expert consensus. We aim to provide advice to the specialist alongside the generalist, and emergency doctors, nurses, and acute physicians in nonspecialist centers. A multidisciplinary team approach is essential in the management of patients with PH, and communication with local and specialist PH centers is paramount. Close hemodynamic monitoring during medical emergencies in patients with preexisting PH is vital, with early referral to critical care recommended given the frequent deterioration and high mortality in this setting.

肺动脉高压（PH）患者的急性医疗紧急情况的处理可能具有挑战性。当面临通常被认为是低风险情况的急性生理挑战时，先前存在PH的患者可能会因右心室代偿失调而迅速恶化。本综述考虑了肺动脉高压患者急性医疗紧急情况的评估和管理，包括肺动脉高压（PAH）和慢性血栓栓塞性肺动脉高压（CTEPH），承认这些是更严重的肺动脉高压组。管理协议以基于系统的方法进行描述。呼吸系统急症包括肺栓塞、气道疾病和肺炎；讨论了心脏急症，包括心律失常和急性心肌梗塞引起的胸痛，以及肺动脉高压特定的急症，例如肺动脉夹层和扩张的近端肺动脉造成的外源性冠状动脉压迫。还考虑其他紧急情况，包括败血症、严重胃肠炎伴脱水、晕厥和肝功能衰竭。我们根据现有证据、国际指南和专家共识提出医疗紧急情况的管理建议。我们的目标是为非专科中心的专科医生、全科医生、急诊医生、护士和急症医生提供建议。多学科团队的方法对于 PH 患者的管理至关重要，与当地和专科 PH 中心的沟通至关重要。在医疗紧急情况下，对已有肺动脉高压的患者进行密切的血流动力学监测至关重要，鉴于这种情况下病情频繁恶化且死亡率高，建议尽早转诊至重症监护室。