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Semin Respir Crit Care Med 2023; 44(06): 777-796
DOI: 10.1055/s-0043-1770120
Review Article

Medical Emergencies in Pulmonary Hypertension

Samuel Seitler
1   National Pulmonary Hypertension Service, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
,
Konstantinos Dimopoulos
1   National Pulmonary Hypertension Service, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
2   National Heart and Lung Institute, Imperial College London, London, United Kingdom
3   Adult Congenital Heart Disease Service, Royal Brompton Hospital, London, United Kingdom
,
Sabine Ernst
3   Adult Congenital Heart Disease Service, Royal Brompton Hospital, London, United Kingdom
,
Laura C. Price
1   National Pulmonary Hypertension Service, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
2   National Heart and Lung Institute, Imperial College London, London, United Kingdom
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Abstract

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.

Keywords

pulmonary hypertension - emergency - pulmonary embolism - arrhythmia - sepsis - hemoptysis


Publication History

Article published online:
18 August 2023

© 2023. Thieme. All rights reserved.

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