Seminars in respiratory and critical care medicine
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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. ⋯ 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.
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The right ventricle plays a pivotal role in patients with pulmonary hypertension (PH). Its adaptation to pressure overload determines a patient's functional status as well as survival. In a healthy situation, the right ventricle is part of a low pressure, high compliance system. ⋯ This review will therefore describe normal RV structure and function and changes that occur during adaptation to increased afterload. Consequences of a failing right ventricle and its implications for RA function will be discussed. Subsequently, we will describe RV and RA assessment in clinical practice.
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Semin Respir Crit Care Med · Dec 2023
ReviewExtracorporeal Life Support in Pulmonary Hypertension: Practical Aspects.
Extracorporeal life support (ECLS), in particular veno-arterial extracorporeal membrane oxygenation, has emerged as a potentially life-saving treatment modality in patients presenting with pulmonary hypertension and right heart failure refractory to conventional treatment. Used mainly as a bridge to lung transplantation, ECLS is also being used occasionally as a bridge to recovery in patients with treatable causes of right heart failure. This review article describes indications, contraindications, techniques, and outcomes of the use of ECLS in patients with PH, focusing on practical aspects in the management of such patients.
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Semin Respir Crit Care Med · Dec 2023
ReviewEnd-of-Life and Palliative Care Issues for Patients Living with Pulmonary Arterial Hypertension: Barriers and Opportunities.
Pulmonary arterial hypertension (PAH) is a progressive, incurable disease that results in significant symptom burden, health care utilization, and eventually premature death. Despite the advancements made in treatment and management strategies, survival has remained poor. End-of-life care is a challenging issue in management of PAH, especially when patients are in younger age group. ⋯ Despite of the benefits of an early intervention, palliative care remains underutilized in patients with PAH, and this significantly raises issues around end-of-life care in PAH. In this review, we will discuss the opportunities offered and the existing barriers in addressing high symptom burden and end-of-life care issues. We will focus on the current evidence, identify areas for future research, and provide a call-to-action for better guidance to PAH specialists in making timely, appropriate interventions that can help mitigate end-of-life care issues.