Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Dec 2023
Monographic Issue on Pulmonary Hypertension: Medical and Interventional Treatment for Chronic Thromboembolic Pulmonary Hypertension.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. The reasons why clots do not resorb are incompletely understood, but the result is partial or complete fibrothrombotic obstruction of pulmonary arteries. A secondary microvasculopathy aggravates the pulmonary hypertension (PH) as a consequence of high flow and shear stress in the nonoccluded arteries. ⋯ PH drugs are used to treat the microvasculopathy. The current paper describes the therapeutic management of inoperable patients: the medical approach with PH drugs used in mono- or combination therapy; the proper use of anticoagulants in CTEPH; the technique, indications, and results at short- and long-term of BPA; the multimodal approach for inoperable patients combining PH drugs and BPA; and the effects of rehabilitation. It shows the importance of a multidisciplinary approach to the disease.
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Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction. Admission to intensive care unit with acutely decompensated right heart failure is a significant negative prognostic event with a high risk of multisystem organ dysfunction and death. Presentations are heterogenous and may combine signs of both diastolic and systolic dysfunction complicating management. ⋯ Volume status management is critical and both noninvasive and invasive testing can aid in prognostication and guide management, including the use of inotropes and vasopressors. In cases of refractory right heart dysfunction, consideration of urgent lung transplantation and mechanical circulatory support is necessary. These patients should be managed at expert centers in an intensive care setting with a multidisciplinary team of practitioners experienced in the management of right heart dysfunction given the high short- and long-term mortality resulting from acute decompensated right heart failure.
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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.