Seminars in respiratory and critical care medicine最新文献

The clinical presentation of pulmonary hypertension (PH) is nonspecific, resulting in significant delays in its detection. In the majority of cases, PH is a marker of the severity of other cardiopulmonary diseases. Differential diagnosis aimed at the early identification of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) who do require specific and complex therapies is as important as PH detection itself. Despite all efforts aimed at the noninvasive assessment of pulmonary arterial pressure, the formal confirmation of PH still requires catheterization of the right heart and pulmonary artery. The current document will give an overview of strategies aimed at the early diagnosis of PAH and CTEPH, while avoiding their overdiagnosis. It is not intended to be a replica of the recently published European Society of Cardiology (ESC) and European Respiratory Society (ERS) Guidelines on Diagnosis and Treatment of Pulmonary Hypertension, freely available at the Web sites of both societies. While promoting guidelines' recommendations, including those on new definitions of PH, we will try to bring them closer to everyday clinical practice, benefiting from our personal experience in managing patients with suspected PH.

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.

Pulmonary hypertension (PH) is a pathophysiological manifestation of a heterogeneous group of diseases characterized by abnormally elevated pulmonary arterial pressures diagnosed on right heart catheterization. The 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) Guidelines for the diagnosis and treatment of PH provides a new hemodynamic definition to define PH by lowering the threshold of the mean pulmonary artery pressure (mPAP) to 20 mm Hg. Precapillary PH is thus now defined as a mPAP >20 mm Hg together with a normal pulmonary artery wedge pressure (<15 mm Hg) and an increased pulmonary vascular resistance (>2 Wood Units). The ESC/ERS 2022 Guidelines also introduce a revised clinical classification of PH while retaining its previous distinction between the five groups according to the underlying pathophysiology.

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. The treatment of CTEPH has long been purely surgical, but many patients were inoperable because of inaccessible lesions or severe comorbidities. Alternatives were developed, including medical therapy and more recently balloon pulmonary angioplasty (BPA). Depending on the generation of the obstructed vessels, the treatment will be surgical, up to the (sub)segmental level, or by BPA for more distal vessels. 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.

Pulmonary hypertension is characterized by right ventricular impairment and a reduced ability to compensate for hemodynamic insults. Consequently, surgery can be challenging but is increasingly considered in view of available specific therapies and improved longer term survival. Optimal management requires a multidisciplinary patient-centered approach involving surgeons, anesthetists, pulmonary hypertension clinicians, and intensivists. The optimal pathway involves risk:benefit assessment for the proposed operation, optimization of pulmonary hypertension and any comorbidities, the appropriate anesthetic approach for the specific procedure and patient, and careful monitoring and management in the postoperative period. Where patients are carefully selected and meticulously managed, good outcomes can be achieved.

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. It is built to accommodate changes in preload, but not very well suited for dealing with pressure overload. In PH, right ventricular (RV) contractility must increase to maintain cardiac output. In other words, the balance between the degree of RV contractility and afterload determines stroke volume. Hypertrophy is one of the major hallmarks of RV adaptation, but it may cause stiffening of the ventricle in addition to intrinsic changes to the RV myocardium. Ventricular filling becomes more difficult for which the right atrium tries to compensate through increased stroke work. Interaction of RV diastolic stiffness and right atrial (RA) function determines RV filling, but also causes vena cava backflow. Assessment of RV and RA function is critical in the evaluation of patient status. In recent guidelines, this is acknowledged by incorporating additional RV parameters in the risk stratification in PH. Several conventional parameters of RV and RA function have been part of risk stratification for many years. Understanding the pathophysiology of RV failure and the interactions with the pulmonary circulation and right atrium requires consideration of the unique RV anatomy. 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.

Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH), provided lesions are proximal enough in the pulmonary vasculature to be surgically accessible and the patient is well enough to benefit from the operation in the longer term. It is a major cardiothoracic operation, requiring specialized techniques and instruments developed over several decades to access and dissect out the intra-arterial fibrotic material. While in-hospital operative mortality is low (<5%), particularly in high-volume centers, careful perioperative management in the operating theater and intensive care is mandatory to balance ventricular performance, fluid balance, ventilation, and coagulation to avoid or treat complications. Reperfusion pulmonary edema, airway hemorrhage, and right ventricular failure are the most problematic complications, often requiring the use of extracorporeal membrane oxygenation to bridge to recovery. Successful PEA has been shown to improve both morbidity and mortality in large registries, with survival >70% at 10 years. For patients not suitable for PEA or with residual PH after PEA, balloon pulmonary angioplasty and/or PH medical therapy may prove beneficial. Here, we describe the indications for PEA, specific surgical and perioperative strategies, postoperative monitoring and management, and approaches for managing residual PH in the long term.

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. Renal dysfunction is often present, but other organ systems can be involved resulting in findings such as acute hepatic dysfunction or bowel wall congestion and ischemia. The goals of therapy are to rapidly reverse ventriculo-arterial decoupling and reduce right ventricular afterload to prevent progression to refractory or irreversible right heart failure. Triggering events must be investigated for and addressed urgently if identified. 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.

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. End-of-life care revolves around symptom palliation and reducing psychosocial disease burden for a dying patient and entails advanced care planning that are often challenging. Thus, support from palliative care specialist becomes extremely important in these patients. Early introduction to palliative care in patients with high symptom burden and psychosocial suffering is suggested. 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.