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Clinics in Chest Medicine Mar 2024Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading... (Review)
Review
Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading to precapillary or postcapillary pulmonary hypertension. Sarcoidosis-associated pulmonary hypertension contributes to severe exertional dyspnea, reduced exercise capacity, and notably compromised the survival. Despite the critical functional and prognostic implications of pulmonary hypertension in sarcoidosis, there is a scarcity of specific guidelines on the management of these patients due to a lack of evidence. Hence, further research is required to identify subgroups of patients who may benefit from pulmonary arterial hypertension-targeted therapies and/or immunosuppressive therapies.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Sarcoidosis; Lung; Prognosis; Sarcoidosis, Pulmonary
PubMed: 38245366
DOI: 10.1016/j.ccm.2023.08.007 -
Obstetrics and Gynecology Nov 2019Pulmonary hypertension is a term used to describe a complex multifactorial group of conditions diagnosed by an elevated mean pulmonary artery pressure of 20 mm Hg or... (Review)
Review
Pulmonary hypertension is a term used to describe a complex multifactorial group of conditions diagnosed by an elevated mean pulmonary artery pressure of 20 mm Hg or higher on right heart catheterization. The diagnosis of pulmonary hypertension in pregnancy is important, as it is associated with high rates of maternal morbidity and mortality, even with modern management. Diagnostic testing is important for establishing the diagnosis, type, and severity of pulmonary hypertension, which in turn, dictates treatment options. Echocardiographic assessment is the first step in diagnosis and the gold standard for monitoring right heart function in patients with pulmonary hypertension. Supportive therapy for pulmonary hypertension includes monitored exercise, vaccination, and avoidance of certain activities. Therapies for pulmonary hypertension are considered conventional or targeted. Conventional therapy includes preventative care, anticoagulation, and calcium channel blockers for appropriate patients. Targeted therapy is usually reserved for patients with World Health Organization group 1 pulmonary arterial hypertension (including idiopathic, heritable, drug-induced or associated with congenital heart disease) and involves different types of direct pulmonary vasodilators. Right heart failure is the end result of pulmonary hypertension and the options for management include medical optimization, support with extracorporeal membrane oxygenation, and combined heart-lung transplantation. With pregnancy, management must be individualized, and patients should be cared for as part of an experienced multidisciplinary team. There are few studies addressing, timing and mode of delivery, including anesthetic considerations. In this review, the natural history of pulmonary hypertension in pregnancy and outcomes are summarized and current evidence-based management is discussed.
Topics: Female; Humans; Hypertension, Pulmonary; Patient Care Management; Pregnancy; Pregnancy Complications, Cardiovascular
PubMed: 31599832
DOI: 10.1097/AOG.0000000000003549 -
Nature Reviews. Disease Primers Jan 2024
Topics: Humans; Hypertension, Pulmonary; Hypertension; Lung
PubMed: 38177187
DOI: 10.1038/s41572-023-00491-w -
American Journal of Perinatology Oct 2023Persistent pulmonary hypertension of the newborn, or PPHN, represents a challenging condition associated with high morbidity and mortality. Management is complicated by...
Persistent pulmonary hypertension of the newborn, or PPHN, represents a challenging condition associated with high morbidity and mortality. Management is complicated by complex pathophysiology and limited neonatal specific evidence-based literature, leading to a lack of universal contemporary clinical guidelines for the care of these patients. To address this need and to provide consistent high-quality clinical care for this challenging population in our neonatal intensive care unit, we sought to develop a comprehensive clinical guideline for the acute stabilization and management of neonates with PPHN. Utilizing cross-disciplinary expertise and incorporating an extensive literature search to guide best practice, we present an approachable, pragmatic, and clinically relevant guide for the bedside management of acute PPHN. KEY POINTS: · PPHN is associated with several unique diagnoses; the associated pathophysiology is different for each unique diagnosis.. · PPHN is a challenging, dynamic, and labile process for which optimal care requires frequent reassessment.. · Key management goals are adequate tissue oxygen delivery, avoiding harm..
Topics: Infant, Newborn; Humans; Persistent Fetal Circulation Syndrome; Hypertension, Pulmonary; Intensive Care Units, Neonatal
PubMed: 34852367
DOI: 10.1055/a-1711-0778 -
Cardiology Clinics Feb 2022Patients with advanced lung disease can develop pulmonary hypertension and succumb to right ventricular failure/cor pulmonale. Patients with pulmonary hypertension owing... (Review)
Review
Patients with advanced lung disease can develop pulmonary hypertension and succumb to right ventricular failure/cor pulmonale. Patients with pulmonary hypertension owing to chronic lung disease, or World Health Organization group 3 pulmonary hypertension, are more limited and carry a high risk of mortality. Adjunctive therapies remain the cornerstones of treatment. Recent evidence suggests that inhaled pulmonary vasodilator therapy can be helpful in patients with pulmonary hypertension owing to interstitial lung disease. Lung transplantation may be the only life-saving option in select patients, whereas palliative care and hospice should be sought for those who are not candidates as the disease progresses.
Topics: Heart Failure; Humans; Hypertension, Pulmonary; Lung Diseases; Pulmonary Artery; Pulmonary Heart Disease
PubMed: 34809919
DOI: 10.1016/j.ccl.2021.08.005 -
Emergency Medicine Clinics of North... Aug 2022Right ventricular dysfunction is an important component of the pathophysiology of several disorders commonly encountered in the emergency department (ED). Interventions... (Review)
Review
Right ventricular dysfunction is an important component of the pathophysiology of several disorders commonly encountered in the emergency department (ED). Interventions often performed routinely early in the ED course such as fluid administration and endotracheal intubation have the potential to cause precipitous clinical deterioration in patients with right ventricular failure and pulmonary hypertension. It is important for emergency physicians to understand the pathophysiology of acute decompensated right ventricular failure in order to avoid common pitfalls in diagnosis and management that can result in significant morbidity and mortality.
Topics: Emergency Service, Hospital; Heart Failure; Humans; Hypertension, Pulmonary; Pulmonary Embolism; Ventricular Dysfunction, Right
PubMed: 35953215
DOI: 10.1016/j.emc.2022.05.006 -
International Journal of Molecular... Jan 2024Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and...
Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and respiratory diseases [...].
Topics: Humans; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary
PubMed: 38256236
DOI: 10.3390/ijms25021166 -
The Journal of the Royal College of... Dec 2021Pulmonary hypertension is a rare and complex disease that arises from a wide range of underlying conditions. Therapeutic options have expanded enormously over the last... (Review)
Review
Pulmonary hypertension is a rare and complex disease that arises from a wide range of underlying conditions. Therapeutic options have expanded enormously over the last two decades resulting in major improvements in prognosis for some patients. As a consequence, it is vital that the disease is recognised early and referred on to specialist centres for further investigation and definitive diagnosis to improve prognosis in this life-altering condition. Unfortunately, it remains the case in the UK that there is a delay in the diagnosis of pulmonary hypertension, often months or years after the onset of symptoms. This review aims to highlight key points in initial management and referral of patients with suspected pulmonary hypertension and presents three cases to underline these areas.
Topics: Humans; Hypertension, Pulmonary; Prognosis; Referral and Consultation
PubMed: 34882144
DOI: 10.4997/JRCPE.2021.419 -
Cardiology Clinics Aug 2020Pulmonary hypertension (PH) is common in adults with congenital heart disease and carries fundamental implications for management and prognosis. A high index of... (Review)
Review
Pulmonary hypertension (PH) is common in adults with congenital heart disease and carries fundamental implications for management and prognosis. A high index of suspicion, combined with knowledge of the pathogenesis and pathophysiology of PH, is required to achieve a timely, accurate diagnosis, and appropriate classification and treatment. This article provides a guide on how to approach the adult with congenital heart disease and suspected PH of different types, including current management.
Topics: Adult; Disease Management; Early Diagnosis; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Prognosis
PubMed: 32622496
DOI: 10.1016/j.ccl.2020.04.008 -
Seminars in Respiratory and Critical... Dec 2023The management of acute medical emergencies in patients with pulmonary hypertension (PH) can be challenging. Patients with preexisting PH can rapidly deteriorate due to... (Review)
Review
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.
Topics: Humans; Hypertension, Pulmonary; Emergencies; Lung; Pulmonary Embolism; Pulmonary Artery; Chronic Disease
PubMed: 37595615
DOI: 10.1055/s-0043-1770120