-
Seminars in Respiratory and Critical... Dec 2023Pulmonary hypertension (PH) is a pathophysiological manifestation of a heterogeneous group of diseases characterized by abnormally elevated pulmonary arterial pressures...
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.
Topics: Humans; Hypertension, Pulmonary; Hemodynamics; Cardiac Catheterization
PubMed: 37595614
DOI: 10.1055/s-0043-1770115 -
Seminars in Respiratory and Critical... Jun 2024Systemic sclerosis is a multisystem connective tissue disease that is associated with substantial morbidity and mortality. Visceral organ involvement is common in... (Review)
Review
Systemic sclerosis is a multisystem connective tissue disease that is associated with substantial morbidity and mortality. Visceral organ involvement is common in patients with systemic sclerosis and occurs independently of skin manifestations. Pulmonary hypertension (PH) is an important and prevalent complication of systemic sclerosis. The clinical classification of PH cohorts conditions with similar pathophysiological mechanisms into one of five groups. While patients with systemic sclerosis can manifest with a spectrum of pulmonary vascular disease, notable clinical groups include group 1 pulmonary arterial hypertension (PAH) associated with connective tissues disease, PAH with features of capillary/venous involvement, group 2 PH associated with left heart disease, and group 3 PH associated with interstitial lung disease. Considerable efforts have been made to advance screening methods for PH in systemic sclerosis including the DETECT and ASIG (Australian Scleroderma Interest Group) composite algorithms. Current guidelines recommend annual assessment of the risk of PAH as early recognition may result in attenuated hemodynamic impairment and improved survival. The treatment of PAH associated with systemic sclerosis requires a multidisciplinary team including a PH specialist and a rheumatologist to optimize immunomodulatory and PAH-specific therapies. Several potential biomarkers have been identified and there are several promising PAH therapies on the horizon such as the novel fusion protein sotatercept. This chapter provides an overview of PH in systemic sclerosis, with a specific focus on group 1 PAH.
Topics: Humans; Scleroderma, Systemic; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Lung Diseases, Interstitial; Biomarkers; Practice Guidelines as Topic
PubMed: 38531379
DOI: 10.1055/s-0044-1782607 -
European Respiratory Review : An... Dec 2023Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared... (Review)
Review
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared pathophysiological and clinical features. In this paper, the first of a series covering all five PH classification groups, the clinical, radiological and pathological features of pulmonary arterial hypertension (PAH) will be reviewed. PAH may develop in the presence of associated medical conditions or a family history, following exposure to certain medications or drugs, or may be idiopathic in nature. Although all forms of PAH share common histopathological features, the presence of certain pulmonary arterial abnormalities, such as plexiform lesions, and extent of co-existing pulmonary venous involvement differs between the different subgroups. Radiological investigations are key to diagnosing the correct form of PH and a systematic approach to interpretation, especially of computed tomography, is essential.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Familial Primary Pulmonary Hypertension; Pulmonary Artery
PubMed: 38123231
DOI: 10.1183/16000617.0138-2023 -
The American Journal of Cardiology Aug 2023
Topics: Humans; Hypertension, Pulmonary; Pulmonary Embolism; Hypertension
PubMed: 37321959
DOI: 10.1016/j.amjcard.2023.05.035 -
Respiratory Medicine and Research Nov 2023COVID-19 remains a health care concern despite the end of the pandemic. Patients with cardiovascular disease (CVD) are at a higher risk for developing severe COVID-19... (Review)
Review
COVID-19 remains a health care concern despite the end of the pandemic. Patients with cardiovascular disease (CVD) are at a higher risk for developing severe COVID-19 complications. Studies investigating the COVID-19 clinical characteristics in pulmonary arterial hypertension (PAH) patients have reported discordant conclusions so far. In this review, we summarize the literature pertaining to the clinical presentation of COVID-19 in patients with PAH. In addition, we discuss common pathological aspects and disease mechanisms between PAH and COVID-19. We present an overview of the different types of PAH-approved therapy and their potential utilization as a treatment in the context of COVID-19. Moreover, we summarize the clinical trials that assessed the safety and efficiency of PAH-approved drugs in COVID-19 patients. Finally, we conclude with proposals for prospective research studies.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Prospective Studies; COVID-19; Familial Primary Pulmonary Hypertension
PubMed: 38236767
DOI: 10.1016/j.resmer.2023.101053 -
Herz Aug 2023Chronic thromboembolic pulmonary hypertension (CTEPH) is an important late sequela of pulmonary embolism and a common form of pulmonary hypertension. Currently, three... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is an important late sequela of pulmonary embolism and a common form of pulmonary hypertension. Currently, three specific treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and targeted medical therapy. The treatment decision depends mainly on the exact localization of the underlying pulmonary arterial obstructions. Pulmonary endarterectomy is the gold standard treatment of CTEPH. For inoperable patients, riociguat and treprostinil are approved. In addition, interventional therapy is recommended if appropriate target lesions are proven. Evaluation and treatment of patients with CTEPH in experienced centers are mandatory.
Topics: Humans; Hypertension, Pulmonary; Chronic Disease; Pulmonary Embolism; Pulmonary Artery; Angioplasty, Balloon
PubMed: 37186021
DOI: 10.1007/s00059-023-05172-8 -
Current Problems in Cardiology May 2024Pulmonary endarterectomy (PEA) is the first-line treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients with CTEPH are... (Review)
Review
Pulmonary endarterectomy (PEA) is the first-line treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients with CTEPH are considered inoperable, and in the last decade, balloon pulmonary angioplasty (BPA) has emerged as a viable therapeutic option for these patients with prohibitive surgical risk or recurrent pulmonary hypertension following PEA. Numerous international centers have increased their procedural volume of BPA and have reported improvements in pulmonary hemodynamics, patient functional class and right ventricular function. Randomized controlled trials have also demonstrated similar findings. Recent refinements in procedural technique, increased operator experience and advancements in procedural technology have facilitated marked reduction in the risk of complications following BPA. Current guidelines recommend BPA for patients with inoperable CTEPH and persistent pulmonary hypertension following PEA. The pulmonary arterial endothelium plays a vital role in the pathophysiologic development and progression of CTEPH.
Topics: Humans; Angioplasty, Balloon; Chronic Disease; Hypertension, Pulmonary; Pulmonary Artery; Randomized Controlled Trials as Topic
PubMed: 38401824
DOI: 10.1016/j.cpcardiol.2024.102481 -
The European Respiratory Journal Dec 2023Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well...
BACKGROUND
Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS.
METHODS
We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected.
RESULTS
44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 40 mmHg; p<0.001) and pulmonary vascular resistance (760 514 dyn·s·cm; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5-99.6%), 89.0% (95% CI 68.9-96.4%) and 67.0% (95% CI 41.4-83.3%), respectively.
CONCLUSIONS
In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.
Topics: Adult; Female; Humans; Child; Stenosis, Pulmonary Artery; Hypertension, Pulmonary; Constriction, Pathologic; Pulmonary Artery; Familial Primary Pulmonary Hypertension; Pulmonary Arterial Hypertension
PubMed: 38061784
DOI: 10.1183/13993003.00763-2023 -
Archivos de Bronconeumologia Oct 2023
Topics: Humans; Pulmonary Arterial Hypertension; Prognosis; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary; Risk Assessment
PubMed: 37147267
DOI: 10.1016/j.arbres.2023.04.003 -
British Journal of Hospital Medicine... Aug 2023Post-thrombotic syndrome and chronic thromboembolic pulmonary hypertension are two distinct clinical syndromes associated with adverse patient outcomes following a... (Review)
Review
Post-thrombotic syndrome and chronic thromboembolic pulmonary hypertension are two distinct clinical syndromes associated with adverse patient outcomes following a venous thromboembolism. Clinical manifestations of post-thrombotic syndrome include persistent pain, swelling and ultimately venous ulceration following a deep venous thrombosis. Patients experiencing chronic thromboembolic pulmonary hypertension may have symptoms ranging from exertional dyspnoea to overt right heart failure. From a physician's perspective, the most effective preventative strategy is good quality anticoagulation for prophylaxis of primary and secondary venous thromboembolism. The treatment of post-thrombotic syndrome mainly involves lifestyle modifications alongside the use of elastic compression stockings while patients with chronic thromboembolic pulmonary hypertension should be offered targeted surgical and medical treatment options available at expert centres. Further research is warranted for both conditions to determine the role of direct oral anticoagulants when used with a preventive or therapeutic intent.
Topics: Humans; Hypertension, Pulmonary; Venous Thromboembolism; Dyspnea; Heart Failure; Life Style
PubMed: 37646549
DOI: 10.12968/hmed.2023.0114