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Journal of Nanobiotechnology Nov 2023Venous thromboembolism (VTE) is a multifactorial disease, and pulmonary hypertension (PH) is a serious condition characterized by pulmonary vascular remodeling leading... (Review)
Review
Venous thromboembolism (VTE) is a multifactorial disease, and pulmonary hypertension (PH) is a serious condition characterized by pulmonary vascular remodeling leading with increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Although VTE and PH have distinct primary etiologies, they share some pathophysiologic similarities such as dysfunctional vasculature and thrombosis. In both conditions there is solid evidence that EVs derived from a variety of cell types including platelets, monocytes, endothelial cells and smooth muscle cells contribute to vascular endothelial dysfunction, inflammation, thrombosis, cellular activation and communications. However, the roles and importance of EVs substantially differ between studies depending on experimental conditions and parent cell origins of EVs that modify the nature of their cargo. Numerous studies have confirmed that EVs contribute to the pathophysiology of VTE and PH and increased levels of various EVs in relation with the severity of VTE and PH, confirming its potential pathophysiological role and its utility as a biomarker of disease severity and as potential therapeutic targets.
Topics: Humans; Hypertension, Pulmonary; Venous Thromboembolism; Endothelial Cells; Extracellular Vesicles; Thrombosis
PubMed: 38037042
DOI: 10.1186/s12951-023-02216-3 -
Circulation Research Nov 2023
Topics: Humans; Hypertension, Pulmonary; Stroke Volume; Heart Failure; Myeloid Cells; Myeloid Progenitor Cells
PubMed: 37943948
DOI: 10.1161/CIRCRESAHA.123.323753 -
Seminars in Respiratory and Critical... Dec 2023
Topics: Humans; Hypertension, Pulmonary
PubMed: 37903490
DOI: 10.1055/s-0043-1772751 -
Clinical Therapeutics Sep 2023Pediatric pulmonary hypertension (PH) is a condition characterized by elevated pulmonary arterial pressure, which has the potential to be life-limiting. The etiology of... (Review)
Review
PURPOSE
Pediatric pulmonary hypertension (PH) is a condition characterized by elevated pulmonary arterial pressure, which has the potential to be life-limiting. The etiology of pediatric PH varies. When compared with adult cohorts, the etiology is often multifactorial, with contributions from prenatal, genetic, and developmental factors. This review aims to provide an up-to-date overview of the causes and classification of pediatric PH, describe current therapeutics in pediatric PH, and discuss upcoming and necessary research in pediatric PH.
METHODS
PubMed was searched for articles relating to pediatric pulmonary hypertension, with a particular focus on articles published within the past 10 years. Literature was reviewed for pertinent areas related to this topic.
FINDINGS
The evaluation and approach to pediatric PH are unique when compared with that of adults, in large part because of the different, often multifactorial, causes of the disease in children. Collaborative registry studies have found that the most common disease causes include developmental lung disease and subsets of pulmonary arterial hypertension, which includes genetic variants and PH associated with congenital heart disease. Treatment with PH-targeted therapies in pediatrics is often guided by extrapolation of adult data, small clinical studies in pediatrics, and/or expert consensus opinion. We review diagnostic considerations and treatment in some of the more common pediatric subpopulations of patients with PH, including developmental lung diseases, congenital heart disease, and trisomy 21.
IMPLICATIONS
The care of pediatric patients with PH requires consideration of unique pediatric-specific factors. With significant variability in disease etiology, ongoing efforts are needed to optimize treatment strategies based on disease phenotype and guide evidence-based practices.
Topics: Pregnancy; Adult; Female; Child; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Heart Defects, Congenital; Phenotype
PubMed: 37517916
DOI: 10.1016/j.clinthera.2023.07.001 -
Pediatrics in Review May 2024
Topics: Child; Humans; Hypertension, Pulmonary
PubMed: 38689108
DOI: 10.1542/pir.2023-006010 -
TLR7/8 activation induces autoimmune vasculopathy and causes severe pulmonary arterial hypertension.The European Respiratory Journal Jul 2023https://bit.ly/3P3H5V0
https://bit.ly/3P3H5V0
Topics: Humans; Pulmonary Arterial Hypertension; Toll-Like Receptor 7; Hypertension, Pulmonary; Vascular Diseases; Familial Primary Pulmonary Hypertension
PubMed: 37290790
DOI: 10.1183/13993003.00204-2023 -
Herz Aug 2023Pulmonary hypertension (PH) is a common condition in patients with left heart disease (LHD) that is highly relevant for morbidity and mortality. While post-capillary in... (Review)
Review
Pulmonary hypertension (PH) is a common condition in patients with left heart disease (LHD) that is highly relevant for morbidity and mortality. While post-capillary in nature, the pathophysiology of PH in patients with LHD (heart failure/cardiomyopathy, valvular heart disease; other: congenital/acquired) is complex, and decisions about management strategies are challenging. Recently, the updated European Society of Cardiology/European Respiratory Society guidelines on the diagnosis and treatment of PH revisited hemodynamic definitions and the sub-classification of post-capillary PH, and provided numerous new recommendations on the diagnosis and management of PH associated with various types of LHD. Here, we review several novel aspects that focus on: (a) updated hemodynamic definitions, including the distinction between isolated post-capillary PH (IpcPH) and combined post- and pre-capillary PH (CpcPH); (b) the pathogenesis of PH-LHD, considering various components contributing to PH, such as pulmonary congestion, vasoconstriction, and vascular remodeling; (c) the prognostic relevance of PH and hemodynamic markers; (d) the diagnostic approach to PH-LHD; (e) management strategies in PH-LHD, distinguishing between targeting the underlying left heart condition, the pulmonary circulation, and/or impaired right ventricular function. In conclusion, precise clinical and hemodynamic characterization and detailed phenotyping are essential for prognostication and the management of patients with PH-LHD.
Topics: Humans; Hypertension, Pulmonary; Heart Diseases; Heart Failure; Hemodynamics; Vasoconstriction
PubMed: 37289211
DOI: 10.1007/s00059-023-05189-z -
Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?Current Opinion in Pulmonary Medicine Sep 2023Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and... (Review)
Review
PURPOSE OF REVIEW
Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and treatment strategies for SSc-associated PAH (SSc-PAH) are informed by those used to treat patients with idiopathic PAH (IPAH). There are, however, important differences between these two diseases that impact diagnosis, treatment, and outcomes.
RECENT FINDINGS
Both SSc-PAH and IPAH are incompletely understood with ongoing research into the underlying cellular biology that characterize and differentiate the two diseases. Additional research seeks to improve identification among SSc patients in order to diagnose patients earlier in the course of their disease. Novel therapies specifically for SSc-PAH such as rituximab and dimethyl fumarate are under investigation.
SUMMARY
Although patients with SSc-PAH and IPAH present with similar symptoms, there are significant differences between these two forms of PAH that warrant further investigation and characterization of optimal detection strategies, treatment algorithms, and outcomes assessment.
Topics: Humans; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Scleroderma, Systemic
PubMed: 37461869
DOI: 10.1097/MCP.0000000000001001 -
European Respiratory Review : An... Sep 2023Pulmonary hypertension (PH) is a progressive disease characterised by elevated pulmonary arterial pressure and right-sided heart failure. While conventional drug... (Review)
Review
Pulmonary hypertension (PH) is a progressive disease characterised by elevated pulmonary arterial pressure and right-sided heart failure. While conventional drug therapies, including prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors, have been shown to improve the haemodynamic abnormalities of patients with PH, the 5-year mortality rate remains high. Thus, novel therapies are urgently required to prolong the survival of patients with PH. Stem cell therapies, including mesenchymal stem cells, endothelial progenitor cells and induced pluripotent stem cells, have shown therapeutic potential for the treatment of PH and clinical trials on stem cell therapies for PH are ongoing. This review aims to present the latest preclinical achievements of stem cell therapies, focusing on the therapeutic effects of clinical trials and discussing the challenges and future perspectives of large-scale applications.
Topics: Humans; Hypertension, Pulmonary; Endothelin Receptor Antagonists; Cell- and Tissue-Based Therapy
PubMed: 37758272
DOI: 10.1183/16000617.0112-2023 -
Expert Opinion on Pharmacotherapy 2023Pulmonary hypertension (PH) is a complex condition that encompasses an array of underlying disease processes and affects a diverse population of infants, including those... (Review)
Review
INTRODUCTION
Pulmonary hypertension (PH) is a complex condition that encompasses an array of underlying disease processes and affects a diverse population of infants, including those with congenital heart disease, congenital diaphragmatic hernia, persistent PH of the newborn, and those with lung disease such as bronchopulmonary dysplasia. While there are treatments available to adults with PH, limited data exists for infants, especially for the newer medications. Therapies that target the three main pathophysiologic pathways of pulmonary hypertension appear to benefit infants, but which are best for each individual disease process is unclear.
AREAS COVERED
A review of the therapies to treat pulmonary hypertension is covered in this article including the prostacyclin pathway, endothelin pathway, and the nitric oxide pathway. Other adjunctive treatments are also discussed. Findings are based on a PubMed literature search of research papers spanning 1990-2023 and a search of ongoing trials registered with clinicaltrials.gov.
EXPERT OPINION
Overall therapies seem to improve outcomes with most infants with PH. However, given the diverse population of infants with PH, it is imperative to understand the basis for the PH in individual patients and understand which therapies can be applicable. Further research into tailored therapy for the specific populations is warranted.
Topics: Humans; Infant; Infant, Newborn; Bronchopulmonary Dysplasia; Hypertension, Pulmonary; Pulmonary Arterial Hypertension
PubMed: 37707346
DOI: 10.1080/14656566.2023.2257598