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Annals of Cardiac Anaesthesia 2018Perioperative management of pulmonary hypertension remains one of the most challenging scenarios during cardiac surgery. It is associated with high morbidity and... (Review)
Review
Perioperative management of pulmonary hypertension remains one of the most challenging scenarios during cardiac surgery. It is associated with high morbidity and mortality due to right ventricular failure, arrhythmias, myocardial ischemia, and intractable hypoxia. Therefore, this review article is intended toward the anesthetic considerations in the perioperative period, with particular emphasis on the selection of technique and choice of anesthesia with maintenance, anesthetic drugs, and the recent intraoperative recommendations for prevention and treatment of pulmonary hypertensive crisis.
Topics: Adult; Anesthesia; Anesthesiologists; Cardiac Surgical Procedures; Female; Humans; Hypertension, Pulmonary; Male; Perioperative Care; Pregnancy
PubMed: 29652270
DOI: 10.4103/aca.ACA_123_17 -
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 -
European Respiratory Review : An... Mar 2023Pulmonary hypertension (PH) is a prevalent disease of the pulmonary vasculature that is characterised by considerable morbidity and mortality. Substantial efforts have...
Pulmonary hypertension (PH) is a prevalent disease of the pulmonary vasculature that is characterised by considerable morbidity and mortality. Substantial efforts have been made in recent years to improve disease recognition, diagnosis and management, and this is reflected in current guidelines. The haemodynamic definition of PH has been revised and a definition for exercise PH has been provided. Risk stratification has been refined and the importance of comorbidities and phenotyping have been highlighted. These changes provide an opportunity to potentially identify pulmonary vascular disease at an earlier stage and to enhance patient-centred, goal-orientated treatment decisions. A promising fourth treatment pathway for pulmonary arterial hypertension and potential targeted therapies for group 3 PH are on the horizon, concepts which seemed inconceivable only a few years ago. Beyond medication, there is a greater appreciation for the importance of supervised training in stable PH and the possible role of interventional therapies in select cases. The landscape of PH is changing and it is characterised by progress, innovation and opportunities. In this article, we highlight some of the new trends in PH, with a specific focus on the revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and management of PH.
Topics: Humans; Hypertension, Pulmonary; Hemodynamics; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Exercise
PubMed: 36813291
DOI: 10.1183/16000617.0211-2022 -
International Journal of Molecular... Jun 2023Pulmonary hypertension (PH) associated with left heart diseases (PH-LHD), also termed group 2 PH, represents the most common form of PH. It develops through the passive... (Review)
Review
Pulmonary hypertension (PH) associated with left heart diseases (PH-LHD), also termed group 2 PH, represents the most common form of PH. It develops through the passive backward transmission of elevated left heart pressures in the setting of heart failure, either with preserved (HFpEF) or reduced (HFrEF) ejection fraction, which increases the pulsatile afterload of the right ventricle (RV) by reducing pulmonary artery (PA) compliance. In a subset of patients, progressive remodeling of the pulmonary circulation resulted in a pre-capillary phenotype of PH, with elevated pulmonary vascular resistance (PVR) further increasing the RV afterload, eventually leading to RV-PA uncoupling and RV failure. The primary therapeutic objective in PH-LHD is to reduce left-sided pressures through the appropriate use of diuretics and guideline-directed medical therapies for heart failure. When pulmonary vascular remodeling is established, targeted therapies aiming to reduce PVR are theoretically appealing. So far, such targeted therapies have mostly failed to show significant positive effects in patients with PH-LHD, in contrast to their proven efficacy in other forms of pre-capillary PH. Whether such therapies may benefit some specific subgroups of patients (HFrEF, HFpEF) with specific hemodynamic phenotypes (post- or pre-capillary PH) and various degrees of RV dysfunction still needs to be addressed.
Topics: Humans; Hypertension, Pulmonary; Heart Failure; Stroke Volume; Pulmonary Circulation; Hemodynamics
PubMed: 37373119
DOI: 10.3390/ijms24129971 -
Genes Nov 2021Pulmonary arterial hypertension (PAH) is a highly heterogeneous disorder with a complex, multifactorial aetiology [...].
Pulmonary arterial hypertension (PAH) is a highly heterogeneous disorder with a complex, multifactorial aetiology [...].
Topics: Genetic Predisposition to Disease; Humans; Hypertension, Pulmonary
PubMed: 34828405
DOI: 10.3390/genes12111798 -
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 -
European Respiratory Review : An... Dec 2016Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the... (Review)
Review
Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy.
Topics: Abortion, Therapeutic; Arterial Pressure; Female; Humans; Hypertension, Pulmonary; Maternal Mortality; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Pregnancy, High-Risk; Pulmonary Artery; Risk Assessment; Risk Factors
PubMed: 27903665
DOI: 10.1183/16000617.0079-2016 -
Journal of the American College of... May 2017Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary,... (Review)
Review
Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure. Recent advancements in PH-targeted therapies and interventional-surgical procedures have contributed to the improvement in quality of life and survival in PH/PHVD. This paper gives an update on recent developments in the diagnosis and treatment of children and young adults with PH. The focus is on the heterogeneous etiology/pathophysiology of PH in the young, and particularly on PHVD associated with congenital heart disease. Moreover, new pharmacological, surgical, and interventional therapies and their practical application in progressive/severe pulmonary arterial hypertension with inadequate response to conventional pharmacotherapy are discussed.
Topics: Child; Disease Management; Disease Progression; Heart Defects, Congenital; Heart Failure; Humans; Hypertension, Pulmonary; Infant; Quality of Life; Young Adult
PubMed: 28521893
DOI: 10.1016/j.jacc.2017.03.575 -
Anatolian Journal of Cardiology Jun 2023Cancer therapy-related pulmonary hypertension is a rare yet potentially fatal cardiotoxicity. However, it is a reversible cause of pulmonary hypertension if detected in... (Review)
Review
Cancer therapy-related pulmonary hypertension is a rare yet potentially fatal cardiotoxicity. However, it is a reversible cause of pulmonary hypertension if detected in its early stages. Cancer therapy-related pulmonary hypertension has been encountered in patients using tyrosine kinase inhibitors, particularly dasatinib. However, it is also well known that many agents used in cancer treatment such as alkylating agents, proteasome inhibitors, thoracic radiation exposure, and immune checkpoint inhibitors are particularly associated with pulmonary hypertension evolution. In case that history, symptoms, and clinical findings suggest a potential cancer therapy-related pulmonary hypertension, echocardiography is considered as the initial tool to detect pulmonary hypertension. If the possibility of pulmonary hypertension is high based on echocardiographic data, cancer treatment, as the initial step, should be discontinued due to its potential risks and other causes for pulmonary hypertension should be investigated thoroughly. Right heart catheterization should be the next step to establish the final diagnosis, and medical management, where appropriate, should be started without delay in these patients according to their pulmonary hypertension subgroup. There exists limited information regarding the diagnostic and management strategies of cancer therapy-related pulmonary hypertension in the current guidelines. In this review article, we aim to present current literature data on the mechanisms and management of cancer therapy-related pulmonary hypertension along with its follow-up algorithm in the setting of cardio-oncology practice.
Topics: Humans; Hypertension, Pulmonary; Dasatinib; Echocardiography; Cardiotoxicity; Heart; Neoplasms
PubMed: 37257013
DOI: 10.14744/AnatolJCardiol.2023.3013 -
Biomolecules Mar 2022Pulmonary hypertension (PH) is a fatal and untreatable disease, ultimately leading to right heart failure and eventually death. microRNAs are small, non-coding... (Review)
Review
Pulmonary hypertension (PH) is a fatal and untreatable disease, ultimately leading to right heart failure and eventually death. microRNAs are small, non-coding endogenous RNA molecules that can regulate gene expression and influence various biological processes. Changes in microRNA expression levels contribute to various cardiovascular disorders, and microRNAs have been shown to play a critical role in PH pathogenesis. In recent years, numerous studies have explored the role of microRNAs in PH, focusing on the expression profiles of microRNAs and their signaling pathways in pulmonary artery smooth muscle cells (PASMCs) or pulmonary artery endothelial cells (PAECs), PH models, and PH patients. Moreover, certain microRNAs, such as miR-150 and miR-26a, have been identified as good candidates of diagnosis biomarkers for PH. However, there are still several challenges for microRNAs as biomarkers, including difficulty in normalization, specificity in PH, and a lack of longitudinal and big sample-sized studies. Furthermore, microRNA target drugs are potential therapeutic agents for PH treatment, which have been demonstrated in PH models and in humans. Nonetheless, synthetic microRNA mimics or antagonists are susceptible to several common defects, such as low drug efficacy, inefficient drug delivery, potential toxicity and especially, off-target effects. Therefore, finding clinically safe and effective microRNA drugs remains a great challenge, and further breakthrough is urgently needed.
Topics: Biomarkers; Cell Proliferation; Cells, Cultured; Endothelial Cells; Humans; Hypertension, Pulmonary; MicroRNAs; Pulmonary Artery
PubMed: 35454085
DOI: 10.3390/biom12040496