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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 -
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 -
Current Opinion in Cardiology Nov 2021In the past decades, the diagnostic and therapeutic management of chronic thromboembolic pulmonary hypertension (CTEPH) has been revolutionized. (Review)
Review
PURPOSE OF REVIEW
In the past decades, the diagnostic and therapeutic management of chronic thromboembolic pulmonary hypertension (CTEPH) has been revolutionized.
RECENT FINDINGS
Advances in epidemiological knowledge and follow-up studies of pulmonary embolism patients have provided more insight in the incidence and prevalence. Improved diagnostic imaging techniques allow accurate assessment of the location and extend of the thromboembolic burden in the pulmonary artery tree, which is important for the determination of the optimal treatment strategy. Next to the pulmonary endarterectomy, the newly introduced technique percutaneous pulmonary balloon angioplasty and/or P(A)H-targeted medical therapy has been shown to be beneficial in selected patients with CTEPH and might also be of importance in patients with chronic thromboembolic pulmonary vascular disease.
SUMMARY
In this era of a comprehensive approach to CTEPH with different treatment modalities, a multidisciplinary approach guides management decisions leading to optimal treatment and follow-up of patients with CTEPH.
Topics: Diagnostic Imaging; Endarterectomy; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Embolism
PubMed: 34433194
DOI: 10.1097/HCO.0000000000000907 -
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 -
The European Respiratory Journal Oct 2021Pulmonary hypertension is a fatal condition of elevated pulmonary pressures, complicated by right heart failure. Pulmonary hypertension appears in various forms; one of... (Review)
Review
Pulmonary hypertension is a fatal condition of elevated pulmonary pressures, complicated by right heart failure. Pulmonary hypertension appears in various forms; one of those is pulmonary arterial hypertension (PAH) and is particularly characterised by progressive remodelling and obstruction of the smaller pulmonary vessels. Neurohormonal imbalance in PAH patients is associated with worse prognosis and survival. In this back-to-basics article on neurohormonal modulation in PAH, we provide an overview of the pharmacological and nonpharmacological strategies that have been tested pre-clinically and clinically. The benefit of neurohormonal modulation strategies in PAH patients has been limited by lack of insight into how the neurohormonal system is changed throughout the disease and difficulties in translation from animal models to human trials. We propose that longitudinal and individual assessments of neurohormonal status are required to improve the timing and specificity of neurohormonal modulation strategies. Ongoing developments in imaging techniques such as positron emission tomography may become helpful to determine neurohormonal status in PAH patients in different disease stages and optimise individual treatment responses.
Topics: Animals; Familial Primary Pulmonary Hypertension; Heart Failure; Humans; Hypertension, Pulmonary; Prognosis; Pulmonary Arterial Hypertension
PubMed: 33766951
DOI: 10.1183/13993003.04633-2020 -
Respiratory Medicine Apr 2020Pulmonary hypertension associated with end-stage renal disease (ESRD) is an important yet under-recognized condition and can lead to life-threatening complications. The... (Review)
Review
Pulmonary hypertension associated with end-stage renal disease (ESRD) is an important yet under-recognized condition and can lead to life-threatening complications. The pathogenesis of pulmonary hypertension is peculiar in ESRD, and understanding it is important to recognize such patients at the earliest and commence appropriate treatment. Many studies have discovered the prevalence of pulmonary hypertension to be up to 80% in ESRD and have been associated with increased mortality. WHO has classified pulmonary hypertension in renal failure to be in group 5, a group defined by unclear multifactorial etiologies. Moreover, there is an improvement with renal transplant and closure of AV fistula, thus confirming the contribution from these. The pharmacological management of pulmonary hypertension in this unique population is not very different from other etiologies. However, one should understand that pulmonary hypertension as such, could be multifactorial, and other secondary causes of pulmonary hypertension should also be recognized and treated accordingly. In this article, we will discuss the concept of pulmonary hypertension in ESRD in detail and the options of treatment.
Topics: Diuretics; Exercise; Humans; Hypertension, Pulmonary; Kidney Failure, Chronic; Kidney Transplantation; Peritoneal Dialysis; Severity of Illness Index; Sleep Apnea Syndromes; Vasodilator Agents
PubMed: 32094103
DOI: 10.1016/j.rmed.2020.105905 -
Current Opinion in Cardiology Jan 2021Pulmonary arterial hypertension (PAH) causes high morbidity and mortality in children. In this review, we discuss advances in diagnosis and treatment of this disorder. (Review)
Review
PURPOSE OF REVIEW
Pulmonary arterial hypertension (PAH) causes high morbidity and mortality in children. In this review, we discuss advances in diagnosis and treatment of this disorder.
RECENT FINDINGS
Proceedings published from the 2018 World Symposium updated the definition of pulmonary hypertension to include all adults and children with mean pulmonary artery pressure more than 20 mmHg. Targeted PAH therapy is increasingly used off-label, but in 2017, bosentan became the first Food and Drug Administration-targeted PAH therapy approved for use in children.
SUMMARY
In recent years, advanced imaging and clinical monitoring have allowed improved risk stratification of pulmonary hypertension patients. New therapies, approved in adults and used off-label in pediatric patients, have led to improved outcomes for affected children.
Topics: Adult; Antihypertensive Agents; Child; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension
PubMed: 33136663
DOI: 10.1097/HCO.0000000000000822 -
Current Cardiology Reports Aug 2021Chronic thromboembolic pulmonary hypertension (CTEPH), included in group 4 PH, is an uncommon complication of acute pulmonary embolism (PE), in which emboli in the... (Review)
Review
PURPOSE OF REVIEW
Chronic thromboembolic pulmonary hypertension (CTEPH), included in group 4 PH, is an uncommon complication of acute pulmonary embolism (PE), in which emboli in the pulmonary vasculature do not resolve but rather form into an organized scar-like obstruction which can result in right ventricular (RV) failure. Here we provide an overview of current diagnosis and management of CTEPH.
RECENT FINDINGS
CTEPH management is complex with treatments that range from surgery, percutaneous interventions, to medical therapies. Current CTEPH medical therapies have largely been repurposed from pulmonary arterial hypertension (PAH). The diagnosis of CTEPH can be challenging, requiring a multimodality approach to differentiate from disease mimics. While these treatments improve symptoms, they may not reverse the underlying pathology of CTEPH.
Topics: Angioplasty, Balloon; Chronic Disease; Endarterectomy; Humans; Hypertension, Pulmonary; Pulmonary Embolism
PubMed: 34410530
DOI: 10.1007/s11886-021-01573-5 -
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 -
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