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Journal of Investigative Medicine : the... Apr 2020Pulmonary hypertension (PH) is a chronic, complex and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial hypertension.... (Review)
Review
Pulmonary hypertension (PH) is a chronic, complex and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial hypertension. Selected review of the literature was conducted incorporating the European Society of Cardiology/European Respiratory Society 2015 guidelines and recommendations from the Sixth World Symposium on Pulmonary Hypertension. PH is classified into five groups based on WHO classification. Echocardiography remains the initial test of choice, and careful assessment of the right system aids in the diagnosis and prognosis of the disease. Right heart catheterization remains the gold standard of diagnosis and key guidance of treatment. Multidisciplinary approach is recommended for the care of patients with PH. Treatment selection is based on individual risk stratification of patients, and early referral to specialized PH centers improves outcomes of patients. Treating PH is complex and is best carried out in PH centers and with multidisciplinary approach. Early diagnosis and referral to those centers are key not to delay treatment.
Topics: Electrocardiography; Hemodynamics; Humans; Hypertension, Pulmonary; Prognosis; Risk Assessment; Severity of Illness Index
PubMed: 32241822
DOI: 10.1136/jim-2020-001291 -
European Respiratory Review : An... Dec 2017Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from... (Review)
Review
Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from impaired right ventricular filling and/or reduced right ventricular flow output. This clinical picture results from an imbalance between the afterload imposed on the right ventricle and its adaptation capacity. Acute decompensated pulmonary hypertension is associated with a very poor prognosis in the short term. Despite its major impact on survival, its optimal management remains very challenging for specialised centres, without specific recommendations. Identification of trigger factors, optimisation of fluid volume and pharmacological support to improve right ventricular function and perfusion pressure are the main therapeutic areas to consider in order to improve clinical condition. At the same time, specific management of pulmonary hypertension according to the aetiology is mandatory to reduce right ventricular afterload. Over the past decade, the development of extracorporeal life support in refractory right heart failure combined with urgent transplantation has probably contributed to a significant improvement in survival for selected patients. However, there remains a considerable need for further research in this field.
Topics: Acute Disease; Adaptation, Physiological; Animals; Antihypertensive Agents; Arterial Pressure; Extracorporeal Membrane Oxygenation; Heart Transplantation; Humans; Hypertension, Pulmonary; Pulmonary Artery; Risk Factors; Treatment Outcome; Ventricular Dysfunction, Right; Ventricular Function, Right; Ventricular Remodeling
PubMed: 29141964
DOI: 10.1183/16000617.0092-2017 -
Trends in Cardiovascular Medicine Feb 2022In patients with severe aortic stenosis (AS), pulmonary hypertension (PH) typically is indicative of a decompensated disease state with exhausted compensatory mechanisms... (Review)
Review
In patients with severe aortic stenosis (AS), pulmonary hypertension (PH) typically is indicative of a decompensated disease state with exhausted compensatory mechanisms of the left ventricle, meaning a heart failure state resulting from AS-related "cardiac injury". In the present review article, we discuss new insights into the pathophysiology of AS-induced PH, the prognostic impact, and potential options to prevent and treat PH in this setting. We emphasize recent data from studies focused on invasive hemodynamics in patients with severe AS that are being evaluated for aortic valve replacement, particularly the key relevance of combined pre- and post-capillary PH. This latter represents an advanced form of cardiac injury that is often associated with right ventricular dysfunction and poor prognosis. Given this context, we highlight the relevance of performing right heart catheterization in combination with non-invasive imaging for the comprehensive assessment of AS patients that are being evaluated for aortic valve replacement. Such comprehensive assessment plays a key role not only to precisely define the extent of AS-related cardiac injury but also to distinguish those PH forms that are unrelated to AS.
Topics: Aortic Valve; Aortic Valve Stenosis; Cardiac Catheterization; Heart Valve Prosthesis Implantation; Hemodynamics; Humans; Hypertension, Pulmonary
PubMed: 33346089
DOI: 10.1016/j.tcm.2020.12.005 -
Medicina (Kaunas, Lithuania) Mar 2021Pulmonary hypertension (PH) is a disease, which targets the pulmonary vasculature affecting the heart and the lungs, and is characterized by a vast array of signs and... (Review)
Review
Pulmonary hypertension (PH) is a disease, which targets the pulmonary vasculature affecting the heart and the lungs, and is characterized by a vast array of signs and symptoms. These manifestations of PH in pregnancy are highly variable and non-specific hence, it is prudent to have a very keen and high index of suspicion while evaluating these patients. This rare disease can be extremely debilitating and can be associated with a poor overall prognosis. Pregnancy in women with PH puts them at an elevated risk because the physiological changes associated with pregnancy are not well endured leading to even higher morbidity and mortality in these patients. Although there are various modalities for evaluation and workup of PH, right heart catheterization (RHC) remains the gold standard. A mean pulmonary artery pressure (PAP) of more than 20 mm of Hg is considered diagnostic. It is indeed heartening to see that in the past decade many novel therapeutic modalities have emerged and along with a better understanding of the disease process have proved to be promising in terms of reducing the adverse outcomes and preventing death in this population of patients.
Topics: Cardiac Catheterization; Female; Humans; Hypertension, Pulmonary; Lung; Pregnancy
PubMed: 33799910
DOI: 10.3390/medicina57030259 -
International Journal of Molecular... Jun 2016Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure over 25 mmHg at rest and is diagnosed by right heart catheterization. Among the different... (Review)
Review
Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure over 25 mmHg at rest and is diagnosed by right heart catheterization. Among the different groups of PH, pulmonary arterial hypertension (PAH) is characterized by a progressive obstruction of distal pulmonary arteries, related to endothelial cell dysfunction and vascular cell proliferation, which leads to an increased pulmonary vascular resistance, right ventricular hypertrophy, and right heart failure. Although the primary trigger of PAH remains unknown, oxidative stress and inflammation have been shown to play a key role in the development and progression of vascular remodeling. These factors are known to increase DNA damage that might favor the emergence of the proliferative and apoptosis-resistant phenotype observed in PAH vascular cells. High levels of DNA damage were reported to occur in PAH lungs and remodeled arteries as well as in animal models of PH. Moreover, recent studies have demonstrated that impaired DNA-response mechanisms may lead to an increased mutagen sensitivity in PAH patients. Finally, PAH was linked with decreased breast cancer 1 protein (BRCA1) and DNA topoisomerase 2-binding protein 1 (TopBP1) expression, both involved in maintaining genome integrity. This review aims to provide an overview of recent evidence of DNA damage and DNA repair deficiency and their implication in PAH pathogenesis.
Topics: Animals; DNA Damage; DNA Repair; Genes, Mitochondrial; Humans; Hypertension, Pulmonary; Oxidative Stress
PubMed: 27338373
DOI: 10.3390/ijms17060990 -
Current Cardiology Reviews 2015Pulmonary arterial hypertension (PAH) is a panvasculopathy that affects the distal pulmonary arteries and leads to restricted blood flow. This increased afterload leads... (Review)
Review
Pulmonary arterial hypertension (PAH) is a panvasculopathy that affects the distal pulmonary arteries and leads to restricted blood flow. This increased afterload leads to adaptive mechanisms of the right ventricle, with eventual failure once it can no longer compensate. Pulmonary hypertension from associated conditions, most importantly left heart disease, i.e. heart failure, can also lead to the same sequela. Patients often experience early vague symptoms of dyspnea and exercise intolerance, and thus PH can elude clinicians until right heart failure symptoms predominate. Evidence-based treatment options with pulmonary vasodilators are available for those with PAH and should be employed early. It is essential that patients be accurately categorized by their etiology of PH, as treatment strategies differ, and can potentially be dangerous if employed in the wrong clinical scenario.
Topics: Heart Failure; Heart Ventricles; Humans; Hypertension, Pulmonary; World Health Organization
PubMed: 24251459
DOI: 10.2174/1573403x09666131117164122 -
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 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 -
Cell and Tissue Research Mar 2017Pulmonary vascular remodeling is the key structural alteration in pulmonary hypertension and involves changes in the intima, media and adventitia, often with the... (Review)
Review
Pulmonary vascular remodeling is the key structural alteration in pulmonary hypertension and involves changes in the intima, media and adventitia, often with the interplay of inflammatory cells. This review examines the pathology of these changes and highlights some of the pathogenetic mechanisms that underlie the remodeling process.
Topics: Animals; Humans; Hypertension, Pulmonary; Inflammation; Vascular Remodeling
PubMed: 28025704
DOI: 10.1007/s00441-016-2539-y