scholarly journals The changing landscape of pulmonary arterial hypertension and implications for patient care

2014 ◽  
Vol 23 (134) ◽  
pp. 450-457 ◽  
Author(s):  
Marius M. Hoeper ◽  
J. Simon R. Gibbs
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Older Patients ◽  
Severe Disease ◽  
Western World ◽  
Diffusing Capacity ◽  
Younger Patients ◽  
Disease Characteristics ◽  
Pulmonary Arterial ◽  
Over Time

Registries have provided a wealth of information on the clinical and disease characteristics of patients living with pulmonary arterial hypertension (PAH) since the 1980s. Certain PAH demographics, such as the prevalence of various PAH subgroups and preponderance of female patients, appear to have remained stable over time. Contemporary registry data indicate that the average age of patients diagnosed with PAH has increased, at least in the Western world. Older patients with PAH are more likely to be diagnosed with a more advanced stage of the disease, have lower exercise capacity and present with multiple comorbidities. They also have worse survival compared with younger patients. Within the PAH population, there is also a subset of patients with a lower diffusing capacity of the lung for carbon monoxide who are generally older and display more severe disease characteristics. This review discusses the implications that the increased age of the PAH population at diagnosis has on the treatment and management of the disease, as well as the need for earlier and improved diagnosis in these patients.

scholarly journals Management of pulmonary arterial hypertension in patients aged over 65 years

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K29-K36 ◽  
Author(s):  
Olivier Sitbon ◽  
Luke Howard
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Older Patients ◽  
Clinical Symptoms ◽  
Survival Rates ◽  
Specific Treatment ◽  
Younger Patients ◽  
Expert Opinions ◽  
Literature Searches ◽  
Pulmonary Arterial

Abstract Historically, pulmonary arterial hypertension (PAH) has been considered a disease of young adults, but over the last three decades, the average age at diagnosis has increased, presenting clinicians with some unique challenges. Clinical symptoms of PAH, including shortness of breath and reduced functional capacity, are not specific for the disease and may be present in older patients because of their age or as a result of comorbid conditions. Eliminating other causes for these symptoms can delay PAH diagnosis and initiation of PAH-specific treatment compared with younger patients. Currently, there are no specific guidelines relating to PAH in older patients and existing guidelines for identifying patients at potential risk of PAH may not be appropriate for patients aged over 65 years. Even though older patients tend to be diagnosed with more advanced symptoms, and evidence suggests that they are less responsive to PAH-specific therapies, treatment is often less aggressive than in younger patients. Even after adjusting for age, survival rates remain disproportionately lower in the older vs. younger PAH populations. Specific guidelines for diagnosis and treatment of older patients with PAH are needed to improve care and outcomes in this growing population. This review aims to assess the challenges associated with diagnosing and managing PAH in older patients, based on literature searches, authors’ experiences, and expert opinions.

scholarly journals Interdependence of hypoxia and β-adrenergic receptor signaling in pulmonary arterial hypertension

2019 ◽  
Vol 317 (3) ◽  
pp. L369-L380 ◽  
Author(s):  
Olivia R. Stephens ◽  
Kelly Weiss ◽  
Matthew Frimel ◽  
Jonathan A. Rose ◽  
Yu Sun ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Glucose Uptake ◽  
Adrenergic Receptor ◽  
Mononuclear Cells ◽  
Severe Disease ◽  
Metabolic Effects ◽  
High Uptake ◽  
Pulmonary Arterial

The β-adrenergic receptor (βAR) exists in an equilibrium of inactive and active conformational states, which shifts in response to different ligands and results in downstream signaling. In addition to cAMP, βAR signals to hypoxia-inducible factor 1 (HIF-1). We hypothesized that a βAR-active conformation (R**) that leads to HIF-1 is separable from the cAMP-activating conformation (R*) and that pulmonary arterial hypertension (PAH) patients with HIF-biased conformations would not respond to a cAMP agonist. We compared two cAMP agonists, isoproterenol and salbutamol, in vitro. Isoproterenol increased cAMP and HIF-1 activity, while salbutamol increased cAMP and reduced HIF-1. Hypoxia blunted agonist-stimulated cAMP, consistent with receptor equilibrium shifting toward HIF-activating conformations. Similarly, isoproterenol increased HIF-1 and erythropoiesis in mice, while salbutamol decreased erythropoiesis. βAR overexpression in cells increased glycolysis, which was blunted by HIF-1 inhibitors, suggesting increased βAR leads to increased hypoxia-metabolic effects. Because PAH is also characterized by HIF-related glycolytic shift, we dichotomized PAH patients in the Pulmonary Arterial Hypertension Treatment with Carvedilol for Heart Failure trial (NCT01586156) based on right ventricular (RV) glucose uptake to evaluate βAR ligands. Patients with high glucose uptake had more severe disease than those with low uptake. cAMP increased in response to isoproterenol in mononuclear cells from low-uptake patients but not in high-uptake patients’ cells. When patients were treated with carvedilol for 1 wk, the low-uptake group decreased RV systolic pressures and pulmonary vascular resistance, but high-uptake patients had no physiologic responses. The findings expand the paradigm of βAR activation and uncover a novel PAH subtype that might benefit from β-blockers.

Atrial septal defect closure with an Amplatzer septal occluder fenestrated with a coronary stent in a child with pulmonary arterial hypertension

2012 ◽  
Vol 23 (5) ◽  
pp. 692-696 ◽  
Author(s):  
Gregory J. Skinner ◽  
Robert M. Tulloh ◽  
Andrew J. Tometzki ◽  
Ingram Schulze-Neick ◽  
Gareth J. Morgan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arterial Pressure ◽  
Severe Disease ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Stent Insertion ◽  
Pulmonary Arterial

AbstractThe presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

Management and Outcomes of Pregnancy in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

2020 ◽  
pp. 29-37
Author(s):  
Wen Zhang ◽  
Mengyuan Yang ◽  
Mei Peng ◽  
Yanling Ding
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pregnant Women ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Severe Disease ◽  
Functional Class ◽  
Adverse Outcomes ◽  
Mother And Child ◽  
Pulmonary Arterial

Background: Pregnant women with pulmonary arterial hypertension associated with congenital heart diseases (PAH-CHD) have a high incidence of mortality and adverse outcomes for mother and child. Methods: We retrospectively examined the treatment strategies and analyzed the outcomes of pregnancy in patients with pulmonary arterial hypertension managed at a single clinical hospital from 2009 to 2018. Results: Analysis of all 102 patients with PAH-CHD in pregnancy showed that maternal and newborn death from the disease was low(<3%, 3/102) compared to rates previously reported. Although patients with mild pulmonary hypertension can deliver safely, those with moderate to severe pulmonary artery pressure (PAP), and high functional class tend to have a high risk of heart failure. Medications were selectively administered to patients with more severe disease, and it was, therefore, challenging to make a universal statement on their benefit, but they appear having some benefits in improving birth outcomes for mother and child. While some treatments such as anticoagulant therapy during pregnancy, and oxytocin after delivery, did not improve the health outcome of pregnant women but seemed to provide some benefits to the newborns. Conclusion: Our retrospective analysis of existing clinical data provides preliminary results for further studies to formally evaluate the efficacy of clinical management of patients with pulmonary arterial hypertension.

Carbon monoxide diffusing capacity and mortality in pulmonary arterial hypertension

2010 ◽  
Vol 29 (2) ◽  
pp. 181-187 ◽  
Author(s):  
Sonal Chandra ◽  
Sanjiv J. Shah ◽  
Thenappan Thenappan ◽  
Stephen L. Archer ◽  
Stuart Rich ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diffusing Capacity ◽  
Pulmonary Arterial

scholarly journals Selenium and Copper as Biomarkers for Pulmonary Arterial Hypertension in Systemic Sclerosis

Nutrients ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1894 ◽  
Author(s):  
Qian Sun ◽  
Julian Hackler ◽  
Julia Hilger ◽  
Hans Gluschke ◽  
Aldina Muric ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diagnostic Marker ◽  
Inflammatory Diseases ◽  
Severe Disease ◽  
Diagnostic Value ◽  
Protein Biomarkers ◽  
Pulmonary Arterial ◽  
Se Status

Circulating selenoprotein P (SELENOP) constitutes an established biomarker of Se status. SELENOP concentrations are reduced in inflammation and severe disease. Recently, elevated SELENOP levels have been suggested as diagnostic marker and therapeutic target in pulmonary arterial hypertension (PAH). We decided to re-evaluate this hypothesis. A group of healthy controls (n = 30) was compared with patients suffering from systemic sclerosis (SSc, n = 66), one third with SSc-related PAH. Serum was analysed for trace elements and protein biomarkers, namely SELENOP, glutathione peroxidase 3 (GPx3) and ceruloplasmin (CP). Compared to controls, patients with SSc-related PAH displayed reduced serum Se (91 ± 2 vs. 68 ± 2 µg/L) and SELENOP concentrations (3.7 ± 0.8 vs. 2.7 ± 0.9 mg/L), along with lower GPx3 activity (278 ± 40 vs. 231 ± 54 U/L). All three biomarkers of Se status were particularly low in patients with skin involvement. Serum Cu was not different between the groups, but patients with SSc-related PAH showed elevated ratios of Cu/Se and CP/SELENOP as compared to controls. Our data indicate that patients with SSc-related PAH are characterized by reduced Se status in combination with elevated CP, in line with other inflammatory diseases. Further analyses are needed to verify the diagnostic value of these TE-related biomarkers in PAH.

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