scholarly journals Sex Differences in Pulmonary Hypertension

2021 ◽  
Vol 2 ◽  
Author(s):  
Juan José Rodriguez-Arias ◽  
Ana García-Álvarez
Keyword(s):  
Pulmonary Hypertension ◽  
Sex Differences ◽  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Gender Bias ◽  
Response To Treatment ◽  
Experimental Animal Models ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) includes multiple diseases that share as common characteristic an elevated pulmonary artery pressure and right ventricular involvement. Sex differences are observed in practically all causes of PH. The most studied type is pulmonary arterial hypertension (PAH) which presents a gender bias regarding its prevalence, prognosis, and response to treatment. Although this disease is more frequent in women, once affected they present a better prognosis compared to men. Even if estrogens seem to be the key to understand these differences, animal models have shown contradictory results leading to the birth of the estrogen paradox. In this review we will summarize the evidence regarding sex differences in experimental animal models and, very specially, in patients suffering from PAH or PH from other etiologies.

Pulmonary Hypertension

2013 ◽  
Author(s):  
George K Istaphanous ◽  
Andreas W Loepke
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Disease Process ◽  
Underlying Disease ◽  
Artery Pressure ◽  
Pulmonary Arterial ◽  
Made In

Pediatric pulmonary arterial hypertension (PAH) is characterized by a pathologically elevated pulmonary artery pressure in children. The etiology of PAH is multifactorial, and while its prognosis is closely related to the reversibility of the underlying disease process, much progress has recently been made in its diagnosis and treatment, significantly decreasing the associated morbidity and mortality.

Abstract 16770: The Epigenetic Modifier EP300: A New Corner Stone in the Regulation of Both Vascular Remodeling and Right Ventricle Failure in Pulmonary Arterial Hypertension

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Alice Bourgeois ◽  
Sarah-Eve Lemay ◽  
Yann Grobs ◽  
Charlotte romanet ◽  
Junichi Omura ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Vascular Remodeling ◽  
Annexin V ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Pulmonary Arterial

Introduction: Pulmonary Arterial Hypertension (PAH) is characterized by excessive proliferation and resistance to apoptosis of pulmonary artery (PA) smooth muscle cells (PASMCs), leading to progressive increases in pulmonary vascular resistance, and ultimately right ventricular (RV) failure and death. Thanks to omics technologies, we made tremendous progress in understanding gene misregulation during disease processes and identified the epigenetic factor EP300 as a critical player in pathological processes like proliferation/apoptosis and hypertrophy/fibrosis all of which are critical features of both PA remodeling and RV failure in PAH. We hypothesized that EP300 is upregulated in PAH and contributes to both PA remodeling and RV failure. Methods and Results: By Western blot (WB) and immunofluorescence (IF), we found that EP300 is up-regulated in isolated PASMCs and distal PAs from PAH patients (n=11-14) compared to controls (n=8-10) (p<0.01). Similar results were observed in 3 PAH animal models, namely the monocrotaline (MCT), the Sugen/Hypoxia (Su/Hx) and the Fawn-Hooded rat (FHR) (p<0.05). In vitro, pharmacological inhibition of EP300 using CCS-1477 reduces PAH-PASMC proliferation (Ki67 labeling & WB PCNA; p<0.05) and resistance to apoptosis (Annexin V assay & WB Survivin; p<0.05). These effects were confirmed at the molecular level by RNA-Seq analysis. In addition, increased EP300 expression was observed in hypertrophied and failed RV from PAH patients, as well as in rats injected with MCT or subjected to pulmonary artery banding (WB, p<0.05). In animal models, EP300 negatively correlates with CO and positively correlates with RVEDP, cardiomyocyte surface area and fibrosis. Finally, we demonstrated that inhibition of EP300 using CCS-1477 or SGC-CBP30 significantly improved established PAH (right heart catheterization) in two animal models (MCT and FHR). Conclusion: EP300 upregulation contributes to both pulmonary vascular remodeling and RV dysfunction seen in PAH and its inhibition represents a promising therapeutic avenue.

scholarly journals Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Blood ◽  
2016 ◽  
Vol 127 (7) ◽  
pp. 820-828 ◽  
Author(s):  
Victor R. Gordeuk ◽  
Oswaldo L. Castro ◽  
Roberto F. Machado
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Cell Disease ◽  
Pulmonary Pressure ◽  
Pulmonary Arterial

Abstract Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.

Myocardial edema, left ventricular function, and pulmonary hypertension

1995 ◽  
Vol 78 (1) ◽  
pp. 132-137 ◽  
Author(s):  
K. L. Davis ◽  
U. Mehlhorn ◽  
G. A. Laine ◽  
S. J. Allen
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Left Ventricular Dysfunction ◽  
Ventricular Dysfunction ◽  
Dry Weight ◽  
Left Ventricular ◽  
Interstitial Edema ◽  
Pulmonary Arterial

Left ventricular dysfunction has been reported in both experimentally induced and clinical pulmonary hypertension. However, the mechanism by which pulmonary hypertension causes left ventricular dysfunction is unknown. We hypothesized that acute pulmonary hypertension causes left ventricular myocardial interstitial edema and that it is this edema that causes left ventricular dysfunction. In pulmonary artery-banded or sham-operated dogs, left ventricular diameter (septal-free wall axis) and pressure were measured using sonomicrometry crystals and a micromanometer, respectively. These measurements were used to calculate preload recruitable stroke work (PRSW), an index of contractility, and the rate of active relaxation (tau) to assess systolic and diastolic left ventricular function, respectively. After 3 h of pulmonary arterial hypertension or control, the dogs were killed and the left ventricles were excised to determine wet-to-dry weight ratios. The wet-to-dry weight ratios were significantly higher in the pulmonary artery-banded dogs (3.57 +/- 0.12) than in the sham-operated dogs (3.41 +/- 0.17). PRSW decreased to 56.8 +/- 30.3% of control after 3 h of pulmonary hypertension. tau Slowed significantly from 29.8 +/- 5.8 ms at baseline to 63.6 +/- 30.4 ms after 3 h of pulmonary arterial hypertension. There were no differences in PRSW or tau in the sham-operated dogs. We conclude that pulmonary hypertension causes left ventricular myocardial interstitial edema, which results in both systolic and diastolic left ventricular dysfunction.

scholarly journals The Effects of Dexmedetomidine Prescription in Paediatric Patients With Pulmonary Hypertension Under Congenital Heart Surgery

2020 ◽  
Author(s):  
Bahram Ghasemzadeh ◽  
Bahador Azizi ◽  
Simin Azemati ◽  
Mostafa Bagherinasab
Keyword(s):  
Blood Pressure ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Systolic Blood Pressure ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Anesthetic Care ◽  
Pulmonary Arterial

Anesthetized patient management for pediatric patients with pulmonary arterial hypertension (PAH) is a major challenge. The aim of this study was to evaluate the ability of dexmedetomidine to reduce pulmonary arterial hypertension in patients with pulmonary arterial hypertension undergoing cardiac surgery. Sixty-six patients with pulmonary arterial hypertension underwent the study. Patients were randomly divided into two groups: group D received a dexmedetomidine injection in a dose of 1 μg/kg in the first hour and then decreased to 0.5 μg/kg/hr, injection continued after surgery until extubation in the post-anesthetic care unit (PACU). Group C received normal saline 0.9% in a similar volume. Pulmonary artery systolic pressure (PASP) and systemic systolic blood pressure (SSBP) were recorded during and after the surgery in the postanesthetic care unit. Needing vasodilators, sedatives, extubation time, and the length of ICU stay were recorded for all patients. Patients in the dexmedetomidine group showed a significant reduction in Pulmonary artery systolic pressure and Pulmonary artery systolic pressure/systemic systolic blood pressure rates during surgery and during the first 24 hours in the post-anesthetic care unit (P<0.001). The dexmedetomidine group, in comparison with the control group, needed a significantly lower dose of a vasodilator (P<0.001) and a lower dose of sedation (P<0.001). It is concluded that the use of dexmedetomidine during the surgery in children with pulmonary hypertension reduces pulmonary artery systolic pressure during and after the surgery.

scholarly journals Improvements in French risk stratification score were correlated with reductions in mean pulmonary artery pressure in pulmonary arterial hypertension: a subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuichi Tamura ◽  
◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract Background Since there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort. Methods We enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements. Results The ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p = 0.03) and with the improvements in mPAP (p < 0.001). Conclusion The improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Improvements in French Risk Stratification Score were Correlated with Reductions in Mean Pulmonary Artery Pressure in Pulmonary Arterial Hypertension: A Subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2020 ◽  
Author(s):  
Yuichi Tamura ◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
Hiroaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract BackgroundSince there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.MethodsWe enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements.ResultsThe ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p=0.03) and with the improvements in mPAP (p<0.001).ConclusionThe improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Severe pulmonary hypertension in lung disease: phenotypes and response to treatment

2015 ◽  
Vol 46 (5) ◽  
pp. 1378-1389 ◽  
Author(s):  
Melanie J. Brewis ◽  
Alistair C. Church ◽  
Martin K. Johnson ◽  
Andrew J. Peacock
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Functional Class ◽  
World Health ◽  
Response To Treatment ◽  
Walking Distance ◽  
Vasodilator Therapy ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) due to lung disease (World Health Organization (WHO) group 3) is common, but severe PH, arbitrarily defined as mean pulmonary artery pressure ≥35 mmHg is reported in only a small proportion. Whether these should be treated as patients in WHO group 1 (i.e.pulmonary arterial hypertension) with PH-targeted therapies is unknown.We compared the phenotypic characteristics and outcomes of 118 incident patients with severe PH and lung disease with 74 idiopathic pulmonary arterial hypertension (IPAH) patients, all treated with pulmonary vasodilators.Lung disease patients were older, more hypoxaemic, and had lower gas transfer, worse New York Heart Association functional class and lower 6-min walking distance (6MWD) than IPAH patients. Poorer survival in those with lung disease was driven by the interstitial lung disease (ILD) cohort.In contrast to IPAH, where significant improvements in 6MWD and N-terminal pro-brain natruiretic peptide (NT-proBNP) occurred, PH therapy in severe PH lung disease did not lead to improvement in 6MWD or functional class, but neither was deterioration seen. NT-proBNP decreased from 2200 to 1596 pg·mL−1(p=0.015). Response varied by lung disease phenotype, with poorer outcomes in patients with ILD and emphysema with preserved forced expiratory volume in 1 s. Further study is required to investigate whether vasodilator therapy may delay disease progression in severe PH with lung disease.

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