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Examples of key factors suggestive of group 2 pulmonary hypertension 

Examples of key factors suggestive of group 2 pulmonary hypertension 

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular dia...

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... Angina, exertional dyspnoea, exhaustion, feebleness, presyncope, and syncope are all typical signs of pulmonary hypertension [14]. With progressive right ventricular failure, fluid administration needs close monitoring as it may cause abdominal distention and pedal oedema due to progressive right ventricular failure. ...
... With progressive right ventricular failure, fluid administration needs close monitoring as it may cause abdominal distention and pedal oedema due to progressive right ventricular failure. Hepatomegaly, ascites, peripheral oedema, a tricuspid regurgitant murmur, an augmented second heart sound, a third heart sound from the right ventricle, raised jugular venous pressure having an uncharacteristic waveform, low-volume arterial pulses, and left parasternal lift or retraction are all possible physical findings [14]. Because the clinical supervision of pulmonary arterial hypertension in children is frequently non-specific, diagnosing the condition may be challenging. ...
Article
Pulmonary hypertension (PH) is a complex condition that can occur as a result of a wide range of disorders, including left heart disease, lung disease, and chronic pulmonary thromboembolism. Multiple improvements have been made in the diagnosis and treatment of pulmonary arterial hypertension (PAH) including a greater understanding of the involvement of extrapulmonary vascular organ systems, validated point of care, clinical assessment tools, and a focus on the initial exposure of numerous pharmacotherapeutics in the appropriate level of care. To achieve a minimal symptom burden, improve the patient's biochemical, hemodynamic, and functional profile, and reduce adverse impact, early diagnosis of PAH is a key objective today. The preferred method of management for thromboembolic PH, which is chronic, is pulmonary endarterectomy since the majority of affected patients are operable. The timing of pulmonary endarterectomy should never be delayed for medical reasons, and risk stratification can enable us to select patients who have a high chance of success. Patients who are not qualified for endarterectomy should be referred for drug trials. Even though there are more effective ways to guarantee a sufficient, long-lasting septostomy, atrial septostomy is promising but undervalued. The procedure's indications remain the same and need to be taken into account more frequently. Class III or IV patients who are not improving need to be consulted at a transplant centre as soon as possible as they may be candidates for potential recipients of bilateral sequential lung or heart-lung transplants, which is a significant choice for some people. PH is rarely linked to other conditions like connective tissue or thromboembolic disease. It is either idiopathic or linked to congenital heart disease. Infants and children with PH are more frequently recognised in conjunction with a congenital diaphragmatic hernia and developmental lung diseases like bronchopulmonary dysplasia. Although the underlying disease has not yet been treated and advanced structural changes have not yet been reversed, the value of natural life and survival have suggestively increased. Children's haemodynamic and functional outcomes have improved as a result of endothelin receptor antagonists, prostacyclin analogues, and phosphodiesterase type 5 inhibitors, which are examples of targeted pulmonary vasodilator therapies. The health maintenance of paediatric PH is still difficult because treatment decisions are largely based on the findings of adult studies that have been supported by evidence and the clinical expertise of paediatric specialists.
... Sarcoidosis is a rare granulomatous multi-organ disease of unknown cause, most commonly affecting the lungs [1]. A well-known severe complication of sarcoidosis is pulmonary hypertension (PH), defined as a mean pulmonary artery pressure of ≥25 mm Hg (mPAP ≥ 25 mm Hg) at rest as assessed by right heart catheterization [2]. Reported prevalence rates of PH in sarcoidosis patients vary widely; ranging from 3% in a prospective cohort of predominantly white patients [3] to 74% in patients awaiting lung transplantation [4]. ...
... According to European guidelines, PH can be clinically categorized into five groups with a similar clinical presentation, pathological findings, and hemodynamics. Sarcoidosis-associated PH (SAPH) is categorized as Group 5, PH with an unclear and/or multifactorial mechanism [2]. The underlying pathophysiological mechanism and subsequent clinical phenotype of PH in sarcoidosis are diverse [6]. ...
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Background: Pulmonary hypertension (PH) is a severe complication of sarcoidosis in a minority of patients. Several genetic defects are known to cause hereditary or sporadic PH, but whether variants in PH-associated genes are also involved in sarcoidosis-associated PH (SAPH) is unknown. Methods: 40 patients with SAPH were individually matched to 40 sarcoidosis patients without PH (SA). Whole exome sequencing was performed to identify rare genetic variants in a diagnostic PH gene panel of 13 genes. Additionally, an exploratory analysis was performed to search for other genes of interest. From 572 genes biologically involved in PH pathways, genes were selected in which at least 15% of the SAPH patients and no more than 5% of patients without PH carried a rare variant. Results: In the diagnostic PH gene panel, 20 different rare variants, of which 18 cause an amino-acid substitution, were detected in 23 patients: 14 SAPH patients carried a variant, as compared to 5 SA patients without PH (p = 0.018). Most variants were of yet unknown significance. The exploratory approach yielded five genes of interest. First, the NOTCH3 gene that was previously linked to PH, and furthermore PDE6B, GUCY2F, COL5A1, and MMP21. Conclusions: The increased frequency of variants in PH genes in SAPH suggests a mechanism whereby the presence of such a genetic variant in a patient may increase risk for the development of PH in the context of pulmonary sarcoidosis. Replication and studies into the functionality of the variants are required for further understanding the pathogenesis of SAPH.
... Pulmonary arterial hypertension, PAH (Group"1) "pre-capillary" pulmonary hypertension [44,109]. Alk1/BMPRII are strong mediators in vessels homeostasis and survival; the mutation of this co-receptor, leading to its dysfunction or inactivation, provokes altered endothelial permeability and pathological remodeling, together with impaired ECs metabolism, increased local inflammation and disrupted DNA repair [110,111]. ...
Article
The endothelium is a mechanosensitive organ whose pleiotropic actions regulate vessel structure to adjust tissue perfusion. To do so, it possesses ion channels, receptor complexes, and signaling pathways responding to blood flow, whose activation will either maintain vascular integrity and quiescence or, on the contrary, remodel the vessel's structure in both health and disease. Recent studies have demonstrated the crucial role of endothelial inflammation, endothelial to mesenchymal transition (EndMT), and perturbed hemodynamics in the progression of pulmonary arterial hypertension and essential hypertension. These two distinct diseases share some common mechanistic cues, pointing towards new potential therapeutic approaches to treat them. In this review, we summarize these common mechanisms to map future drug development strategies targeting flow sensing mechanisms and vascular remodeling.
... Pulmonary hypertension (PH) is a pathological condition characterized by increased pulmonary vascular pressure. According to the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines PH is defined as mean pulmonary artery pressure (mPAP) greater than 25 mmHg at rest [1]. The diagnosis of pulmonary hypertension is based on right heart catheterization, but echocardiography is an approved tool to estimate the probability of PH. ...
... We assessed the probability of pulmonary hypertension in all patients according to the European Society of Cardiology/European Respiratory Society Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension [1]. The evaluation of pulmonary hypertension was based on Tricuspid valve regurgitation maximal Velocity (TR Vmax) and additional echocardiographic PH indicators, which were separated into three categories (A, B, and C): ...
... The observation confirms the dependence between the probability of PH and diastolic dysfunction. In some cases, echocardiographic indices of PH (for example maximal velocity of tricuspid valve regurgitation) measured in patients with LV diastolic dysfunction were abnormal but didn't meet the criteria of an echocardiographic high or moderate probability of PH according to the European Society of Cardiology/European Respiratory Society Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension [1]. It may suggest, that in the early stage of LV diastolic dysfunction we observe the right ventricular abnormalities, but we can't diagnose pulmonary hypertension. ...
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Background: Pulmonary hypertension (PH) is an independent risk factor of increased morbidity and mortality in cardiac surgery patients (CS). The most common cause underlying PH is left ventricular (LV) diastolic dysfunction. This study aimed to evaluate the echocardiographic probability of PH in patients undergoing CS and its correlation with postoperative respiratory adverse events (RAE). Methods: The echocardiographic probability of PH and its correlation with LV diastolic dysfunction was assessed in 56 consecutive adult patients who were qualified for coronary artery bypass grafting (CABG). Later, the postoperative RAE (such as pneumonia, pulmonary congestion, or hypoxemia), the length of intensive care unit (ICU) treatment and mortality in groups with moderate or high (PH-m/h) and low (PH-l) probability of pulmonary hypertension were examined. Results: PH-m/h was observed in 29 patients, of whom 65.5 % had LV diastolic dysfunction stage II or III. A significantly higher occurrence of RAE was observed in the PH-m/h group as compared to the PH-l group. There were no differences between the PH-m/h and PH-l patient groups regarding the in-hospital length of stay or mortality. Conclusions: High or intermediate probability of PH is common in cardiac surgical patients with left ventricular diastolic dysfunction and correlates with respiratory adverse events.
... Parasternal, apical and subxiphoid images were assessed for: The 60/60-sign (concomitant pulmonary valve acceleration time (PVAT) < 60 ms and tricuspid valve regurgitation gradient (TRmax) < 60 mmHg), D-sign (abnormal septal flattening or bulging towards the LV due to RV pressure overload), McConnell's sign (distinctive akinesia of the RV free wall with normal or hyperkinetic apical motion), intracardiac thrombi, basal right ventricular end diastolic diameter (RVEDD)/left ventricular end diastolic diameter (LVEDD)-ratio, pericardial effusion and tricuspid annular plane systolic excursion (TAPSE). The probability of present PH was assessed in accordance with table 8A and B of the collaborative guidelines by the ERS and ESC [11]. ...
Article
PurposeThe purpose of this descriptive feasibility study was to assess the clinical impact and feasibility of conducting a multiorgan ultrasound examination of patients with pulmonary embolism at both time of diagnosis and at clinical follow-up.Methods Hemodynamically stable patients with pulmonary embolism verified by CT pulmonary angiography or ventilation perfusion scintigraphy were eligible for inclusion. Enrolled patients underwent multiorgan ultrasound investigation encompassing echocardiography supplemented with focused lung and deep venous ultrasound emphasizing right ventricular strain, subpleural consolidations and presence of deep venous thrombi. Identical investigations were conducted at 3 months follow-up. The presence of ultrasonographic findings at diagnosis and follow-up was compared and the clinical impact of any remaining pathology or strain was described.ResultsTwenty-one patients were enrolled in the study of whom 20 survived to attend follow-up. Mean age was 62 ± 15 years and 48% were female. At diagnosis, the most prevalent ultrasonographic findings were subpleural consolidations in 11 patients and right ventricular dilation in eight. At follow-up, signs of right ventricular strain had resolved in all patients. However, in one patient, no resolution was seen in a subpleural consolidation observed at the time of pulmonary embolism diagnosis, resulting in referral to a chest CT. Additionally, one patient exhibited residual deep venous thrombotic material, leading to prolongation of anticoagulative treatment.Conclusion In patients with pulmonary embolism, multiorgan ultrasound is feasible in follow-up and adequately powered studies should determine the clinical utility of such an approach.
... Pulmonary arterial hypertension (PAH) is defined according to the recommendation at the time of the study as a resting mean pulmonary artery pressure (mPAP) of 25 mmHg or above. According to the 5th World Symposium on Pulmonary Hypertension (WSPH), PH is divided into: group 1-pulmonary arterial hypertension (PAH); group 2-PH due to left heart disease; group 3-PH due to lung disease and/or hypoxia; group 4-PH due to chronic thromboembolic disease and other pulmonary artery obstructions; and group 5-PH with unclear and/or multifactorial mechanisms [1]. The distribution of etiologies for pediatric PAH is different to that of adults, with a greater predominance of idiopathic PAH (IPAH), PAH associated with congenital heart disease (CHD-PAH) and developmental lung diseases [2,3]. ...
... This was an observational cohort study of patients diagnosed with PAH from 3 months to 18 years of age who underwent baseline diagnostic RHC procedures at a large single tertiary referral center (Beijing Anzhen Hospital, Beijing, China) between January 2005 and December 2020. According to the "2015 ESC/ERS Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension" [1], PH was defined as an increase of mean pulmonary artery pressure (mPAP) ≥ 25 mmHg at rest as assessed by RHC. PAH was defined by a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 3 Wood units in the absence of other causes of precapillary PH. ...
... Acute vasodilator testing (AVT) was performed with an initial dose of inhaled iloprost of 5 µg for 15 min (Ventavis; Bayer-Schering Pharma, Berlin, Germany), since nitric oxide is not available for medical use in China. Acute vasodilator testing (AVT) was performed in PAH patients whose mPAP > 40 mmHg without clinical features of left heart failure, and a positive response was defined according to current guidelines as a decrease in mPAP of at least 10 mmHg to <40 mmHg with a stable cardiac output [1,12,13]. Hemodynamics obtained from RHC procedures and responses to AVT were also collected. Patients diagnosed with complex CHD (e.g., univentricular physiology) or with left or right ventricular outflow obstruction were excluded; however, data collected following anatomic correction were included. ...
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Aim: To define the clinical characteristics, hemodynamics, and adverse events for pediatric patients with pulmonary arterial hypertension (PAH) undergoing right heart catheterization (RHC). Methods: The large referral single center data of 591 diagnostic RHC procedures performed between 2005 and 2020 on pediatric PAH patients was retrospectively collected and analyzed. Results: A total of 591 RHC procedures performed on 469 patients with congenital heart disease (CHD)-PAH (median age 8.8 years, 7.9% New York Heart Association (NYHA) class > II, 1.5% with syncope) and 122 patients with idiopathic PAH (median age of 9.0 years, 27.0% NYHA class > II, 27.0% with syncope) were included. Of those, 373 (63.1%) procedures were performed under general anesthesia. Eighteen patients (18/591, 3.0%) suffered adverse events (mainly pulmonary hypertensive crisis, PHC, n = 17) during the RHC procedure, including 14 idiopathic pulmonary arterial hypertension (IPAH) patients and 4 CHD-PAH patients, and one IPAH patient died in hospital 63 hours after RHC. The risk of developing PHC was significantly increased in patients with IPAH (OR = 14.02, 95%CI: 4.49-43.85, p < 0.001), atrial blood gas pH < 7.35 (OR = 12.504, 95%CI: 3.545-44.102, p < 0.001) and RAP > 14 mmHg (OR = 10.636, 95%CI: 3.668-30.847, p < 0.001). Conclusions: RHC is generally a low-risk procedure in pediatric patients with PAH. However, PHC occur in approximately 3% of patients. Therefore, RHC should be performed in a large, experienced referral pediatric cardiology center, especially in pediatric patients with IPAH requiring general anesthesia.
... 59 Currently approved and available PAH therapieswhich are mostly systemically applied pulmonary vasodilators-are not recommended for patients with PH-ILD based on established PH as well IPF guidelines. 55,60 There is theoretic concern that they may impair gas exchange due to the inhibition of hypoxic pulmonary vasoconstriction, although it has rarely been seen in either clinical trials or everyday practice. [61][62][63] Until recently, there has been a lack of data supporting their efficacy, as well as other data having demonstrated a negative benefit-/risk profile. ...
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Pulmonary hypertension (PH) has been linked to worse outcome in chronic lung diseases. The presence of PH in the setting of underlying Interstitial Lung Disease (ILD) is strongly associated with decreased exercise and functional capacity, increased risk of hospitalizations and death. Examining the scope of this issue and its impact on patients is the first step in trying to define a roadmap to facilitate and encourage future research in this area. The aim of our working group is to strengthen the communities understanding of PH due to lung diseases and to improve the care and quality of life of affected patients. This introductory statement provides a broad overview and lays the foundation for further in‐depth papers on specific topics pertaining to PH‐ILD. This article is protected by copyright. All rights reserved.
... During the 6th World Symposium on Pulmonary Hypertension (WSPH), the risk stratification of PAH patients was greatly emphasized, not only to define long-term prognosis but also to guide therapeutic management [3]. Several multiparametric risk stratification approaches have been used to stratify PAH patients into low-, intermediate-, and high-risk based on expected transplant-free survival, with the aim of titrating medical therapy to achieve low-risk status [4][5][6][7][8][9]. However, studies have shown that the majority of PAH patients remained in the intermediate-risk category after receiving initial PAH-specific therapy [5][6][7], including those receiving recommended upfront combination therapy with a phosphodiesterase type 5 inhibitor (PDE5-I) and an endothelin receptor antagonist (ERA) [10,11]. ...
... However, studies have shown that the majority of PAH patients remained in the intermediate-risk category after receiving initial PAH-specific therapy [5][6][7], including those receiving recommended upfront combination therapy with a phosphodiesterase type 5 inhibitor (PDE5-I) and an endothelin receptor antagonist (ERA) [10,11]. Several treatment options are available for patients who remain in the intermediate-risk category at follow-up [4], and the best treatment strategy for these patients remains uncertain. ...
... The role of 2D echocardiography, a routinely used imaging modality in PAH patients, has been underscored in risk assessment and stratification [3,12]. While both cardiac magnetic resonance and transthoracic echocardiography are non-invasive modalities instrumental for the evaluation of right ventricular (RV) morphology and function with relevant prognostic implications in PAH [13][14][15][16][17], the role of imaging in risk stratification approaches has been limited to the assessment of right atrial (RA) area and the presence of pericardial effusion [4,7]. ...
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Background: Given the morbidity and mortality associated with pulmonary arterial hypertension (PAH), risk stratification approaches that guide therapeutic management have been previously employed. However, most patients remain in the intermediate-risk category despite initial therapy. Herein, we sought to determine whether echocardiographic parameters could improve the risk stratification of intermediate-risk patients. Methods: Prevalent PAH patients previously enrolled in observational studies at 3 pulmonary hypertension centers were included in this study. A validated PAH risk stratification approach was used to stratify patients into low-, intermediate-, and high-risk groups. Right ventricular echocardiographic parameters were used to further stratify intermediate-risk patients into intermediate-low- and intermediate-high-risk groups based on transplant-free survival. Results: From a total of 146 patients included in our study, 38 patients died over a median follow-up of 2.5 years. Patients with intermediate-/high-risk had worse echocardiographic parameters. Tricuspid annular plane systolic excursion (TAPSE) and the degree of tricuspid regurgitation (TR) were highly associated with survival (p < 0.01, p = 0.04, respectively) and were subsequently used to further stratify intermediate-risk patients. Among intermediate-risk patients, survival was worse for patients with TAPSE < 19 mm compared to those with TAPSE ≥ 19 mm (estimated one-year survival 74% vs. 96%, p < 0.01) and for patients with moderate/severe TR compared to those with no/trace/mild TR (estimated one-year survival 70% vs. 93%, p < 0.01). Furthermore, among intermediate-risk patients, those with both TAPSE < 19 mm and moderate/severe TR had an estimated one-year survival (56%) similar to that of high-risk patients (56%), and those with both TAPSE ≥ 19 mm and no/trace/mild TR had an estimated one-year survival (97%) similar to that of low-risk patients (95%). Conclusions: Echocardiography, a routinely performed, non-invasive imaging modality, plays a pivotal role in discriminating distinct survival phenotypes among prevalent intermediate-risk PAH patients using TAPSE and degree of TR. This can potentially help guide subsequent therapy.
... Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature with high morbidity and mortality rate [1]. PAH is a severe complication of connective tissue disease (CTD) including systemic sclerosis (SSc), systemic lupus erythematosus (SLE), undifferentiated CTD (UCTD) and to a lesser extent, Sjögren's syndrome (SS), rheumatoid arthritis (RA) and dermatomyositis [2]. PAH patients often present with dyspnea and functional limitations, negatively affecting patient's health related quality of life (HRQoL) [3,4]. ...
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Backgrounds The EmPHasis-10 questionnaire is a disease-specific quality of life (QoL) measurement in patients with pulmonary hypertension. We report the results of cross-cultural validation of the Chinese version of the EmPHasis-10 and its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). Methods The Emphasis-10 was administered to 75 CTD-PAH patients along with the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The diagnosis of PAH was confirmed by right heart catheterization. Demographic and clinical data were obtained. Multivariable logistic regression was conducted based on the low risk profile assessed by a 4-strata risk assessment model (COMPERA 2.0) at follow-up. Results Date from 75 patients with CTD-PAH were analysed. The EmPHasis-10 demonstrated satisfactory reliability (Cronbach α = 0.95) and convergent validity showed by the significant relationship with WHO Functional Class (P = 0.003), SF-36 (P < 0.001) and EQ-5D (P = 0.002). EmPHasis-10 was significantly associated with achieving the low risk profile at 12 months of follow-up (Odds ratio: 0.928, P = 0.029) after adjusting for WHO Functional Class. Conclusion EmPHasis-10 has acceptable reliability and validity in CTD-PAH patients and may serve as an additional parameter in risk stratification.
... The RHC parameters that are prognostic and currently included in risk assessment include mRAP, CI, SvO2, and PVR. (Benza et al., 2019;Galiè et al., 2015) Our study focused on group 1 PAH and correlated antimony level to the prognostic hemodynamic markers that are currently used in PAH risk assessment which reflect right ventricular function. Given the significant correlation between plasma antimony level and the prognostic hemodynamic markers of PAH that reflects the right ventricular function, it is possible that PAH patients who have higher levels of environmental antimony levels can be predisposed to right ventricular dysfunction which is one of the main determinants of outcomes in PAH. ...
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It is unknown if environmental antimony exposure influences pulmonary arterial hypertension (PAH) and right ventricular function. We performed a pilot study to evaluate antimony levels in 20 PAH patients and 10 controls. Also, we explored the correlation of antimony level with PAH prognostic hemodynamic markers. Antimony blood and plasma levels were significantly higher in PAH patients when compared to controls [blood: PAH mean ± SD (95%CI) 1.3 ± 0.6 (1.0-1.5) ng/ml vs. control mean ± SD (95%) 0.7 ± 0.5 (0.4-1.0) ng/ml, p = 0.017] [plasma: PH mean ± SD (95%CI) 2.6 ± 1 (2.2-3.1) ng/ml vs. control mean ± SD (95%CI) 1.5 ± 0.8 (1.0-2.0) ng/ml, p = 0.004]. Also, antimony blood and plasma levels were significantly higher in idiopathic-PAH patients and non-idiopathic PAH when compared to controls. There was a trend for higher blood and plasma antimony levels in idiopathic PAH [blood1.6 ± 0.6 (1.1-2.1) ng/ml and plasma 3.1 ± 1.2 (2.2-4.1) ng/ml] when compared to non-idiopathic PAH [blood 1.1 ± 0.6(0.8-1.4) ng/ml and plasma 2.5 ± 0.9(2-2.9) ng/ml], but it did not reach statistical significance. There was a significant correlation between plasma antimony level and all the prognostic hemodynamic parameters of PAH including mRAP (r = 0.47, p = 0.036), CO (r = -0.50, p = 0.026), CI (r = -0.54, p = 0.014), PVR (r = 0.52, p = 0.019), and SvO2 (r = -0.54, p = 0.016).