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Schistosomiasis-associated PAH (Sch-PAH) falls under group I pulmonary hypertension and it affects 230 million people, mainly in sub-Saharan Africa. This significant burden has prompted studies on the clinical characteristics and outcomes of Sch-PAH to improve awareness and management, particularly in developing regions. This study investigates the characteristics of Sch-PAH, including demographics, clinical and hemodynamic features, and survival outcomes compared to idiopathic Pulmonary arterial hypertension (IPAH), while evaluating the prognosis and current clinical practices. A cohort of 83 patients, including 41 with Sch-PAH and 42 with IPAH, were studied retrospectively over five years (2019–2024) from 3 pulmonary hypertension centres in Egypt. Data collection focused on demographic details, comorbidities, echocardiographic findings, and survival rates. Sch-PAH patients were significantly older (50.4 ± 12 years) and predominantly male ( P  < 0.001) compared to IPAH patients (34.5 ± 9.7 years). Higher comorbidity rates, chiefly chest and hepatic disorders, were observed in Sch-PAH. The Sch-PAH group exhibited more WHO functional class IV cases and greater left atrium (LA) and pulmonary artery (PA) dilation ( P  = 0.007, P  = 0.006, P  = 0.01, respectively). Lower ejection fraction (EF%) and absence of PA dilation and portal hypertension were linked to improved survival, with EF% emerging as an independent survival predictor (OR = 0.72 and 0.55; P  = 0.002 and < 0.001). The EF% cutoff below 66% showed high predictive accuracy for survival (AUC = 0.85, P  = 0.008). Sch-PAH affects primarily older males with significant comorbidities. Its prevalence poses a public health challenge, making the identification of mortality predictors crucial for early patient risk assessment and improved prognosis management.

Objective The objective of this randomized, placebo-controlled, double-blind, parallel group, trial was to assess the effect of ambrisentan on mean pulmonary arterial pressure (mPAP) in patients with systemic sclerosis (SSc) and mildly elevated pulmonary hypertension (PH). Methods Thirty-eight SSc patients with mildly elevated mPAP at rest between 21 and 24 mmHg and/or > 30 mmHg during low-dose exercise were randomly assigned to treatment with either ambrisentan 5–10 mg/day or placebo. Right heart catheterization and further clinical parameters were assessed at baseline and after 6 months. The primary endpoint was the difference of mPAP change at rest between groups. Results After 6 months, the two groups did not differ in the primary endpoint (ambrisentan mPAP − 1 ± 6.4 mmHg vs. placebo − 0.73 ± 3.59 mmHg at rest, p  = 0.884). However, three patients from the placebo group but none of the ambrisentan group progressed to SSc-associated pulmonary arterial hypertension. Furthermore, ambrisentan treatment showed significant improvements in the secondary endpoints cardiac index (CI) and pulmonary vascular resistance (PVR) at rest (CI 0.36 ± 0.66 l/min/m 2 vs. − 0.31 ± 0.71 l/min/m 2 , p  = 0.010; PVR − 0.70 ± 0.78 WU vs. 0.01 ± 0.71 WU, p  = 0.012) and during exercise (CI 0.7 ± 0.81 l/min/m 2 vs. − 0.45 ± 1.36 l/min/m 2 , p  = 0.015; PVR − 0.84 ± 0.48 WU vs. − 0.0032 ± 0.34 WU, p  < 0.0001). Conclusion This is the first randomized, double-blind, placebo-controlled study testing the effect of ambrisentan in patients with mildly elevated mPAP and/or exercise PH. The primary endpoint change in mPAP did only tendentially improve in the ambrisentan group, but the significant improvement of other hemodynamic parameters points to a possible benefit of ambrisentan and will be helpful to design future trials. Trial registration www.ClinicalTrials.gov, unique identifier NCT: NCT02290613 , registered 14 th of November 2014.

Abstract Rationale Despite the recognition that acute lung injury (ALI) can elevate pulmonary artery (PA) pressure and right ventricular afterload, the impact of pulmonary vascular dysfunction on outcomes of these patients is not well defined. Objectives To investigate the impact of pulmonary vascular dysfunction in patients with acute lung injury. Methods Secondary analysis of the Fluid and Catheter Treatment Trial. A total of 501 patients who received a PA catheter were evaluated for associations between increases in transpulmonary gradient (TPG) (PA mean pressure − PA occlusion pressure) or pulmonary vascular resistance index (PVRi) and 60-day mortality, ventilator-, intensive care unit (ICU)–, and cardiovascular-free days (days with mean arterial pressure ≥ 60 mm Hg off vasopressor support). Measurements and Main Results We were able to measure the TPG in 475 (95%) and the PVRi in 470 (92%) patients. Patients with an elevated baseline TPG had an increased 60-day mortality (30 versus 19%; P = 0.02), and lower numbers of median ventilator- [25–75% quartiles] (15 [0–22] versus 19 [7–24]; P = 0.005), ICU- (14 [0–21] versus 18 [5–22]; P = 0.005), and cardiovascular-free days (23 [12–27] versus 25 [18–27]; P = 0.03). The median PVRi (305 [204–431] dyne s/cm5/m2) was elevated early in the course of ALI. PVRi was statistically higher in patients who died (326 [209–518] versus 299 [199–416]; P = 0.01). In individual multivariate models, TPG and PVRi remained independent risk factors for 60-day mortality and decrease in the number of ventilator-, ICU-, and cardiovascular-free days. Conclusions Pulmonary vascular dysfunction is common in ALI, and is independently associated with poor outcomes. Future trials targeting pulmonary vascular dysfunction may be indicated.

Study Objectives: Pulmonary hypertension (PH) is prevalent in obesity hypoventilation syndrome (OHS). However, there is a paucity of data assessing pathogenic factors associated with PH. Our objective is to assess risk factors that may be involved in the pathogenesis of PH in untreated OHS. Methods: In a post hoc analysis of the Pickwick trial, we performed a bivariate analysis of baseline characteristics between patients with and without PH. Variables with a P value ≤ .10 were defined as potential risk factors and were grouped by theoretical pathogenic mechanisms in several adjusted models. Similar analysis was carried out for the 2 OHS phenotypes, with and without severe concomitant obstructive sleep apnea. Results: Of 246 patients with OHS, 122 (50%) had echocardiographic evidence of PH defined as systolic pulmonary artery pressure ≥ 40 mm Hg. Lower levels of awake PaO 2 and higher body mass index were independent risk factors in the multivariate model, with a negative and positive adjusted linear association, respectively (adjusted odds ratio 0.96; 95% confidence interval 0.93 to 0.98; P = .003 for PaO 2 , and 1.07; 95% confidence interval 1.03 to 1.12; P = .001 for body mass index). In separate analyses, body mass index and PaO 2 were independent risk factors in the severe obstructive sleep apnea phenotype, whereas body mass index and peak in-flow velocity in early/late diastole ratio were independent risk factors in the nonsevere obstructive sleep apnea phenotype. Conclusions: This study identifies obesity per se as a major independent risk factor for PH, regardless of OHS phenotype. Therapeutic interventions targeting weight loss may play a critical role in improving PH in this patient population. Clinical Trial Registration: Registry: Clinicaltrial.gov; Name: Alternative of Treatment in Obesity Hypoventilation Syndrome; URL: https://clinicaltrials.gov/ct2/show/NCT01405976 ; Identifier: NCT01405976. Citation: Masa JF, Benítez ID, Javaheri S, et al. Risk factors associated with pulmonary hypertension in obesity hypoventilation syndrome. J Clin Sleep Med . 2022;18(4):983–992.

Background No population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population. Method We used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model. Results Of 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59–2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively. Conclusions PAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.

Objective This study aims to analyze the genetic characteristics, genotype-phenotype correlation and long-term prognosis of children with idiopathic/hereditary pulmonary arterial hypertension (IPAH/HPAH) in a Chinese tertiary medical center. Methods A retrospective review was conducted for all children with IPAH/HPAH treated at Beijing Anzhen Hospital over the past 15 years. All patients underwent genetic testing. Results In total, 170 children with IPAH/HPAH were included in the study (females n  = 95, 56%), with a median age of diagnosis 6.46 (3.80, 10.70) years. The study population presented with severe conditions at baseline, with 77 patients assessed as clinically high-risk. Genetic testing identified pathogenic variants in 110 patients (64%), with BMPR2 , ACVRL1 , and TBX4 accounted for the main causal genes. Compared to non-carriers, carriers of pathogenic variants had a higher clinical risk at baseline (54% vs. 30%, p  = 0.04). After targeted therapy, carriers experienced greater clinical deterioration ( p  = 0.008). The overall follow-up duration was 2.68 (1.60, 4.98) years, with the survival rate at 1-, 3-, and 5-year was 93.4%, 86.7%, and 68.6%, respectively. The prognosis of carriers was significantly worse than that of non-carriers (Log-rank p  < 0.001). Multivariate Cox regression analysis indicated that pathogenic variants and higher pulmonary vascular resistance index (PVRI) and were associated with a higher risk of death. Conclusion We uncovered a higher rate of pathogenic variants in Chinese pediatric PAH, while targeted therapy improves the overall prognosis of children with PAH, patients with pathogenic variants presented with poorer response to therapy and poorer prognosis.

ObjectivesTo evaluate initial combination therapy with ambrisentan plus tadalafil (COMB) compared with monotherapy of either agent (MONO), and the utility of baseline characteristics and risk stratification in predicting outcomes, in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and the systemic sclerosis (SSc)–pulmonary arterial hypertension (PAH) subpopulation.MethodsThis post hoc analysis of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) study included patients with CTD-PAH from the modified intention-to-treat population. Time to clinical failure (TtCF) was assessed by baseline characteristics, treatment assignment and risk group (low, intermediate and high) at baseline and week 16. TtCF was compared between groups using Kaplan-Meier curves and Cox proportional hazards regression modelling.ResultsThe analysis included 216 patients (COMB, n=117; MONO, n=99). The risk of clinical failure was lower with COMB versus MONO (risk reduction: CTD-PAH 51.7%, SSc-PAH 53.7%), particularly in patients with haemodynamic parameters characteristic of typical PAH without features of left heart disease and/or restrictive lung disease at baseline. The risk of clinical failure was lower with COMB versus MONO in the baseline low-risk group (HR not calculated due to no events in COMB), baseline intermediate-risk group (HR 0.519, 95% CI 0.297 to 0.905) and in the week 16 low-risk group (HR 0.069, 95% CI 0.009 to 0.548).ConclusionsThe benefit of COMB over MONO was demonstrated in patients with CTD-PAH, particularly in those with typical PAH haemodynamic characteristics at baseline. COMB is appropriate for patients categorised as low risk and intermediate risk at baseline and low risk at follow-up.Trial registration number NCT01178073.

Background: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. Objectives: The aim of the study was to evaluate the relationship between precapillary PH and RA. Methods: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population. RA phenotypes, clinical, functional, and hemodynamic data, and patient outcomes were recorded. Results: RA was confirmed in 20 patients (70% female; mean age 52 years) with precapillary PH, including 10 patients with PAH, 6 with severe PH due to lung disease, and 4 with chronic thromboembolic PH. The prevalence of RA was 0.35% (95% CI: 0.23–0.54) in the French PH Registry and 0.58% (95% CI: 0.30–1.11) in idiopathic PAH, comparable to that in the general population. The RA phenotype was characterized by the presence of specific RA autoantibodies and joint erosions in 75% of the patients. The outcomes of PH in the RA patients were unremarkable compared to those in other patients from the registry, and RA therapies had no major impact on the cardiopulmonary parameters. Conclusion: When precapillary PH occurs in RA patients, all PH subsets may be identified. The RA prevalence in the French PH Registry is similar to that in the general population, which does not support a specific association or an indication for PH screening in RA patients.

ObjectivesTo investigate clinical characteristics, symptom profile, testing practices, treatment patterns and quality of life (QoL) among patients with pulmonary arterial hypertension (PAH) in Latin America.DesignData from the Adelphi Real World PAH Disease Specific Programme, a cross-sectional survey with retrospective data collection.SettingUniversity/teaching hospital, regional centres, private practices and government institutions in Argentina, Brazil, Colombia and Mexico.Participants246 physicians provided data for 958 patients, of which 533 patients also self-reported data.ResultsMean (SD) patient age was 53.7 (17) years, 70% of patients were female and 79% were WHO functional class (WHO FC) I–II. Overall, 76% had undergone a right heart catheterisation, ranging from 92% in Argentina to 64% in Brazil (p<0.0001). Only 28% underwent a simplified risk assessment strategy in the past 12 months, ranging from 46% in Argentina to 16% in Brazil. Fatigue and dyspnoea on exertion were reported most commonly by physicians (37% and 53%) and patients (68% and 67%). Patient–physician agreement on symptom reporting was minimal-to-weak (kappa, 0.21–0.42). PAH-specific combination therapy varied across countries (21% Mexico, 30% Brazil, 70% Colombia and 79% Argentina, p<0.0001)). Overall, 73% of patients received a phosphodiesterase type 5 inhibitor; 52% an endothelin receptor antagonist, 15% a prostacyclin pathway agent and 11% a soluble guanylate cyclase stimulator. The mean (SD) EQ-5D (generic instrument to define quality of life)utility ranged from 0.66 (0.20) to 0.70 (0.20) across countries and the mean (SD) EQ-5D Visual Analogue Scale (VAS) was 67.0 (18.10). Lower VAS and utility scores were reported among patients with higher WHO FC (p<0.05).ConclusionsPatients reported a high burden of PAH in terms of symptoms and QoL, particularly within higher WHO FC. Low usage of risk assessment strategies and PAH-specific combination therapy was seen in Brazil and Mexico. Further research could identify barriers to prescribing optimal treatment.

Patients considering destination therapy left ventricular assist devices (DT LVAD) often have high comorbid burden but the association between these comorbidities and post-decision outcomes is unknown. We included subjects in DECIDE-LVAD (NCT02344576), a stepped-wedge multicenter trial of patients considering LVADs, recording comorbidities per INTERMACS protocol. We compared decisional conflict, regret, perceived stress, quality of life (EQ-VAS), depression (PHQ-2), struggle with- and acceptance of illness by comorbid burden and amongst the most common comorbidities. Of 239 patients, LVAD recipients (n = 164) and non-recipients (n = 75) had a similar proportion with ≥1 comorbidity (70% v. 80%, P = .09). Patients with comorbidities were younger regardless of LVAD implantation status. After adjusting for age, overall and amongst LVAD recipients, patients with ≥1 comorbidity had higher mean decision conflict at baseline (23.2 ± 1.5 vs. 17.4 ± 2.2), and at 6 months, higher stress (13.0 ± 0.6 vs. 10.4 ± 1.0) and struggle with illness (13.3 ± 0.4 vs. 11.1 ± 0.6) than those without comorbidities (P < .05). No difference was noted in decision regret, PHQ-2, EQ-VAS, acceptance of illness and survival overall and amongst LVAD recipients. Of the three most common comorbidities, while patients with pulmonary hypertension had worse decision regret, depression, stress and acceptance of illness at 6-month follow-up than those who did not have pulmonary hypertension, no difference was noted in patients with chronic renal disease or high body mass index. Patients considering LVAD implantation with comorbidities experience increased decision conflict, stress and struggle with illness. These findings provide insights in the role comorbidities play in patient decision-making and decisional outcomes.