Pulmonary Circulation[JOURNAL]
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200 papers
Repair of Sinus Venosus Atrial Septal Defect in 2 Patients With Severe Pulmonary Arterial Hypertension.
Sakuma R, Morgan G, Yeung E … +3 more , Frank BS, Simpkin C, Ivy D
Pulm Circ · 2026 Jan · PMID 42394956 · Full text
Pretreatment with targeted pulmonary arterial hypertension (PAH) therapy in adults with unrepaired atrial septal defects (ASDs) has been increasingly reported as a means to improve pulmonary hemodynamics and expand candi...
Pretreatment with targeted pulmonary arterial hypertension (PAH) therapy in adults with unrepaired atrial septal defects (ASDs) has been increasingly reported as a means to improve pulmonary hemodynamics and expand candidacy for defect closure. However, most published reports describe heterogeneous ASD cohorts, predominantly composed of secundum ASDs, with limited data on sinus venosus atrial septal defects (SVASDs). We describe two cases of adult superior SVASD with severe PAH, initially deemed unsuitable for closure, who underwent prolonged pretreatment with PAH-targeted therapy and subsequently achieved successful repair, one via transcatheter intervention. Both patients demonstrated improved functional status after repair and were able to reduce PAH therapy. These cases underscore the potential of pre-treatment with PAH-targeted therapy to bridge to repair in selected SVASD patients and highlight the need for further study in this unique subgroup.
Changes in Arterial-Alveolar Oxygen Gradient and Mixed Venous Oxygen Saturations in Mechanical Thrombectomy for Pulmonary Embolism: A Prospective Cohort Study.
Walsh AD, Warner J, Dunn A … +1 more , Ahmad K
Pulm Circ · 2026 Jun · PMID 42370294 · Full text
Large bore mechanical thrombectomy (LBMT) is an effective therapy for intermediate-high risk PE, however, objective physiological endpoints to guide intraprocedural decision-making remain poorly defined. The alveolar-art...
Large bore mechanical thrombectomy (LBMT) is an effective therapy for intermediate-high risk PE, however, objective physiological endpoints to guide intraprocedural decision-making remain poorly defined. The alveolar-arterial (A-a) oxygen gradient, mixed venous oxygen saturation (SvO), and cardiac index (CI) may provide clinically useful insights during LBMT. We sought to determine if changes in alveolar-arterial gradients and estimated cardiac index were sufficiently consistent to be used to inform clinical decision making. This was a single-center, prospective, pre-post cohort study of patients undergoing LBMT for intermediate-high risk PE. Arterial and mixed venous blood gases were aspirated immediately before and after thrombectomy. CI was estimated via the indirect Fick method. Primary outcomes were changes in A-a gradient and mixed venous oxygen saturations; secondary outcomes included estimated CI and Modified Borg Dyspnea Scale. Sensitivity analyses excluded physiologically implausible outliers and accounted for intraprocedural hemoglobin changes. Twenty-seven patients were included. LBMT was associated with a nonsignificant reduction in A-a gradient (-38.5 ± 129.9 mmHg, = 0.052), which became significant after exclusion of outliers (-41.5 ± 62.9 mmHg, = 0.0096). Mixed SvO decreased in most patients by 3.8% ± 7.3% ( = 0.015), corresponding to a modest reduction in estimated CI (-0.16 ± 0.67 L/min/m, = 0.032) which persisted after controlling for hemoglobin loss. Dyspnea scores improved in most patients with paired measurements. Acute improvements in oxygenation efficiency were reflected by reductions in A-a gradient, while paradoxical reductions in SvO and estimated CI suggest altered oxygen extraction ratio post-procedure. These parameters may serve as novel intraprocedural physiological markers to complement thrombectomy endpoints.
Echocardiography-Derived Exercise Pulmonary Hypertension and Longitudinal Changes in Pulmonary Artery Pressures in Systemic Sclerosis: A Non-Invasive Assessment for Risk Stratification.
Kagami K, Yuasa N, Harada T … +7 more , Tani Y, Endo Y, Takama N, Wada N, Motegi SI, Ishii H, Obokata M
Pulm Circ · 2026 Jan · PMID 42370293 · Full text
Pulmonary artery pressures (PAPs) often become abnormal during exercise in patients with systemic sclerosis (SSc). This study sought to determine whether exercise-induced pulmonary hypertension (EIPH) assessed by exercis...
Pulmonary artery pressures (PAPs) often become abnormal during exercise in patients with systemic sclerosis (SSc). This study sought to determine whether exercise-induced pulmonary hypertension (EIPH) assessed by exercise stress echocardiography (ESE) would be associated with subsequent increases in resting PAPs in patients with SSc. Patients with SSc who underwent serial echocardiography > 6 months apart were retrospectively screened ( = 543). Of those, patients undergoing ESE were divided based on the presence of EIPH, defined as estimated mean PAP (mPAP) > 30 mmHg during exercise and a mPAP/cardiac output (CO) > 3 mmHg/L/min. Patients who did not undergo ESE and had a mPAP < 20 mmHg were selected as controls ( = 198). The primary endpoint was a longitudinal change in sPAP from baseline to last follow-up echocardiography. Compared to controls and patients with non-EIPH ( = 71), those with EIPH ( = 56) had a higher sPAP at baseline (22 ± 4 mmHg, 28 ± 6 mmHg, and 31 ± 6 mmHg, < 0.0001). Over a median follow-up duration of 2.4 years, a longitudinal change in resting sPAP from baseline to follow-up echocardiography was higher in patients with EIPH than those without (+ 1.2 mmHg vs. -1.0 mmHg, = 0.048). In a sensitivity analysis excluding 30 patients who received pulmonary vasodilators or heart failure medications after baseline echocardiography, the difference in sPAP change over time between the two groups increased further (+ 2.2 mmHg in the EIPH group vs. -1.6 mmHg in the non-EIPH group, = 0.0008). In conclusion, echocardiography-derived EIPH was associated with a longitudinal increase in sPAP, supporting ESE as a non-invasive tool for risk stratification in SSc-PH.
Sotatercept as a Therapeutic Option for Systemic Sclerosis-Associated Pulmonary Arterial Hypertension With Features of PVOD/PCH.
Nishioka Y, Imanishi K, Matsuda E … +4 more , Nakamura W, Shirayanagi R, Ojiro H, Katagiri A
Pulm Circ · 2026 Jun · PMID 42358521 · Full text
A 53-year-old woman with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) presented with severe exertional desaturation, markedly reduced diffusing capacity (%DLCO 30%), and computed tomography fin...
A 53-year-old woman with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) presented with severe exertional desaturation, markedly reduced diffusing capacity (%DLCO 30%), and computed tomography findings demonstrating features of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH). Despite triple oral therapy, pulmonary hypertension progressively worsened, with mean pulmonary arterial pressure increasing from 34 to 51 mmHg. Sotatercept was initiated because of concerns about the risk of pulmonary edema with parenteral prostacyclin therapy. Despite intermittent treatment interruption due to hemoglobin elevation, improvements were observed in World Health Organization functional class (III-II), 6-min walk distance (217-301 m), brain natriuretic peptide (206.4-52.8 pg/mL), and mean pulmonary arterial pressure (51-45 mmHg), without pulmonary edema. This case suggests a potential benefit of sotatercept in selected patients with SSc-PAH with features of PVOD/PCH.
Patient Perspectives on Palliative Care in Pulmonary Arterial Hypertension in the United States.
El-Kersh K, Crawford R, DuBrock H … +5 more , Classi P, Phan P, McLeod L, Wu B, Sese D
Pulm Circ · 2026 Jun · PMID 42358520 · Full text
Palliative care can be offered concurrently as a treatment option to patients with pulmonary arterial hypertension (PAH) to relieve symptoms and improve quality of life. However, it is infrequently used and is associated...
Palliative care can be offered concurrently as a treatment option to patients with pulmonary arterial hypertension (PAH) to relieve symptoms and improve quality of life. However, it is infrequently used and is associated with misconceptions. Limited studies have reported patients' perspectives of palliative care as a treatment option for PAH. We conducted a web-based survey, informed by a brief targeted literature review and input from patients and clinicians, which was completed by adults (≥ 18 years) in the United States (US) with self-reported PAH. Patients reported demographic and disease variables and their perspectives and experiences with palliative care. Patients self-reported their Pulmonary Hypertension Functional Class Self-Report class, EmPHasis-10, and 6-min walk test. A total of 200 respondents (86.5% female; mean age 58.5 years) from 42 US states completed the survey between 19 September and 3 October 2023. Most respondents described palliative care as "end-of-life care" ( = 43, 25.7%) and associated it with the need for extra support ( = 111, 55.5%). Many respondents ( = 171, 85.5%) had not discussed palliative care services, nor had a palliative care consultation with their healthcare provider (HCP); most had not been offered a consultation ( = 109/171, 63.7%). Despite this low frequency of discussions, respondents reported feeling comfortable discussing palliative care with their HCPs ( = 177, 88.5%). The survey results demonstrated that these patients with PAH had limited experiences with palliative care, where palliative care was frequently subject to misinterpretation. These findings emphasize the need for proactive engagement between patients and HCPs to ensure palliative care is optimally used and understood.
Right Ventricular Mechanical Dyssynchrony Reflects Hemodynamic Load in Pre-Capillary Pulmonary Hypertension.
Igata S, Tahara N, Tahara A … +5 more , Honda A, Kugai T, Eto Y, Maeda-Ogata S, Fukumoto Y
Pulm Circ · 2026 Jun · PMID 42345008 · Full text
Right ventricular (RV) mechanical dyssynchrony represents a maladaptive response to increased afterload in pulmonary hypertension (PH). However, its hemodynamic and biochemical determinants remain unclear. This study aim...
Right ventricular (RV) mechanical dyssynchrony represents a maladaptive response to increased afterload in pulmonary hypertension (PH). However, its hemodynamic and biochemical determinants remain unclear. This study aimed to identify the clinical, hemodynamic, and biochemical correlates of RV dyssynchrony in patients with pre-capillary PH. Thirty patients with pre-capillary PH (83% female; age 56 ± 19 years), all without intraventricular conduction delay (QRS < 120 ms), underwent right heart catheterization, cardiac magnetic resonance imaging, and two-dimensional speckle-tracking echocardiography. RV dyssynchrony was quantified using the coefficient of variation of time-to-peak systolic strain in four mid-basal RV segments (RV-CV4) adjusted for heart rate. Mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), and RV-CV4 averaged 34.1 ± 11.2 mmHg, 522.3 (interquartile range, 289.4-692.6) dyn·s·cm, and 0.134 ± 0.083, respectively, in a cohort with predominantly mild-to-moderate RV systolic dysfunction. RV dyssynchrony correlated significantly with mPAP ( = 0.509) PVR ( = 0.507), and RV-pulmonary artery coupling ( = -0.406) (all < 0.05). In multivariate analysis, mPAP remained the sole independent predictor of RV dyssynchrony ( = 0.509, 95% CI 0.001-0.006, = 0.004), accounting for 26% of its variance. Neither RV end-diastolic volume nor RV ejection fraction were independently associated with dyssynchrony. RV mechanical dyssynchrony reflects hemodynamic afterload, particularly elevated mPAP, rather than structural remodeling. Notably, dyssynchrony was evident even in mild-to-moderate RV dysfunction, indicating an early manifestation of pressure-induced impairment. RV-CV4 may serve as a noninvasive marker of pulmonary vascular load and highlight the importance of afterload reduction in optimizing RV mechanical coordination. Accordingly, RV dyssynchrony assessment may provide a practical echocardiographic surrogate for invasive hemodynamic evaluation in clinical practice.
HIV-Associated Pulmonary Arterial Hypertension in Resource-Limited Settings: A "Forgotten" Diagnosis, a Great Imitator, and a Multi-Hit Pulmonary Vasculopathy.
Eroume A Egom E, Adamou M, Djouldé I … +6 more , Ibrahim N, Célestin M, Ambe LA, Fabrice MM, Moustapha, Kamgang R
Pulm Circ · 2026 Jun · PMID 42328183 · Full text
HIV-associated pulmonary arterial hypertension (HIV-PAH) remains one of the most consequential cardiopulmonary complications in people living with HIV (PLWH), persisting despite expanded antiretroviral therapy (ART) cove...
HIV-associated pulmonary arterial hypertension (HIV-PAH) remains one of the most consequential cardiopulmonary complications in people living with HIV (PLWH), persisting despite expanded antiretroviral therapy (ART) coverage. Although contemporary hemodynamic criteria define pulmonary hypertension (PH) as mean pulmonary arterial pressure (mPAP) > 20 mmHg and pulmonary arterial hypertension (PAH) as pre-capillary physiology confirmed by right heart catheterization (mPAP > 20 mmHg, pulmonary arterial wedge pressure ≤ 15 mmHg, pulmonary vascular resistance > 2 Wood units), global epidemiologic certainty remains uneven. In high-income settings, prospective screening paradigms incorporating catheter confirmation estimate HIV-PAH prevalence at approximately 0.5%, exemplified by the multicenter French study reporting 0.46%. In contrast, in sub-Saharan Africa-home to more than 26 million PLWH-the population prevalence of catheter-confirmed HIV-PAH remains undefined, with echocardiography-based studies in symptomatic cohorts reporting substantially higher rates of elevated pulmonary pressures. Mechanistically, HIV-PAH is best conceptualized as a multi-hit pulmonary vasculopathy driven by viral proteins (Tat, Nef, gp120), chronic immune activation, endothelial dysfunction, oxidative stress, dysregulated endothelin signaling, and growth-factor pathways, with amplification by endemic co-factors and host susceptibility. Clinically, HIV-PAH is a "clinically nonspecific phenotype", frequently obscured by tuberculosis-related lung disease, anemia, left heart disease, and other causes of dyspnea in resource-limited settings. Early recognition is critical because prognosis depends on preservation of right ventricular function. A practical, resource-appropriate strategy is required: heightened clinical suspicion, echocardiography-anchored detection, systematic exclusion of alternative PH etiologies, rational biomarker use, and referral pathways to right heart catheterization where feasible. ART optimization is necessary but insufficient; PAH-targeted therapies can improve functional and hemodynamic outcomes, though HIV-specific randomized evidence remains limited. Implementation science and mechanism-informed research in endemic regions are urgently needed to close the diagnostic and therapeutic gap.
Correction to "Male Sex Is Associated With Severe Pulmonary Hypertension and Worse Outcome in Chronic Obstructive Pulmonary Disease Patients Referred for Right Heart Catheterization".
Pulm Circ · 2026 Jun · PMID 42306239 · Full text
[This corrects the article DOI: 10.1002/pul2.70317.].
[This corrects the article DOI: 10.1002/pul2.70317.].
A Phase 2 Study of the Safety and Tolerability of Treprostinil Palmitil Inhalation Powder in Patients With Pulmonary Hypertension Associated With Interstitial Lung Disease.
Church C, Molina-Molina M, Arias-Guillén M … +12 more , Muchmore P, Lavon BR, Backer J, Makulova N, Mistry B, Guo J, Wu J, Fahumy J, Teper A, Zhao PL, Mange KC, O'Brien G
Pulm Circ · 2026 Jun · PMID 42306238 · Full text
Treprostinil palmitil (TP) inhalation powder (TPIP), an investigational formulation of the treprostinil prodrug TP, was designed to provide prolonged pulmonary vasodilation with once-daily administration. In this phase 2...
Treprostinil palmitil (TP) inhalation powder (TPIP), an investigational formulation of the treprostinil prodrug TP, was designed to provide prolonged pulmonary vasodilation with once-daily administration. In this phase 2 study (NCT05176951), adults with pulmonary hypertension associated with interstitial lung disease (PH-ILD) were randomized 3:1 to receive TPIP ( = 29) or placebo ( = 10) for 16 weeks. TPIP was up-titrated from 80 µg to each patient's maximum tolerated dose (≤ 640 µg). Study endpoints were safety and tolerability (primary); pharmacokinetics of TP and treprostinil (secondary); and clinical worsening, change from baseline in 6-min walk distance (6MWD), and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels (exploratory). Maximum TPIP dose (640 µg) once daily was achieved by 79.3% of patients. TPIP was well tolerated in this study, with a safety profile generally consistent with previously reported route-specific treatment-emergent adverse events (TEAEs) of inhaled treprostinil, such as cough and dyspnea. TEAEs occurred in 93.1% and 90.0% of patients on TPIP and placebo, respectively. Most TEAEs were mild or moderate in both groups. In patients on TPIP and placebo, TEAEs leading to treatment discontinuation occurred in 4 (13.8%) and 3 (30.0%) patients, respectively, and serious AEs occurred in 6 (20.7%) and 4 (40.0%) patients, respectively. Following the 640 µg TPIP dose, the treprostinil elimination half-life was 7.1 h. In TPIP and placebo groups, clinical worsening was observed in 3 (10.3%) and 5 (50.0%) patients, respectively. Trends for improvement were observed in 6MWD and NT-proBNP with TPIP treatment. These results suggest that TPIP may be a suitable once-daily treatment for PH-ILD.
Factors Influencing Physical Activity Engagement Amongst Those Living With Pulmonary Hypertension in the UK.
Hardcastle SJ, Wells Z, Keen C … +3 more , Condliffe R, Kiely DG, McCormack C
Pulm Circ · 2026 Jun · PMID 42293310 · Full text
This study explored attitudes towards exercise, recollections of physical activity advice received, and the dimensions that influence physical activity (PA) engagement in individuals diagnosed with pulmonary hypertension...
This study explored attitudes towards exercise, recollections of physical activity advice received, and the dimensions that influence physical activity (PA) engagement in individuals diagnosed with pulmonary hypertension. Virtual semi-structured interviews ( = 21) were conducted with those diagnosed with pulmonary hypertension. Participants (mean age 57.4 (SD ± 13.1) were recruited through the Pulmonary Hypertension Association of the UK. Interviews were transcribed verbatim and analysed using reflexive thematic analysis. Four main themes were generated: (i) Fear of breathlessness and overdoing it; (ii) lack of motivation and desire for monitoring and targets; (iii) self-presentation: keeping up appearances, and (iv) Little PA advice: patient-driven communication. A lack of motivation was a common barrier to PA engagement amongst PH patients. Other dimensions included fear and anxiety related to dyspnoea and overexertion and protective self-presentation. The influence of self-presentation and identity in exercise-avoidance in PH is a novel finding. The study also found recollections of little PA advice from treating clinicians and a tendency for patient driven PA information. Future PA interventions that alleviate fear and anxiety through cognitive restructuring, provide reassurance on the safety and benefits of exercise, and include clear instruction on behaviour and graded exposure would be worthwhile to increase PA in PH. Interventions that broaden the conception of PA and promote lifestyle-based PA alongside fostering patient motivation for PA through goal setting, self-monitoring and review of behaviour are also likely to be valuable.
A Multicenter, Double-Blind, Placebo-Controlled, Randomized Clinical Trial of Oral Anticoagulation in Systemic Sclerosis-Related Pulmonary Arterial Hypertension-Results From the SPHInX Study.
Nikpour M, Calderone A, Proudman SM … +14 more , Hansen D, Gabbay E, Dwyer N, Keir G, Thakkar V, Wong PKK, Keogh A, Burns A, Prior D, Nandurkar H, Celermajer DS, Sahhar J, Buchbinder R, Stevens W
Pulm Circ · 2026 Jun · PMID 42266819 · Full text
The SPHInX study is the first-ever randomized controlled trial (RCT) seeking to inform an area of equipoise regarding the efficacy of oral anticoagulation as adjunct therapy in systemic sclerosis-related pulmonary arteri...
The SPHInX study is the first-ever randomized controlled trial (RCT) seeking to inform an area of equipoise regarding the efficacy of oral anticoagulation as adjunct therapy in systemic sclerosis-related pulmonary arterial hypertension (SSc-PAH). The SPHInX study was an Australian multicenter, double-blind Phase III RCT of 1:1 oral apixaban 2.5 mg twice daily versus placebo over 3 years, as additional therapy in patients with SSc-PAH. The primary end-point was time to clinical worsening (TtCW), or death. A sample size of 85 per arm was required to show a two-fold reduction in TtCW. Participants experiencing clinical worsening events continued in the study, allowing for assessment of exploratory endpoints (including physical function and quality of life measures) up until 3 years of treatment. Among 11 SSc-PAH participants assigned apixaban treatment and 14 participants assigned placebo, apixaban demonstrated no benefit compared to placebo for TtCW from commencement until 30 days after discontinuation of study drug, Cox proportional hazard ratio 0.92 (95% confidence interval 0.32-2.66), = 0.88. There was no difference between treatment groups in event-free survival, all-cause mortality, or exploratory endpoints. New iron deficiency anemia occurred in 9 (81.8%) apixaban treated participants compared to 4 (28.6%) controls. Although strict selection criteria in a complex disease meant recruitment was insufficient for the primary efficacy endpoint, the SPHInX RCT showed no signal for benefit with anticoagulation as adjunct therapy, with a high frequency of iron deficiency anemia suggesting that even at low doses, risk may outweigh benefit with anticoagulation in SSc-PAH. Australian New Zealand Clinical Trials Registry Registration Number: ACTRN12614000418673 (http://www.ANZCTR.org.au/ACTRN12614000418673.aspx).
Efficacy and Safety of Yiqi Traditional Chinese Medicine Injections for Chronic Pulmonary Heart Disease: A Systematic Review With Pairwise Meta-Analyses and Indirect Comparisons.
Yue H, Chai J, Liao H … +2 more , Jia Y, Wang P
Pulm Circ · 2026 Jun · PMID 42266818 · Full text
In this study, we compared the clinical efficacy and safety of various Yiqi Chinese medicine injections in chronic pulmonary heart disease (CPHD) through pairwise meta-analyses and indirect comparisons within a systemati...
In this study, we compared the clinical efficacy and safety of various Yiqi Chinese medicine injections in chronic pulmonary heart disease (CPHD) through pairwise meta-analyses and indirect comparisons within a systematic review. We searched seven databases for randomized controlled trials (RCTs) of the protective effects of Yiqi Chinese medicine injections combined with standard care on CPHD. The literature search period extended from the establishment of the database to June 30, 2025. RevMan 5.4.1 and Stata 14.0 were used for quality assessment, pairwise meta-analyses, and indirect comparisons. This study incorporated a total of 72 articles, which collectively involved 5836 patients and examined six distinct types of traditional Chinese medicine injections. The results indicated that the adjuvant conventional treatments of Shenfu, Shenmai, Huangqi, Shengmai, Xinmailong, and Shenqi Fuzheng injections were more effective than conventional treatment alone ( < 0.05) in enhancing clinical efficacy. Furthermore, based on limited evidence, Shenqi Fuzheng Injection may have potential benefits, but this finding is highly uncertain (surface under the cumulative ranking [SUCRA] = 79.7%). In addition, Shenqi Fuzheng injection also had a good effect on improving partial pressure of carbon dioxide (PaCO) (SUCRA = 81.9%) and partial pressure of oxygen (PaO) (SUCRA = 79.7%). On the basis of conventional treatment, the combination of the six traditional Chinese medicine injections mentioned above can significantly enhance the clinical efficacy rate. Compared with other injections, Shenqi Fuzheng injection may have benefits, but this finding is highly uncertain.
Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in an Adolescent With a Novel Frameshift Variant in PROS1.
Fujii H, Isobe S, Taniguchi Y … +1 more , Tamura Y
Pulm Circ · 2026 Jun · PMID 42254881 · Full text
Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in adolescents, and evidence regarding balloon pulmonary angioplasty (BPA) in young patients with hereditary thrombophilia remains limited. A 16year-old male...
Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in adolescents, and evidence regarding balloon pulmonary angioplasty (BPA) in young patients with hereditary thrombophilia remains limited. A 16year-old male was diagnosed with CTEPH and protein S deficiency, characterized by markedly low activity and antigen levels. Genetic analysis revealed a novel PROS1 frameshift variant (c.53del, p.Val18GlyfsTer69) predicted to cause loss of function. Despite anticoagulation and riociguat therapy, pulmonary hypertension persisted, and pulmonary angiography demonstrated predominantly peripheral organized thrombotic lesions. The patient underwent staged BPA at ages 16 and 17, with an additional session at 22. All procedures were completed without complications and were associated with sustained improvement in hemodynamics and exercise capacity during long-term follow-up. This case highlights the importance of considering hereditary thrombophilia in adolescent patients with CTEPH and suggests that staged BPA may be a safe and effective treatment option in selected patients with predominantly peripheral disease.
Balancing Risk and Hope: Insights Into Pregnancy Outcomes in Pulmonary Arterial Hypertension.
Segura de la Cal T, Pérez-Olivares C, Luna-López R … +12 more , Martín de Miguel I, Cruz-Utrilla A, Villar O, Sarnago F, Muñoz P, Santiago Sánchez M, López Gude MJ, Velázquez M, Forcén L, Delgado JF, Arribas Ynsaurriaga F, Escribano-Subias P
Pulm Circ · 2026 Jun · PMID 42254880 · Full text
Pregnancy poses substantial risks for women with Pulmonary Arterial Hypertension (PAH). This study aimed to evaluate maternal and neonatal outcomes in a contemporary PAH cohort under modern management, specifically inves...
Pregnancy poses substantial risks for women with Pulmonary Arterial Hypertension (PAH). This study aimed to evaluate maternal and neonatal outcomes in a contemporary PAH cohort under modern management, specifically investigating whether vasoreactive status warrants distinct clinical recommendations. We conducted a retrospective, longitudinal cohort study of PAH pregnancies managed at our institution between February 2010 and December 2023, comparing clinical characteristics, maternal complications, long-term outcomes, and neonatal parameters between vasoreactive and non-vasoreactive patients. Among 29 patients, 8 underwent pregnancy termination, and 21 proceeded with the pregnancy; 7 (33.3%) of whom were vasoreactive. Non-vasoreactive patients experienced significantly higher rates of major complications (35.7% vs. 0%, = 0.039) and minor complications (92.9% vs. 0%, < 0.001). Complications in the non-vasoreactive subgroup included prostacyclin initiation (78.6%), inotropic support (35.7%), mechanical circulatory support (14.3%), and one death (7.14%). All neonates born to non-vasoreactive patients were delivered preterm and showed a trend toward higher complication rates (84.6% vs. 42.9%, = 0.054). Over a median 5-year follow-up, two non-vasoreactive patients died, whereas none of the vasoreactive patients lost their vasoreactive profile. In conclusion, despite advances in management, pregnancy in PAH remains associated with substantial maternal and neonatal morbidity. Vasoreactive patients, however, exhibit a distinctly favorable prognosis for both mother and offspring, which suggest the need for tailored recommendations for this subgroup.
Long-Term Outcomes After Catheter-Directed Thrombolysis or Mechanical Thrombectomy in Intermediate-High-Risk Pulmonary Embolism: A Propensity-Matched Analysis From a TriNetX Database.
Giorgi C, Bertoletti L, Luis JP … +10 more , Rivera-Lebron B, Gayen S, Omid P, Reeve D, Illipparambil L, Weaver S, Naranjo-Tovar M, Truong V, El-Moghraby A, Rali P
Pulm Circ · 2026 Jun · PMID 42254879 · Full text
Catheter-directed thrombolysis (CDL) and mechanical thrombectomy (MT) are increasingly used for intermediate-high-risk pulmonary embolism (PE), yet comparative long-term outcomes remain uncertain. We aimed to compare the...
Catheter-directed thrombolysis (CDL) and mechanical thrombectomy (MT) are increasingly used for intermediate-high-risk pulmonary embolism (PE), yet comparative long-term outcomes remain uncertain. We aimed to compare the effectiveness and safety of CDL versus MT in real-world clinical practice. Using the multi-institutional TriNetX network, we identified adults with acute intermediate-high-risk PE treated with CDL or MT. One-to-one propensity score matching was performed across demographics, comorbidities, and laboratory variables. Outcomes were assessed up to 2 years post-index. The primary outcome was all-cause mortality, while secondary outcomes included hospitalization, major adverse cardiovascular events (MACE), pulmonary hypertension (PH), and bleeding. After matching, 1290 patients remained in each group with balanced baseline characteristics, with a median follow-up of approximately 1.5 years. Long-term mortality was similar between CDL and MT (3.1% vs. 3.5%; = 0.061). MACE rates were comparable (5.2% vs. 4.6%; = 0.933), with no significant difference in PH (3.0% vs. 3.24%; = 0.352). CDL was associated with higher hospitalization rates (2.2% vs. 0.9%; = 0.026), while bleeding events were low and similar between groups. In this large real-world cohort of patients with intermediate-high-risk PE, CDL and MT demonstrated broadly comparable long-term outcomes, suggesting no clear superiority of either strategy and supporting ongoing clinical equipoise. Ongoing randomized trials are needed to further inform optimal management.
Reduced Circulating HIF-1α, HIF-2α, HIF-3α, and VHL Levels in Patients With Pulmonary Arterial Hypertension.
Kan H, Alsancak Y, Uyaner Kan M … +4 more , Göktaş E, Çelik M, Gürbüz AS, Düzenli MA
Pulm Circ · 2026 Jun · PMID 42254878 · Full text
Pulmonary arterial hypertension (PAH) involves complex vascular remodeling. The hypoxia-inducible factor (HIF) pathway is a central oxygen-sensing mechanism, but its circulating profile in PAH is poorly defined. This cas...
Pulmonary arterial hypertension (PAH) involves complex vascular remodeling. The hypoxia-inducible factor (HIF) pathway is a central oxygen-sensing mechanism, but its circulating profile in PAH is poorly defined. This case-control study included 71 patients with PAH and 93 age- and sex-matched healthy controls. Serum levels of HIF-1α, HIF-2α, HIF-3α, and VHL were measured by ELISA. Serum levels of HIF-1α, HIF-2α, HIF-3α, and VHL were significantly lower in PAH patients compared with controls (all < 0.05). HIF-1α demonstrated the highest diagnostic accuracy (AUC: 0.891; 95% CI: 0.836-0.947, < 0.001). HIF-2α levels were significantly lower in patients in the intermediate-high risk category compared to the low-intermediate risk category ( = 0.0449). Circulating HIF pathway proteins are downregulated in PAH. These markers, particularly HIF-1α and HIF-2α, show potential for diagnostic screening and risk stratification, suggesting a systemic exhaustion of oxygen-sensing mechanisms.
Mental Disorders and Quality of Life in Patients With Pulmonary Arterial Hypertension Treated With Sotatercept.
Fuge J, Olsson KM, Remmert K … +6 more , Stapel B, Kamp JC, Park DH, Hoeper MM, Winter L, Kahl KG
Pulm Circ · 2026 Jun · PMID 42254877 · Full text
Sotatercept has recently expanded treatment options for pulmonary arterial hypertension (PAH), but its mental health and quality-of-life impact is not well characterized. We assessed psychiatric diagnoses and patient-rep...
Sotatercept has recently expanded treatment options for pulmonary arterial hypertension (PAH), but its mental health and quality-of-life impact is not well characterized. We assessed psychiatric diagnoses and patient-reported outcomes in PAH patients treated with sotatercept within the clinical trial program. PAH Patients previously assessed for psychiatric disorders and quality of life were re-evaluated after ≥ 6 months of sotatercept exposure. A senior psychiatrist performed a Structured Clinical Interview for DSM-5, and participants completed validated questionnaires assessing anxiety/depression (HADS), metacognitive beliefs (MCQ-30), quality of life (WHOQOL-BREF), and PAH-related symptoms/impacts (PAH-SYMPACT™), alongside items capturing patient experiences during therapy. Twenty patients (85% female; median age 46 years) were included. Hemodynamics, biomarkers, and 6-min walk distance improved. The prevalence of at least one current psychiatric disorder remained high and largely unchanged from baseline to follow-up (50% to 45%). Major depressive disorder remained stable (25%), whereas adjustment disorder newly occurred in 25% of patients. HADS and MCQ-30 scores did not change. WHOQOL-BREF global scores increased modestly, but the mental health domain declined substantially (69.6 ± 13.3 to 48.1 ± 15.5, = 0.001). PAH-SYMPACT™ indicated low symptom burden at follow-up. Many patients reported increased optimism and plans to travel or return to work, while some expressed concerns about finances and social security. While sotatercept treatment led to clinical improvements in PAH patients, the prevalence of mental disorders remained high with an increased prevalence in adjustment disorder. These findings support routine mental health assessment and targeted psychosocial support when initiating life-changing PAH therapies.
Prevalence, Characteristics, and Outcomes Associated With Early Vasculopathy in Patients With Chronic Lung Disease Undergoing Right Heart Catheterization.
Kosak Lopez E, Geller A, Leguizamon R … +10 more , Lam J, Srikulmontri T, Vera Ricaurte M, Siqueira Tavares de Melo M, Stavola T, Malin J, Lo KB, Martinez-Manzano JM, Pacheco E, Azmaiparashvili Z
Pulm Circ · 2026 Jun · PMID 42254876 · Full text
Pulmonary hypertension associated with chronic lung disease (PH-CLD) meets precapillary PH criteria. Less is known CLD patients with "early vasculopathy," a phenotype characterized by elevated pulmonary vascular resistan...
Pulmonary hypertension associated with chronic lung disease (PH-CLD) meets precapillary PH criteria. Less is known CLD patients with "early vasculopathy," a phenotype characterized by elevated pulmonary vascular resistance (PVR) and normal mean pulmonary artery pressure (mPAP). In this study, we aimed to determine the prevalence, characteristics, and outcomes of the early vasculopathy phenotype among CLD patients. We performed a retrospective cohort analysis of adults with chronic obstructive pulmonary disease or obstructive sleep apnea undergoing right heart catheterization between 2018 and 2023. Patients were categorized into three cohorts: non-PH cohort (mPAP ≤ 20 mmHg, PVR ≤ 2 WU), early vasculopathy cohort (mPAP ≤ 20 mmHg, PVR > 2 WU), and precapillary PH cohort (mPAP > 20 mmHg, PVR > 2 WU). The primary endpoint was a 1-year composite of mortality and major adverse cardiovascular events. Of 243 patients with CLD, 7.4% ( = 18) had early vasculopathy, whereas 35.4% ( = 86) had non-PH, and 57.2% ( = 139) had precapillary PH. Among CLD patients with early vasculopathy, the median age was 67 years, 56% were males, and 67% were African Americans. Compared to other groups, the early vasculopathy cohort had the lowest right atrial pressure, pulmonary artery wedge pressure, and cardiac output. On multivariable analysis, the early vasculopathy group was at increased risk of 1-year composite outcome compared to the non-PH group (adjusted hazard ratio [aHR] = 2.37, [95% confidence interval [CI] 1.11-5.06]; = 0.025). The association was attenuated after adjusting for left ventricular systolic dysfunction (aHR = 1.24, 95% CI [0.57-2.71]; = 0.581). Early vasculopathy is an uncommon hemodynamic pattern potentially associated with adverse outcomes seemingly mediated by subclinical heart failure.
Artificial Intelligence-Enhanced Electrocardiogram for Longitudinal Evaluation of Right Ventricular Dysfunction After Acute Pulmonary Embolism: Rational and Design of CURES-CARE Study.
Xu F, Lyu Y, Li Z … +22 more , Fan G, Ma J, Zhang X, Xu X, Qiao L, Liang Y, Qi Y, Shang J, Han J, Liu W, Gao C, Yan K, Li F, Wang X, Li Y, Cheng Z, Geng S, Li A, Yang P, Hong S, Zhai Z, Wang D
Pulm Circ · 2026 Jun · PMID 42254875 · Full text
Right ventricular dysfunction (RVD) due to pulmonary embolism (PE) is associated with poor prognosis and serves as a potential contributor to the occurrence and development of post-PE syndrome (PPES). However, the longit...
Right ventricular dysfunction (RVD) due to pulmonary embolism (PE) is associated with poor prognosis and serves as a potential contributor to the occurrence and development of post-PE syndrome (PPES). However, the longitudinal evolution of right ventricular function remains poorly understood due to the limited feasibility of repeated assessments. We aim to characterize the dynamic progression of RVD after acute PE using longitudinal electrocardiogram (ECG) signals analyzed with artificial intelligence (AI), together with selected biomarkers. China pUlmonary thromboembolism REgistry Study-CARioElectro (CURES-CARE) study, a prospective multicenter cohort study, will enroll 500 patients with intermediate- or high-risk acute PE from seven tertiary hospitals in China. All enrolled patients will be followed up for 12 months. During hospitalization, patients will undergo continuous ECG monitoring for 72 h, followed by daily ECG recording by a portable device throughout the follow-up period. Blood samples will be collected at admission, discharge, and at 3, 6, and 12 months. AI-based approaches will be applied to identify dynamic patterns in ECG signals and biomarker trajectories, integrated with biomarkers and imaging, to identify phenotypes of RVD progression and prognosis. CURES-CARE study will determine the incidence and risk factors of persistent RVD after acute PE, explore ECG-derived features associated with RV function, and evaluate the predictive value of multimodal data integration for long-term outcomes of PE. The study is expected to provide new insights into evolution of RVD, enable earlier risk stratification, and inform personalized management strategies to improve long-term outcomes in PE survivors. ClinicalTrials.gov Identifier: NCT06541353.
Towards a Safe Pathway to Biological Parenthood for Pulmonary Arterial Hypertension.
Ingram JD, Nelson SM, Brewis MJ … +2 more , Johnson MK, Church C
Pulm Circ · 2026 Jun · PMID 42164137 · Full text
Pulmonary arterial hypertension (PAH) carries a high risk of maternal morbidity and mortality in pregnancy, and current guidelines advise against conception. For women with PAH who wish to have genetically related childr...
Pulmonary arterial hypertension (PAH) carries a high risk of maternal morbidity and mortality in pregnancy, and current guidelines advise against conception. For women with PAH who wish to have genetically related children, in vitro fertilisation (IVF) combined with gestational surrogacy presents a viable and increasingly safe alternative. Advances in IVF protocols-including gonadotropin-releasing hormone (GnRH) antagonist stimulation, agonist trigger, personalised gonadotropin dosing, and elective embryo cryopreservation-have markedly reduced the risks of ovarian hyperstimulation syndrome and made oocyte retrieval feasible even in high-risk populations. The addition of pre-implantation genetic testing for monogenic disorders (PGT-M) enables the selection of embryos free of heritable PAH-related mutations, while pre-implantation genetic testing for aneuploidy (PGT-A) improves implantation rates and reduces miscarriage. For women who, due to personal, cultural or legal reasons, decline surrogacy and choose to carry a pregnancy themselves, a conservative approach-using natural cycle frozen embryo transfer and single euploid embryos-can reduce, but not eliminate, risk. The risks of pregnancy in this population are acknowledged to be high, and the guidelines continue to mandate against PAH patients becoming pregnant. However, there are alternative strategies developing which may allow biological parenthood without some of the inherent cardiopulmonary risk. This paper reviews the available evidence and proposes a clinical framework for offering IVF safely in this population, while advocating for the development of a dedicated registry and expert consensus to guide future care.
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