Pulmonary Circulation最新文献

{"title":"A Patient With Pulmonary Hypertension Carrying <i>FLNA</i> Loss-of-Function Variant.","authors":"Zongye Cai, Shuangxiang Lin, Nan Jin, Yahong Fu, Ying Zhang, Jifang Cheng, Yue Mao, Yuanshi Li, David Montani, Jun Jiang","doi":"10.1002/pul2.70110","DOIUrl":"10.1002/pul2.70110","url":null,"abstract":"<p><p>In a recent study conducted by Laura Stourm et al., the authors identified that patients with pulmonary hypertension (PH) harboring loss-of-function variants in the <i>Filamin A (FLNA)</i> gene exhibit a unique spectrum of phenotypes. These include pulmonary involvement with lung parenchymal abnormalities and emphysema, as well as a range of extrapulmonary manifestations such as dysmorphic facial features, epilepsy, congenital heart defects, valvular and aortic diseases, thrombocytopenia, and periventricular nodular heterotopia (PVNH). Based on these findings, the study advocates for <i>FLNA</i> genetic screening in patients with PH who present with these specific phenotypic features. Here, we describe a clinical case that aligns closely with the observations reported in their study.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70110"},"PeriodicalIF":2.2,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240676/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144609172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"2025 SELECT ABSTRACTS FROM THE 18^th ANNUAL INTERNATIONAL CONFERENCE ON NEONATAL AND CHILDHOOD PULMONARY VASCULAR DISEASE.","authors":"","doi":"10.1002/pul2.70114","DOIUrl":"https://doi.org/10.1002/pul2.70114","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70114"},"PeriodicalIF":2.2,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12237822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta-Registry.","authors":"Arun Jose, Athiththan Yogeswaran, Meike Fuenderich, David Kiely, Andrew J Sweatt, Roham T Zamanian, Paul M Hassoun, Antoine Mouawad, Aparna Balasubramanian, Martin Wilkins, Allan Lawrie, Luke Howard, Sandeep Sahay, Horst Olschewski, Gabor Kovacs, Khaled Saleh, Hani Sabbour, Christina A Eichstaedt, Ekkehard Grünig, George Giannakoulas, Alexandra Arvanitaki, Yuriy Sirenko, Olena Torbas, Hector Cajigas, Robert Frantz, Laura Scelsi, Stefano Ghio, Raphael W Majeed, Jochen Wilhelm, Hossein Ardeschir Ghofrani, Friedrich Grimminger, Khodr Tello, Jean Elwing, Werner Seeger","doi":"10.1002/pul2.70121","DOIUrl":"10.1002/pul2.70121","url":null,"abstract":"<p><p>Portopulmonary hypertension (PoPH), a type of pulmonary arterial hypertension (PAH) in patients with liver disease, is associated with high morbidity and mortality. The relationship between cardiopulmonary hemodynamics, PAH therapy, and survival in PoPH remains unclear. We performed a retrospective cohort study of PoPH patients from the international pulmonary hypertension (PH) meta-registry, PVRI GoDeep. PAH was defined by a mean pulmonary arterial pressure > 20 mmHg, pulmonary arterial wedge pressure ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) > 2 Wood Units. PoPH diagnoses were assigned by each center's PH specialist based on international guidelines at the time of enrollment. 246 incident PoPH patients met eligibility criteria and were included in the analysis, equally split between males (51%) and females (49%), with a median age of 54 years. When compared to both patients with IPAH and those with other subtypes of PAH (not classified as PoPH or IPAH), those with PoPH had significantly lower 5-year survival rates (46% vs. 68% vs. 65%, log-rank <i>p</i> < 0.001). Amongst the PoPH patients, however, there was no significant difference in 5-year survival when dichotomized by disease severity, either by a PVR of 5 Wood Units or a CI of 2.5 L/min/m². Treatment of the PoPH patients with PAH-targeted therapies was associated with significantly higher 5-year survival rates compared to those not receiving such treatments, as shown by Kaplan-Meier analysis. This survival benefit was observed for PDE5i (50% vs. 34%, log-rank <i>p</i> = 0.029), ERA (58% vs. 34%, log-rank <i>p</i> < 0.001), and the combination of PDE5i and/or ERA (51% vs. 22%, log-rank <i>p</i> < 0.001), as well as any PAH-targeting treatment (50% vs. 26%, log-rank <i>p</i> = 0.007). Corresponding survival advantage was noted when including only PoPH patients with MELD Score ≥ 13. PoPH is a disease with significantly worse long-term survival than other PAH subtypes, but targeted PAH therapy is associated with a robust survival benefit. Survival did not differ across high-risk PVR and cardiac index thresholds, suggesting the factors that influence prognosis and survival in PoPH may be unique as compared to other PAH subtypes, and warrant further investigation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70121"},"PeriodicalIF":2.5,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12237829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601361","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Practical Considerations for Managing Patients on Tyvaso DPI (Treprostinil Inhalation Powder).","authors":"Jennifer H Keeley, Jacqueline Skarre, Lori Reed, Loida A Johnson, Catherine Falardeau, Brittany N J Davis, Manisit Das, Howard Castillo, Susanne McDevitt","doi":"10.1002/pul2.70119","DOIUrl":"10.1002/pul2.70119","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival. Inhaled treprostinil, a prostacyclin analog, has been approved for both PAH and PH-ILD in two formulations: Tyvaso (treprostinil nebulizer) and the more recent Tyvaso DPI (treprostinil dry powder inhaler) [United Therapeutics]. Both formulations deliver therapy directly to the lung vasculature, minimizing the risk of ventilation-perfusion mismatch, reducing systemic exposure, and decreasing the incidence of adverse events commonly associated with parenteral and oral prostacyclin formulations. While practical recommendations for the treprostinil nebulizer have been previously published, Tyvaso DPI provides a well-tolerated, convenient administration option for patients requiring prostacyclin therapy. This review provides an overview of inhaled prostacyclin therapy with a focus on practical considerations for managing PAH and PH-ILD patients treated with Tyvaso DPI. Recommendations from a panel of pulmonary hypertension advanced practice providers include patient selection, education, communication, onboarding and monitoring, transition and titration, side effect mitigation, and the availability of clinician- and patient-facing resources.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70119"},"PeriodicalIF":2.2,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12224286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144560964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outpatient Intravenous Dobutamine as Right Ventricular Support During Inhaled Prostacyclin Uptitration in Severe PH-ILD.","authors":"A Kumar, Siddharth V Ramanan, Brett Carollo, Kristen Swanson, Raj Parikh","doi":"10.1002/pul2.70111","DOIUrl":"10.1002/pul2.70111","url":null,"abstract":"<p><p>PH-ILD carries a poor prognosis, particularly with PVR > 5 WU. The INCREASE trial demonstrated the successful use of inhaled treprostinil, but slow uptitration delays the effects. Our study suggests outpatient IV dobutamine as interim support, improving contractility and reducing PVR during uptitration.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 3","pages":"e70111"},"PeriodicalIF":2.2,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12215808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144554305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exercise-Induced Oxygen Desaturation and Heart Rate Response During 6-Min Walk Test Predict Pulmonary Hypertension in Exertional Dyspnea: A Retrospective Cohort Study.","authors":"Haojie Zhang, Menghuan Yan, Feng Li, Qi Chen, Rui Lu, Ziyu Wang, Xuan Zheng, Gangcheng Zhang","doi":"10.1002/pul2.70120","DOIUrl":"10.1002/pul2.70120","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a life-threatening condition frequently associated with exertional dyspnea. It remains diagnostically challenging due to limitations in current screening modalities. While the 6-min walk test (6MWT) has been applied for risk stratification in confirmed PH, its potential role in screening remains unexplored. This retrospective cohort study investigated the diagnostic utility of 6MWT-derived parameters in 180 patients with exertional dyspnea. PH diagnosis was confirmed by right heart catheterization with the definition of mean pulmonary artery pressure > 20 mmHg. Among 79 PH patients (43.9%), a significantly reduced 6-min walk distance (6MWD) was observed compared to non-PH patients (469.5 ± 106.4 m vs. 509.8 ± 74.9 m, <i>p</i> = 0.019). Continuous physiological monitoring revealed that the SpO₂ trough and the heart rate (HR) peak occurred at different time points during 6MWT. Propensity score-matched case-control analysis further demonstrated greater exercise-induced desaturation of SpO₂ from the rest to minimal levels (ΔSpO₂_rest _-min: 9 ± 9% vs. 4 ± 6%, <i>p</i> < 0.001) and exaggerated HR response from the rest to maximal levels (ΔHR_max _-rest: 51±21bpm vs. 34±14bpm, <i>p</i> < 0.001) in PH patients. Multivariable analysis identified ΔSpO₂_rest _-min ≥ 5% (AUC = 0.715, 95% CI: 0.640-0.852; <i>p</i> < 0.001) and ΔHR_max _-rest ≥ 42 bpm (AUC = 0.740, 95% CI: 0.656-0.823; <i>p</i> < 0.001) as independent predictors of PH. The number of these predictors discriminated the risk of PH in dyspneic patients. A risk-stratification model incorporating these thresholds demonstrated improved predictive value for PH screening, with a C-statistic of 0.786 (95% CI: 0.710-0.863, <i>p</i> < 0.001). These findings suggest that parameters derived from the 6MWT, particularly exercise-induced SpO₂ desaturation and HR response, may facilitate noninvasive PH screening in exertional dyspneic patients.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70120"},"PeriodicalIF":2.2,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12197869/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypopituitarism Induced by Continuous Infusion of PGI2 Analogues: A Case Series and the Role of ACTH Screening and Hydrocortisone Treatment.","authors":"Taijyu Satoh, Yuichi Tamura, Noriaki Takama, Hiromi Matsubara, Nobuhiro Tanabe, Takumi Inami, Takahiro Hiraide, Kohtaro Abe, Yoshihiro Dohi, Yoshito Ogihara, Takeshi Ogo, Shiro Adachi, Kazuhiko Nakazato, Ichizo Tsujino, Hideki Ota, Kohei Komaru, Haruka Sato, Yuta Tezuka, Yoshikiyo Ono, Rika Suda, Kazuya Hosokawa, Sarasa Isobe, Takatoyo Kiko, Yuki Koga, Junichi Nakamura, Koichiro Sugimura, Masaru Hatano, Yoshihiro Fukumoto, Satoshi Yasuda","doi":"10.1002/pul2.70116","DOIUrl":"10.1002/pul2.70116","url":null,"abstract":"<p><p>Hypopituitarism has been reported in patients receiving continuous infusions of prostaglandin I2 (PGI2) analogues for pulmonary hypertension (PH). However, these patients' clinical characteristics, treatment, and prognoses remain unclear. This retrospective multicentre study included 22 patients who developed hypopituitarism while on continuous PGI2 analogue infusion between 1999 and 2021. All patients were female, and idiopathic pulmonary arterial hypertension was the most common underlying condition (63.6%). Their mean age was 38.8 ± 7.9 years. Epoprostenol was the predominant PGI2 analogue used (90.9%). At the time of hypopituitarism onset, the median PGI2 dose was 67.2 ng/kg/min (31.8-88.7 ng/kg/min), and the median treatment duration was 889.0 days (450.5-1941.5 days), suggesting that hypopituitarism occurred independent of its dose or treatment duration. Diagnoses were based on decreased adrenocorticotropic hormone levels. The hypopituitarism classification revealed isolated pituitary dysfunction in 54.5% of the cases, partial dysfunction in 18.1%, and complete dysfunction in 27.2%. Most cases could be managed without requiring specific therapies. After hypopituitarism onset, 63.6% of the patients continued to receive the same PGI2 analogue. Hydrocortisone therapy was administered to 81.8% of the patients, leading to clinical stabilisation. No deaths were reported. In conclusions, hypopituitarism may occur during continuous PGI2 analogue infusion for PH, irrespective of its dose or treatment duration. Initiating hydrocortisone therapy may be important for stabilising the clinical course.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70116"},"PeriodicalIF":2.2,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12188693/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144497960","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anxiety and Depression Screening of Youth in Pediatric Pulmonary Hypertension Clinic: A Multi-Center, Cross-Sectional Study.","authors":"Claire Parker, Elise Whalen, Michael A Smith, Jasmine Becerra, Leah Stevens, Catherine M Avitabile, Anna Brown, Michelle Cash, Emma Olson Jackson, Julia McSweeney, Kathleen Miller-Reed, Janette T Reyes, Cathy Sheppard, Mary P Mullen","doi":"10.1002/pul2.70117","DOIUrl":"10.1002/pul2.70117","url":null,"abstract":"<p><p>Children with chronic diseases, including pulmonary hypertension (PH), have an increased risk of anxiety and depression (AD), impacting mental health (MH), and quality of life (QoL). We sought to characterize the prevalence of AD in pediatric PH and identify associated factors. We developed a prospective cross-sectional study with 10 Pediatric Pulmonary Hypertension Network (PPHNet) centers. Eligible subjects aged 12-21, diagnosed with PH, and English or Spanish speaking, completed validated AD screening questionnaires during routine outpatient clinic visits. Caregivers provided socioeconomic status (SES) data and MH history via survey. Patient demographics and clinical characteristics were analyzed using standard descriptive statistics. Eighty-eight patients were enrolled (female = 54, 61%). Forty-six (51.7%) identified at least mild symptoms of AD. Females were more likely to report AD than males (OR 2.67, 95% CI 1.11-6.61, <i>p</i> = 0.030). There were no significant associations between AD and PH severity, MH history, family dynamics, SES status, race, or ethnicity. Twenty-seven of those patients (58.7%) received MH education/counseling by MH professionals; ten (21.7%) were referred to MH providers, and nine patients (19.6%) were assessed for suicide safety. Adolescents with PH have a high prevalence of AD. Female patients had increased AD compared to male patients; no other predictors were linked to the prevalence of AD. Routine AD screening should be integrated into outpatient PH clinic visits with a focus on psychosocial support for young females diagnosed with PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70117"},"PeriodicalIF":2.2,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12187978/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144497959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploring Providers' Behaviors, Attitudes, and Preferences on the Treatment of Pulmonary Arterial Hypertension With Endothelin Receptor Antagonist (ERA) + Phosphodiesterase-5 Inhibitors (PDE5i).","authors":"Nicholas A Kolaitis, Martha Kingman, Melisa Wilson, Gabriela Gomez Rendon, David Lopez, Carly J Paoli, Mohammad Rahman, Ashley Martin, November McGarvey, Abraham Lee, Lana Melendres-Groves","doi":"10.1002/pul2.70113","DOIUrl":"10.1002/pul2.70113","url":null,"abstract":"<p><p>This study aims to understand healthcare providers' (HCPs) decision to adopt double combination therapy with ERA + PDE5i for pulmonary arterial hypertension (PAH), and to explore whether a single tablet combination therapy (STCT) might increase adoption practices. 195 US HCPs completed a survey evaluating their PAH treatment preferences. HCPs' willingness to use double combination ERA + PDE5i was assessed using a discrete choice experiment (DCE). The sample predominantly included physicians (73.3%) from centers of excellence (63.1%), with a mean of 117.4 PAH patients treated in the past year. Key factors influencing ERA + PDE5i adoption in the DCE were the patient's current treatment (17.9), PAH etiology (16.2), existing comorbidities (14.1), and history of side effects (12.7), with higher scores indicating stronger preference. HCPs were more likely to select ERA + PDE5i for patients currently on PDE5i monotherapy, with idiopathic PAH, and without comorbidities or a history of side effects. Regarding STCT, most HCPs reported that it would allow them to initiate ERA + PDE5i sooner (76.4%) and improve patient compliance (82.6%). However, concerns regarding cost/insurance issues (63.6%) and a history of side effects (50.8%) were identified as limitations to adopting STCT. Patients' current therapy, the cause of PAH, comorbidities, and side effects are key factors influencing whether US providers are willing to treat them with ERA + PDE5i. Providers perceive that STCT may help HCPs initiate ERA + PDE5i sooner, improve compliance, and simplify initiation of upfront double therapy and delivery of triple therapy. Addressing cost and insurance barriers will be critical to realizing these potential benefits.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70113"},"PeriodicalIF":2.2,"publicationDate":"2025-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12182709/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric Use of Inhaled Iloprost.","authors":"Stephanie S Handler, Steven H Abman, Nidhy P Varghese, D Dunbar Ivy","doi":"10.1002/pul2.70115","DOIUrl":"10.1002/pul2.70115","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70115"},"PeriodicalIF":2.2,"publicationDate":"2025-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12182708/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}