Pub Date : 2024-06-23eCollection Date: 2024-04-01DOI: 10.1002/pul2.12400
Marta Braksator, Magdalena Jachymek, Amena Rahmani, Katarzyna Widecka, Maciej Lewandowski, Łukasz Jodko, Małgorzata Peregud-Pogorzelska
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from impaired patency of the pulmonary arteries by a clot, and the treatment method of choice is pulmonary endarterectomy (PEA). In inoperable patients, balloon pulmonary angioplasty (BPA) is recommended, but we need to implement pharmacological bridge therapy to BPA in some cases. We report a case of a 38-year-old male diagnosed with CTEPH, disqualified from PEA due to comorbidity, who developed right ventricular (RV) failure. The case shows a complex pharmacological treatment method that can be successfully used as an effective bridge therapy to BPA in patients with CTEPH and severe RV dysfunction, disqualified from surgery.
{"title":"Nonsurgical treatment of a patient with decompensated right ventricular failure due to chronic thromboembolic pulmonary hypertension with proximal clot location-A case report.","authors":"Marta Braksator, Magdalena Jachymek, Amena Rahmani, Katarzyna Widecka, Maciej Lewandowski, Łukasz Jodko, Małgorzata Peregud-Pogorzelska","doi":"10.1002/pul2.12400","DOIUrl":"10.1002/pul2.12400","url":null,"abstract":"<p><p>Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from impaired patency of the pulmonary arteries by a clot, and the treatment method of choice is pulmonary endarterectomy (PEA). In inoperable patients, balloon pulmonary angioplasty (BPA) is recommended, but we need to implement pharmacological bridge therapy to BPA in some cases. We report a case of a 38-year-old male diagnosed with CTEPH, disqualified from PEA due to comorbidity, who developed right ventricular (RV) failure. The case shows a complex pharmacological treatment method that can be successfully used as an effective bridge therapy to BPA in patients with CTEPH and severe RV dysfunction, disqualified from surgery.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11194116/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443206","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}
Pub Date : 2024-06-22eCollection Date: 2024-04-01DOI: 10.1002/pul2.12405
Lucilla Piccari, Gabor Kovacs, Steve Jones, Hall Skaara, Claudia Roca Herms, Gabriela Silvina Bacchini Jeanneret, Melquiades Calzado Vinardell, Nuria Gonzalez-Rojas Guix, Miriam Fernandez Delgado, Héctor Gálvez García, David Schwicker
Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic, progressive condition. Yet little is known of the lived experiences, perspectives, priorities, and viewpoints of patients and carers living with PH-ILD. The Voice of the Patient meeting at the center of this qualitative research study aims to provide these difficult-to-obtain insights from a European perspective for the first time. The multistakeholder approach brought together four PH-ILD patients, three primary caregivers, two patient associations, clinical experts, sponsor representatives, and a facilitator. Of the six major themes identified in the thematic analysis, symptoms, and physical limitations were the most impactful. Shortness of breath was the most bothersome symptom affecting patients daily. Further symptoms included fatigue, cough, dizziness, syncope, edema, and palpitations. Physical limitations focused on reduced mobility, impacting patients' ability to perform daily tasks, hobbies, sports, and to enjoy travel. Existing antifibrotic and pulmonary arterial hypertension-targeted treatments were perceived as beneficial. However, despite advances in treatment, severe disease burdens and high unmet medical needs persist from the perspectives of patients. Most meaningful to patients' daily wellbeing was supplemental oxygen, enabling greater mobility. Patients and carers reported difficulties and barriers in navigating the healthcare system and obtaining adequate information to reduce their considerable uncertainties, documenting the substantial challenges that rare and complex conditions such as PH-ILD pose for routine clinical practice beyond PH expert centers and indicating an urgent need for high-quality patient- and clinician-directed information to support patient-centered care.
{"title":"The European Voice of the Patient living with pulmonary hypertension associated with interstitial lung disease: Diagnosis, symptoms, impacts, and treatments.","authors":"Lucilla Piccari, Gabor Kovacs, Steve Jones, Hall Skaara, Claudia Roca Herms, Gabriela Silvina Bacchini Jeanneret, Melquiades Calzado Vinardell, Nuria Gonzalez-Rojas Guix, Miriam Fernandez Delgado, Héctor Gálvez García, David Schwicker","doi":"10.1002/pul2.12405","DOIUrl":"10.1002/pul2.12405","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic, progressive condition. Yet little is known of the lived experiences, perspectives, priorities, and viewpoints of patients and carers living with PH-ILD. The Voice of the Patient meeting at the center of this qualitative research study aims to provide these difficult-to-obtain insights from a European perspective for the first time. The multistakeholder approach brought together four PH-ILD patients, three primary caregivers, two patient associations, clinical experts, sponsor representatives, and a facilitator. Of the six major themes identified in the thematic analysis, symptoms, and physical limitations were the most impactful. Shortness of breath was the most bothersome symptom affecting patients daily. Further symptoms included fatigue, cough, dizziness, syncope, edema, and palpitations. Physical limitations focused on reduced mobility, impacting patients' ability to perform daily tasks, hobbies, sports, and to enjoy travel. Existing antifibrotic and pulmonary arterial hypertension-targeted treatments were perceived as beneficial. However, despite advances in treatment, severe disease burdens and high unmet medical needs persist from the perspectives of patients. Most meaningful to patients' daily wellbeing was supplemental oxygen, enabling greater mobility. Patients and carers reported difficulties and barriers in navigating the healthcare system and obtaining adequate information to reduce their considerable uncertainties, documenting the substantial challenges that rare and complex conditions such as PH-ILD pose for routine clinical practice beyond PH expert centers and indicating an urgent need for high-quality patient- and clinician-directed information to support patient-centered care.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11193837/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443207","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}
Pub Date : 2024-06-19eCollection Date: 2024-04-01DOI: 10.1002/pul2.12390
Tracey Weiss, Dena R Ramey, Ngan Pham, Nazneen Fatima Shaikh, Dajun Tian, Xiaohui Zhao, Aimee M Near, Dominik Lautsch, Steven D Nathan
This retrospective study was conducted to evaluate all-cause healthcare resource utilization (HCRU) and costs in commercially insured patients living with pulmonary arterial hypertension (PAH) and explore end-of-life (EOL)-related HCRU and costs. Data from the IQVIA PharMetrics® Plus database (October 2014 to May 2020) were analyzed to identify adults (≥18 years) with PAH (PAH cohort) and those without PH (non-PH cohort). Patients were required to have data for ≥12 months before (baseline) and ≥6 months after (follow-up) the first observed PH diagnosis (index date) for PAH cohort or pseudo index date for non-PH cohort. A PAH EOL cohort was similarly constructed using a broader data window (October 2014 to March 2022) and ≥1 month of follow-up. Annualized all-cause HCRU and costs during follow-up were compared between PAH and non-PH cohorts after 1:1 matching on propensity scores derived from patient characteristics. EOL-related HCRU and costs were explored within 30 days and 6 months before the death date and estimated by a claims-based algorithm in PAH EOL cohort. The annual all-cause total ($183,616 vs. $20,212) and pharmacy ($115,926 vs. $7862; both p < 0.001) costs were 8 and 14 times higher, respectively, in the PAH cohort versus matched non-PH cohort (N = 386 for each). In PAH EOL cohort (N = 28), the mean EOL-related costs were $48,846 and $167,524 per patient within 30 days and 6 months before the estimated death, respectively. Hospitalizations contributed 58.8%-70.8% of the EOL-related costs. The study findings indicate substantial HCRU and costs for PAH. While pharmacy costs were one of the major sources, hospitalization was the primary driver for EOL-related costs.
{"title":"Excess healthcare resource utilization and costs for commercially insured patients with pulmonary arterial hypertension: A real-world data analysis.","authors":"Tracey Weiss, Dena R Ramey, Ngan Pham, Nazneen Fatima Shaikh, Dajun Tian, Xiaohui Zhao, Aimee M Near, Dominik Lautsch, Steven D Nathan","doi":"10.1002/pul2.12390","DOIUrl":"10.1002/pul2.12390","url":null,"abstract":"<p><p>This retrospective study was conducted to evaluate all-cause healthcare resource utilization (HCRU) and costs in commercially insured patients living with pulmonary arterial hypertension (PAH) and explore end-of-life (EOL)-related HCRU and costs. Data from the IQVIA PharMetrics® Plus database (October 2014 to May 2020) were analyzed to identify adults (≥18 years) with PAH (PAH cohort) and those without PH (non-PH cohort). Patients were required to have data for ≥12 months before (baseline) and ≥6 months after (follow-up) the first observed PH diagnosis (index date) for PAH cohort or pseudo index date for non-PH cohort. A PAH EOL cohort was similarly constructed using a broader data window (October 2014 to March 2022) and ≥1 month of follow-up. Annualized all-cause HCRU and costs during follow-up were compared between PAH and non-PH cohorts after 1:1 matching on propensity scores derived from patient characteristics. EOL-related HCRU and costs were explored within 30 days and 6 months before the death date and estimated by a claims-based algorithm in PAH EOL cohort. The annual all-cause total ($183,616 vs. $20,212) and pharmacy ($115,926 vs. $7862; both <i>p</i> < 0.001) costs were 8 and 14 times higher, respectively, in the PAH cohort versus matched non-PH cohort (<i>N</i> = 386 for each). In PAH EOL cohort (<i>N</i> = 28), the mean EOL-related costs were $48,846 and $167,524 per patient within 30 days and 6 months before the estimated death, respectively. Hospitalizations contributed 58.8%-70.8% of the EOL-related costs. The study findings indicate substantial HCRU and costs for PAH. While pharmacy costs were one of the major sources, hospitalization was the primary driver for EOL-related costs.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11186841/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141432663","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}
Pub Date : 2024-06-17eCollection Date: 2024-04-01DOI: 10.1002/pul2.12398
Dirk Habedank, Till Ittermann, Sabine Kaczmarek, Beate Stubbe, Alexander Heine, Anne Obst, Ralf Ewert
Parameters of cardiopulmonary exercise testing significantly discriminate between healthy subjects and patients with pulmonary hypertension (PH), also according to the new 2022 definition of pulmonary hypertension (mean pulmonary arterial pressure mPAP > 20 mmHg). The cut-offs indicating on PH were peakVO2 ≤ 16.7 mL/min/kg (Youden-Index YI = 0.79), petCO2@AT ≤ 34 mmHg (YI = 0.67), and VE/VCO2@AT ≤ 30 (YI = 0.76).
{"title":"Cardiopulmonary exercise testing and the 2022 definition of pulmonary hypertension.","authors":"Dirk Habedank, Till Ittermann, Sabine Kaczmarek, Beate Stubbe, Alexander Heine, Anne Obst, Ralf Ewert","doi":"10.1002/pul2.12398","DOIUrl":"10.1002/pul2.12398","url":null,"abstract":"<p><p>Parameters of cardiopulmonary exercise testing significantly discriminate between healthy subjects and patients with pulmonary hypertension (PH), also according to the new 2022 definition of pulmonary hypertension (mean pulmonary arterial pressure mPAP > 20 mmHg). The cut-offs indicating on PH were peakVO<sub>2</sub> ≤ 16.7 mL/min/kg (Youden-Index YI = 0.79), p<sub>et</sub>CO<sub>2</sub>@AT ≤ 34 mmHg (YI = 0.67), and VE/VCO<sub>2</sub>@AT ≤ 30 (YI = 0.76).</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11181771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141420650","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}
Pub Date : 2024-06-17eCollection Date: 2024-04-01DOI: 10.1002/pul2.12395
Carlos Jerjes-Sánchez, Alicia Ramírez-Rivera, Nayeli Zayas Hernandez, Guillermo Cueto Robledo, Humberto García-Aguilar, Pedro Gutiérrez-Fajardo, Mario Seoane García de León, Francisco Moreno Hoyos-Abril, Miguel Ernesto Beltrán Gámez, Jose Elizalde, Tomás Pulido Fccp, Julio Sandoval
Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.
{"title":"Demographic, hemodynamic characteristics, and therapeutic trends of pulmonary hypertension patients: The Pulmonary Hypertension Mexican registry (REMEHIP).","authors":"Carlos Jerjes-Sánchez, Alicia Ramírez-Rivera, Nayeli Zayas Hernandez, Guillermo Cueto Robledo, Humberto García-Aguilar, Pedro Gutiérrez-Fajardo, Mario Seoane García de León, Francisco Moreno Hoyos-Abril, Miguel Ernesto Beltrán Gámez, Jose Elizalde, Tomás Pulido Fccp, Julio Sandoval","doi":"10.1002/pul2.12395","DOIUrl":"10.1002/pul2.12395","url":null,"abstract":"<p><p>Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m<sup>2</sup>, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m<sup>2</sup>, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m<sup>2</sup>, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11181772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141420651","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}
Pub Date : 2024-06-17eCollection Date: 2024-04-01DOI: 10.1002/pul2.12401
Jose M Martinez Manzano, Alexander Prendergast, Tara John, Raul Leguizamon, Ian McLaren, Rasha Khan, Andrew Geller, Phuuwadith Wattanachayakul, John Malin, Simone A Jarrett, Kevin Bryan Lo, Sadia Benzaquen, Christian Witzke
Pulmonary hypertension (PH) is associated with adverse outcomes in chronic kidney disease (CKD) patients. Our study suggests mildly elevated pulmonary vascular resistance ( > 2 to ≤ 3) is independently associated with major adverse cardiovascular events at 1-year follow-up. Early diagnosis of precapillary PH in CKD patients can potentially improve clinical outcomes.
{"title":"Association of mildly elevated pulmonary vascular resistance with major cardiovascular events in pulmonary hypertension and chronic kidney disease: A retrospective cohort analysis.","authors":"Jose M Martinez Manzano, Alexander Prendergast, Tara John, Raul Leguizamon, Ian McLaren, Rasha Khan, Andrew Geller, Phuuwadith Wattanachayakul, John Malin, Simone A Jarrett, Kevin Bryan Lo, Sadia Benzaquen, Christian Witzke","doi":"10.1002/pul2.12401","DOIUrl":"10.1002/pul2.12401","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is associated with adverse outcomes in chronic kidney disease (CKD) patients. Our study suggests mildly elevated pulmonary vascular resistance ( > 2 to ≤ 3) is independently associated with major adverse cardiovascular events at 1-year follow-up. Early diagnosis of precapillary PH in CKD patients can potentially improve clinical outcomes.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11181768/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141420649","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}
Pub Date : 2024-06-14eCollection Date: 2024-04-01DOI: 10.1002/pul2.12381
Chermaine T Kwant, Frances S de Man, Harm J Bogaard, Anton Vonk Noordegraaf
This article examines technical use of Fitbit during an intervention for pulmonary hypertension (PAH)-patients. Technical issues with the device led to data being unavailable(37.5%). During intervention objective daily physical activity (DPA) decreased and subjective DPA increased. This emphasizes that an assessment of DPA in PAH requires incorporating both objective and subjective measurements.
{"title":"Evaluating the technical use of a Fitbit during an intervention for patients with pulmonary arterial hypertension with quality of life as primary endpoint: Lessons learned from the UPHILL study.","authors":"Chermaine T Kwant, Frances S de Man, Harm J Bogaard, Anton Vonk Noordegraaf","doi":"10.1002/pul2.12381","DOIUrl":"10.1002/pul2.12381","url":null,"abstract":"<p><p>This article examines technical use of Fitbit during an intervention for pulmonary hypertension (PAH)-patients. Technical issues with the device led to data being unavailable(37.5%). During intervention objective daily physical activity (DPA) decreased and subjective DPA increased. This emphasizes that an assessment of DPA in PAH requires incorporating both objective and subjective measurements.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11177024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331564","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}
Pub Date : 2024-06-12eCollection Date: 2024-04-01DOI: 10.1002/pul2.12386
Allan Lawrie, Kelly Chin, Yiu-Lian Fong, Cynthia Gargano, Xavier Gitton, Cheng He, David G Kiely, Li Zhou, Lihan Zhou, Bradley A Maron, Debbie Quinn, Stephan Rosenkranz, Dimitri Stamatiadis, Mark Toshner, Martin R Wilkins, Luke Howard, Ioana R Preston
A blood test identifying patients at increased risk of pulmonary hypertension (PH) could streamline the investigative pathway. The prospective, multicenter CIPHER study aimed to develop a microRNA-based signature for detecting PH in breathless patients and enrolled adults with a high suspicion of PH who had undergone right heart catheterization (RHC). The CIPHER-MRI study was added to assess the performance of this CIPHER signature in a population with low probability of having PH who underwent cardiac magnetic resonance imaging (cMRI) instead of RHC. The microRNA signature was developed using a penalized linear regression (LASSO) model. Data were modeled both with and without N-terminal pro-brain natriuretic peptide (NT-proBNP). Signature performance was assessed against predefined thresholds (lower 98.7% CI bound of ≥0.73 for sensitivity and ≥0.53 for specificity, based on a meta-analysis of echocardiographic data), using RHC as the true diagnosis. Overall, 926 CIPHER participants were screened and 888 were included in the analysis. Of 688 RHC-confirmed PH cases, approximately 40% were already receiving PH treatment. Fifty microRNA (from 311 investigated) were algorithmically selected to be included in the signature. Sensitivity [97.5% CI] of the signature was 0.85 [0.80-0.89] for microRNA-alone and 0.90 [0.86-0.93] for microRNA+NT-proBNP, and the corresponding specificities were 0.33 [0.24-0.44] and 0.28 [0.20-0.39]. Of 80 CIPHER-MRI participants with evaluable data, 7 were considered PH-positive by cMRI whereas 52 were considered PH-positive by the microRNA signature. Due to low specificity, the CIPHER miRNA-based signature for PH (either with or without NT-proBNP in model) did not meet the prespecified diagnostic threshold for the primary analysis.
{"title":"Two prospective, multicenter studies for the identification of biomarker signatures for early detection of pulmonary hypertension (PH): The CIPHER and CIPHER-MRI studies.","authors":"Allan Lawrie, Kelly Chin, Yiu-Lian Fong, Cynthia Gargano, Xavier Gitton, Cheng He, David G Kiely, Li Zhou, Lihan Zhou, Bradley A Maron, Debbie Quinn, Stephan Rosenkranz, Dimitri Stamatiadis, Mark Toshner, Martin R Wilkins, Luke Howard, Ioana R Preston","doi":"10.1002/pul2.12386","DOIUrl":"10.1002/pul2.12386","url":null,"abstract":"<p><p>A blood test identifying patients at increased risk of pulmonary hypertension (PH) could streamline the investigative pathway. The prospective, multicenter CIPHER study aimed to develop a microRNA-based signature for detecting PH in breathless patients and enrolled adults with a high suspicion of PH who had undergone right heart catheterization (RHC). The CIPHER-MRI study was added to assess the performance of this CIPHER signature in a population with low probability of having PH who underwent cardiac magnetic resonance imaging (cMRI) instead of RHC. The microRNA signature was developed using a penalized linear regression (LASSO) model. Data were modeled both with and without N-terminal pro-brain natriuretic peptide (NT-proBNP). Signature performance was assessed against predefined thresholds (lower 98.7% CI bound of ≥0.73 for sensitivity and ≥0.53 for specificity, based on a meta-analysis of echocardiographic data), using RHC as the true diagnosis. Overall, 926 CIPHER participants were screened and 888 were included in the analysis. Of 688 RHC-confirmed PH cases, approximately 40% were already receiving PH treatment. Fifty microRNA (from 311 investigated) were algorithmically selected to be included in the signature. Sensitivity [97.5% CI] of the signature was 0.85 [0.80-0.89] for microRNA-alone and 0.90 [0.86-0.93] for microRNA+NT-proBNP, and the corresponding specificities were 0.33 [0.24-0.44] and 0.28 [0.20-0.39]. Of 80 CIPHER-MRI participants with evaluable data, 7 were considered PH-positive by cMRI whereas 52 were considered PH-positive by the microRNA signature. Due to low specificity, the CIPHER miRNA-based signature for PH (either with or without NT-proBNP in model) did not meet the prespecified diagnostic threshold for the primary analysis.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11167234/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141311513","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}
Pub Date : 2024-06-07eCollection Date: 2024-04-01DOI: 10.1002/pul2.12396
Donna Steinbacher, Brian Murray, Thomas Devlin, Shannon S Carson, H James Ford
Prostacyclin therapy is a mainstay of the management of pulmonary arterial hypertension (PAH). Inhaled prostacyclins present safe and effective options for the management of PAH that limit systemic side effects. We describe the first reported case of life-threatening bronchospasm and acute respiratory failure associated with inhaled prostacyclin administration.
{"title":"Severe bronchospasm and acute respiratory failure associated with inhaled prostacyclin therapy.","authors":"Donna Steinbacher, Brian Murray, Thomas Devlin, Shannon S Carson, H James Ford","doi":"10.1002/pul2.12396","DOIUrl":"10.1002/pul2.12396","url":null,"abstract":"<p><p>Prostacyclin therapy is a mainstay of the management of pulmonary arterial hypertension (PAH). Inhaled prostacyclins present safe and effective options for the management of PAH that limit systemic side effects. We describe the first reported case of life-threatening bronchospasm and acute respiratory failure associated with inhaled prostacyclin administration.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11157496/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141296673","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}