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Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension with bronchial obstruction by a carcinoid tumor Pulm. Circ. (IF 2.6) Pub Date : 2024-03-14 Yuki Monden, Dai Une, Sho Mitsumune, Hiroto Shimokawahara, Hirofumi Okada, Kenji Yoshida, Shutaro Kato, Suzuka Kamaguchi, Mikizo Nakai, Motomi Ando
Pulmonary endarterectomy (PEA) is a standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH combined with bronchial obstruction by a tumor is rare but should be assessed carefully because PEA for obstructed segments can be less therapeutic and make the subsequent surgical resection challenging. This report describes a case of CTEPH with bronchial obstruction by a typical
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Unlocking the potential of genetic research in pulmonary arterial hypertension: Insights from clinicians, researchers, and study team Pulm. Circ. (IF 2.6) Pub Date : 2024-03-12 Emilia M. Swietlik, Michaela Fay, Nicholas W. Morrell
Genetic research and testing are increasingly important for understanding and treating pulmonary arterial hypertension. We aimed to explore how attitudes toward genetic research among clinical and research teams impacted the engagement in genetic research and the integration of genetic insights into clinical practice. We conducted 53 semistructured interviews and focus groups with patients, clinicians
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Safety of inhaled nitric oxide withdrawal in severe chronic pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-03-10 Gregorio Miguel Pérez-Peñate, Gabriel Juliá-Serdá, Helena Galván-Fernández, Desireé Alemán-Segura, Fernando León-Marrero, Antonio Garcia-Quintana, Iñigo Rúa-Fernández de Larrinoa, José Ramón Ortega-Trujillo, Miguel Ángel Gómez-Sánchez
Inhaled nitric oxide (iNO) is a potent and selective pulmonary vasodilator with a safety concern due to rebound pulmonary hypertension (PH) associated with its withdrawal. We report short-term pulsed iNO in patients with severe pulmonary arterial hypertension (PAH) and nonoperable chronic thromboembolic PH (nCTEPH). This is a retrospective analysis of 33 patients: 22 with PAH and 11 with nCTEPH. We
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Evaluating the efficacy and safety of oral triple sequential combination therapy for treating patients with pulmonary arterial hypertension: A multicenter retrospective study Pulm. Circ. (IF 2.6) Pub Date : 2024-03-10 Qin-Hua Zhao, Jun Chen, Fa-Dong Chen, Hong-Yun Ruan, Wei Zhang, Yan-Li Zhou, Qi-Qi Wang, Xiao-Ling Xu, Ke-Fu Feng, Jian-Zhou Guo, Su-Gang Gong, Rui-Feng Zhang, Lan Wang
This study aimed to evaluate the effectiveness and safety of an oral sequential triple combination therapy with selexipag after dual combination therapy with endothelin receptor antagonist (ERA) and phosphodiesterase-5 inhibitor (PDE5I)/riociguat in pulmonary arterial hypertension (PAH) patients. A total of 192 PAH patients from 10 centers had received oral sequential selexipag therapy after being
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Actigraphy methodology in the Kids Mod PAH trial: Physical activity as a functional endpoint in pediatric clinical trials Pulm. Circ. (IF 2.6) Pub Date : 2024-03-08 Catherine M. Avitabile, Usha S. Krishnan, Delphine Yung, Stephanie S. Handler, Nidhy Varghese, Angela Bates, Jeff Fineman, Rachel Sullivan, Grace Friere, Eric Austin, Mary P. Mullen, Carol Pereira, Eric J. Christensen, Gayane Yenokyan, Joseph M. Collaco, Steven H. Abman, Lew Romer, D. Dunbar Ivy, Erika B. Rosenzweig
Pulmonary vasodilator treatment can improve hemodynamics, right ventricular function, symptoms, and survival in pediatric pulmonary hypertension (PH). However, clinical trial data are lacking due to many constraints. One major limitation is the lack of relevant trial endpoints reflective of hemodynamics or functional status in patients in whom standard exercise testing is impractical, unreliable, or
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An inverse causal relationship between serum 25-hydroxyvitamin D levels and pulmonary hypertension: A two-sample Mendelian randomization study Pulm. Circ. (IF 2.6) Pub Date : 2024-03-07 Ce Chao, Min Wang, Kun Mei, Chao Ma, Yongxiang Qian, Xiaoying Zhang
Observational studies have confirmed that 25-hydroxyvitamin D (25(OH)D) is associated with pulmonary hypertension (PH), but the causal association between each other is unclear. Therefore, Mendelian randomization (MR) method was performed to validate the causal association between PH and serum 25(OH)D levels. The summary data for 25(OH)D and PH were from the National Human Genome Research Institute-European
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Pulmonary vascular pressure respiratory swings in COPD and ILD candidates for lung transplantation: Large but different Pulm. Circ. (IF 2.6) Pub Date : 2024-03-06 Juan C. Grignola, Alvaro Calabuig, Pedro Trujillo, Carles Bravo, Fernando Azpiroz, Manuel López Messeguer, Enric Domingo
We analyzed the effect of respiratory swings on interpreting intravascular pulmonary vascular pressures (PVPs) in chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) candidates for lung transplantation (LTx) and the role of the alterations in pulmonary function tests on the dynamic respiratory variations. Twenty-eight consecutive patients were included. All patients underwent
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Change in health-related quality of life at early follow-up in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-02-27 Bodil Ivarsson, Anders Johansson, Barbro Kjellström
Symptoms associated with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) impact patient's health-related quality of life (HRQoL). Studies on change and if a minimal clinically important difference (MCID) in HRQoL is reached within a year after diagnosis are lacking. The aim was to investigate the change in HRQoL as well as the proportion of patients that
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Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience Pulm. Circ. (IF 2.6) Pub Date : 2024-02-27 Amaya Martínez-Meñaca, Alejandro Cruz-Utrilla, Víctor Manuel Mora-Cuesta, Raquel Luna-López, Teresa Segura-de la Cal, Ángela Flox-Camacho, Pilar Alonso-Lecue, Pilar Escribano-Subias, José Manuel Cifrián-Martínez
A simplified 4-strata risk stratification approach based on three variables is widespread in pulmonary arterial hypertension (PAH) at follow-up. This study aimed to assess the impact of replacing the 6-min walk test (6MWT) with the peak 02 uptake evaluated by the cardiopulmonary exercise test (CPET) on risk stratification by this scale. We included 180 prevalent patients with PAH from two reference
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Recognition, diagnosis, and operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross-sectional scientific survey (CLARITY) Pulm. Circ. (IF 2.6) Pub Date : 2024-02-21 Grzegorz Kopeć, Paul Forfia, Kohtaro Abe, Amélie Beaudet, Virginie Gressin, Mitja Jevnikar, Catherina Meijer, Yan Zhi Tan, Olga Moiseeva, Karen Sheares, Nika Skoro-Sajer, Mario Terra-Filho, Helen Whitford, Zhenguo Zhai, Gustavo A. Heresi
Early recognition and diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is crucial for improving prognosis and reducing the disease burden. Established clinical practice guidelines describe interventions for the diagnosis and evaluation of CTEPH, yet limited insight remains into clinical practice variation and barriers to care. The CTEPH global cross-sectional scientific survey (CLARITY)
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The beneficial effects of balloon pulmonary angioplasty for patients with chronic thromboembolic pulmonary hypertension are accompanied by increased body mass index and improved nutritional status Pulm. Circ. (IF 2.6) Pub Date : 2024-02-17 Shinya Fujii, Shinya Nagayoshi, Takashi Miyamoto, Kazuo Ogawa, Michihiro Yoshimura
Although balloon pulmonary angioplasty (BPA) improves pulmonary hypertension and the prognosis of patients with chronic thromboembolic pulmonary hypertension (CTEPH), subsequent changes in body mass index (BMI), nutritional status, and appetite have not been fully investigated. This retrospective study aimed to clarify changes in BMI, nutritional status, and appetite after BPA. Fifty-two consecutive
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Understanding what drives genetic study participation: Perspectives of patients, carers, and relatives Pulm. Circ. (IF 2.6) Pub Date : 2024-02-15 Emilia M. Swietlik, Michaela Fay, Nicholas W. Morrell
Genetic research's growing importance in understanding pulmonary arterial hypertension (PAH) and developing effective treatments prompted the RAPID-PAH study. This study sought feedback from stakeholders who participated in two genomic studies to enhance genetic study delivery and clinical integration. Stakeholders from nine UK PH centres, representing various roles, ages, genders, and mutation statuses
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Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-02-12 Justin J. Kochanski, Jeffrey A. Feinstein, Michelle Ogawa, Victor Ritter, Rachel K. Hopper, Gregory T. Adamson
Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single-center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular
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Pulmonary artery wave intensity analysis in pulmonary hypertension associated with heart failure and reduced left ventricular ejection fraction Pulm. Circ. (IF 2.6) Pub Date : 2024-02-12 Ivan H. W. Yim, Kim H. Parker, Nigel E. Drury, Hoong Sern Lim
Wave intensity analysis (WIA) uses simultaneous changes in pressure and flow velocity to determine wave energy, type, and timing of traveling waves in the circulation. In this study, we characterized wave propagation in the pulmonary artery in patients with pulmonary hypertension associated with left-sided heart disease (PHLHD) and the effects of dobutamine. During right heart catheterization, pressure
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Hemodynamic response to inhaled nitric oxide in patients with pulmonary hypertension and chronic kidney disease: A retrospective cohort study Pulm. Circ. (IF 2.6) Pub Date : 2024-02-10 Kathryn T. del Valle, Michael J. Krowka, Carrie A. Schinstock, Karl A. Nath, Charles D. Burger, Yogesh N. Reddy, Robert P. Frantz, Y. S. Prakash, Hilary M. DuBrock
Pulmonary hypertension (PH) associated with chronic kidney disease (CKD) (PH-CKD) affects approximately 20%–40% of CKD patients and is associated with increased morbidity and mortality. PH and CKD are both pathophysiologically associated with nitric oxide (NO) deficiency. The NO pathway, an important therapeutic domain in pulmonary arterial hypertension (PAH), is an intriguing but unexplored target
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Using PAH-SYMPACT to assess quality of life in patients with pulmonary hypertension associated with chronic lung disease Pulm. Circ. (IF 2.6) Pub Date : 2024-02-10 Morgan E. Bailey, Louise Durst, Hector R. Cajigas, Garvan C. Kane, Michael J. Krowka, Sudhir S. Kushwaha, Robert B. McCully, Joseph G. Murphy, Yogesh N. Reddy, Robert P. Frantz, Hilary M. DuBrock
Chronic lung disease (CLD) is the second leading cause of pulmonary hypertension (PH) and is associated with significant morbidity and mortality. Although PH associated with CLD (PH-CLD) leads to impaired health-related quality of life (HRQOL), there are no validated tools to assess HRQOL in PH-CLD. The Pulmonary Arterial Hypertension–Symptoms and Impact Questionnaire (PAH-SYMPACT) is an HRQOL instrument
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Clinical evaluation of code-based algorithms to identify patients with pulmonary arterial hypertension in healthcare databases Pulm. Circ. (IF 2.6) Pub Date : 2024-02-08 Eva-Maria Didden, Di Lu, Andrew Hsi, Monika Brand, Haley Hedlin, Roham T. Zamanian
Pulmonary arterial hypertension (PAH) is a rare subgroup of pulmonary hypertension (PH). Claims and administrative databases can be particularly important for research in rare diseases; however, there is a lack of validated algorithms to identify PAH patients using administrative codes. We aimed to measure the accuracy of code-based PAH algorithms against the true clinical diagnosis by right heart
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Structured evaluation of unclear dyspnea–An attempt to shorten the diagnostic delay in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-02-04 Salaheldin Ahmed, Abdulla Ahmed, Göran Rådegran
Pulmonary hypertension (PH) is a common condition, present in up to 10% in individuals aged >65 years.1 Whitin this entity, pulmonary arterial hypertension (PAH; group I PH) and chronic thromboembolic pulmonary hypertension (CTEPH; group 4 PH) comprise two rare, but detrimental conditions characterized by pulmonary vasculopathies and remodeling of pulmonary arteries, leading ultimately to right heart
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Experimental Schistosoma haematobium pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-02-01 Biruk Kassa, Dara C. Fonseca-Balladares, Rahul Kumar, Michael H. Lee, Claudia Mickael, Linda Sanders, Kevin Nolan, Brian B. Graham
Whether all Schistosoma species cause pulmonary hypertension (PH) is unclear. Experimentally exposing mice to Schistosoma haematobium eggs caused PH, which was less severe than that induced by S. mansoni exposure. These findings align with the relatively uncommon reports of pulmonary arterial hypertension associated with S. haematobium.
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Patients with CTEPH and mild hemodynamic severity of disease improve to a similar level of exercise capacity after pulmonary endarterectomy compared to patients with severe hemodynamic disease Pulm. Circ. (IF 2.6) Pub Date : 2024-01-24 Coen van Kan, Jelco Tramper, Paul Bresser, Lilian J. Meijboom, Petr Symersky, Jacobus A. Winkelman, Esther J. Nossent, Jurjan Aman, Harm Jan Bogaard, Anton Vonk Noordegraaf, Josien van Es
The correlation between hemodynamics and degree of pulmonary vascular obstruction (PVO) is known to be poor in chronic thromboembolic pulmonary hypertension (CTEPH), which makes the selection of patients eligible for pulmonary endarterectomy (PEA) challenging. It can be postulated that patients with similar PVO but different hemodynamic severity have different postoperative hemodynamics and exercise
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Pulmonary hypertension patient perspectives toward pulmonary rehabilitation Pulm. Circ. (IF 2.6) Pub Date : 2024-01-24 Morgan Bailey, Hilary M. DuBrock
Pulmonary rehabilitation (PR) is a supervised exercise program for patients with chronic lung disease. Among patients with pulmonary hypertension (PH), PR has been shown to improve both quality of life and exercise capacity. The purpose of this study was to assess the prevalence of PR participation among PH patients, patient perspectives regarding PR, and to identify potential barriers to PR participation
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Exercise and pulsatile pulmonary vascular loading in chronic thromboembolic pulmonary disease Pulm. Circ. (IF 2.6) Pub Date : 2024-01-20 Sinan Osman, Natasha R. Girdharry, Elizabeth Karvasarski, Robert F. Bentley, Stephen P. Wright, Nadia Sharif, Micheal McInnis, John T. Granton, Marc dePerrot, Susanna Mak
Chronic thromboembolic pulmonary disease (CTEPD) is characterized by organized nonresolving thrombi in pulmonary arteries (PA). In CTEPD with pulmonary hypertension (PH), chronic thromboembolic PH (CTEPH), early wave reflection results in abnormalities of pulsatile afterload and augmented PA pressures. We hypothesized that exercise during right heart catheterization (RHC) would elicit more frequent
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Impact of body position on hemodynamic measurements during exercise: A tale of two bikes Pulm. Circ. (IF 2.6) Pub Date : 2024-01-11 Pradhab Kirupaharan, James Lane, Celia Melillo, Deborah Paul, Alla Amoushref, Sami Al Abdi, Adriano R. Tonelli
The addition of exercise testing during right heart catheterization (RHC) is often required to accurately diagnose causes of exercise intolerance like early pulmonary vascular disease, occult left heart disease, and preload insufficiency. We tested the influence of body position (supine vs. seated) on hemodynamic classification both at rest and during exercise. We enrolled patients with exercise intolerance
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BMPR2 mutation and clinical response to imatinib in a case of heritable pulmonary arterial hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-01-10 Shine Kumar, Lalitha Biswas, Anju Choorakottayil Pushkaran, Raman Krishna Kumar
Bone morphogenetic protein receptor 2 (BMPR2) mutation is the most common gene mutation implicated in the pathogenesis of pulmonary arterial hypertension (PAH). We describe, for the first time, an excellent clinical response to tyrosine kinase inhibitor imatinib in a patient with heritable PAH from BMPR2 mutation.
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Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative—Group 3 Pulmonary Hypertension Pulm. Circ. (IF 2.6) Pub Date : 2024-01-10 Oksana A. Shlobin, Eric Shen, Stephen J. Wort, Lucilla Piccari, John A. Scandurra, Paul M. Hassoun, Sylvia M. Nikkho, Steven D. Nathan
Pulmonary hypertension (PH) due to interstitial lung disease (ILD), a commonly encountered complication of fibrotic ILDs, is associated with significant morbidity and mortality. Until recently, the studies of pulmonary vasodilator therapy in PH-ILD have been largely disappointing, with some even demonstrating the potential for harm. This paper is part of a series of Consensus Statements from the Pulmonary
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A rare case of scimitar syndrome with pulmonary arterial hypertension in an adult female Pulm. Circ. (IF 2.6) Pub Date : 2024-01-03 Yang Liu, Weiliang Ruan, Ziye Li, Hua Wang, Shenghai Chen, Yuhong Ding, Jianfeng Jin
Scimitar syndrome is a rare congenital anomaly characterized by partial or total anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We report a case of a 67-year-old female who presented with cough and dyspnea and was diagnosed with scimitar syndrome and pulmonary arterial hypertension based on comprehensive imaging and hemodynamic evaluation. This case highlights the
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CS1, a controlled-release formulation of valproic acid, for the treatment of patients with pulmonary arterial hypertension: Rationale and design of a Phase 2 clinical trial Pulm. Circ. (IF 2.6) Pub Date : 2024-01-03 Raymond L. Benza, Philip B. Adamson, Deepak L. Bhatt, Fredrik Frick, Gunnar Olsson, Niklas Bergh, Björn Dahlöf
Although rare, pulmonary arterial hypertension (PAH) is associated with substantial morbidity and a median survival of approximately 7 years, even with treatment. Current medical therapies have a primarily vasodilatory effect and do not modify the underlying pathology of the disease. CS1 is a novel oral, controlled-release formulation of valproic acid, which exhibits a multi-targeted mode of action
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Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis Pulm. Circ. (IF 2.6) Pub Date : 2024-01-03 Steven D. Nathan, Benham Tehrani, Qiong Zhao, Rafael Arias, Dennis Kim, Antonia Pellegrini, Ashley Claire Collins, Jack Diviney, Shourjo Chakravorty, Vikramjit Khangoora, Oksana A. Shlobin, Christopher Thomas, Ben R. Lavon, Christopher S. King, Abhimanyu Chandel
Pulmonary vascular dysfunction in the absence of pulmonary hypertension (PH) has been observed in patients with idiopathic pulmonary fibrosis (IPF). We describe the prevalence and etiology of elevated pulmonary vascular resistance (PVR) without PH among patients with IPF. Hemodynamic, echocardiographic, and functional respiratory imaging (FRI) data was compared between patients with IPF without PH
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A rare sequelae of esophageal perforation: Fibrosing mediastinitis Pulm. Circ. (IF 2.6) Pub Date : 2024-01-03 Jingwen Zhang, Mingwang Ding, Aiping Tang, Yunshan Cao
Fibrosing mediastinitis (FM) is a rare disease caused by different causes. If left untreated, the prognosis is poor. The common causes of FM are Tuberculosis and Histoplasma capsulatum infection. Esophageal perforation is also a rare condition that is often easily under- and mis-diagnosed due to the lack of specificity of symptoms. Here we report a case of FM caused by esophageal perforation.
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Safety and efficacy of balloon pulmonary angioplasty for technically operable chronic thromboembolic pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-12-29 Jinzhi Wang, Jixiang Liu, Xincao Tao, Wanmu Xie, Shengfeng Wang, Shuai Zhang, Zhu Zhang, Zhihui Fu, Haobo Li, Yunjing Zhang, Yishan Li, Xincheng Li, Yu Zhang, Linfeng Xi, Dong Liu, Qiang Huang, Yunwei Zhao, Zhenguo Zhai
Balloon pulmonary angioplasty (BPA) has been proven effective for addressing technically inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the effectiveness of BPA in technically operable CTEPH patients who, for various reasons, did not undergo the procedure remains an area requiring exploration. This study sought to assess the safety and efficacy of BPA in such cases. We collected
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Remote exercise testing in pulmonary hypertension (PHRET) Pulm. Circ. (IF 2.6) Pub Date : 2023-12-25 Harrison Stubbs, Stephanie Lua, Jamie Ingram, Bhautesh D. Jani, Melanie Brewis, Colin Church, Martin Johnson
Remote exercise tests for patients with pulmonary hypertension (PH) would improve the telemedicine strategies in this disease. The PHRET study assessed the validity and feasibility of four remote exercise tests performed by PH patients at home. Participants undergoing diagnostic assessment for PH were included. At baseline, patients completed a 6MWT followed by a range of study tests including a Timed
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Real-world evidence to advance knowledge in pulmonary hypertension: Status, challenges, and opportunities. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative's Real-world Evidence Working Group Pulm. Circ. (IF 2.6) Pub Date : 2023-12-21 Kellie Morland, Christian Gerges, Jean Elwing, Scott H. Visovatti, Jason Weatherald, Kari R. Gillmeyer, Sandeep Sahay, Stephen C. Mathai, Athénaïs Boucly, Paul G. Williams, Sivadasanpillai Harikrishnan, Evan P. Minty, Lukas Hobohm, Arun Jose, Roberto Badagliacca, Edmund M. T. Lau, Zhi-Cheng Jing, Rebecca R. Vanderpool, Charles Fauvel, Jose Leonidas Alves, Geoff Strange, Tomas Pulido, Junyan Qian, Mengtao
This manuscript on real-world evidence (RWE) in pulmonary hypertension (PH) incorporates the broad experience of members of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative Real-World Evidence Working Group. We aim to strengthen the research community's understanding of RWE in PH to facilitate clinical research advances and ultimately improve patient care. Herein,
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Current diagnosis and treatment practice for pulmonary hypertension in bronchopulmonary dysplasia—A survey study in Germany (PUsH BPD) Pulm. Circ. (IF 2.6) Pub Date : 2023-12-21 Friederike Häfner, Caroline Johansson, Larissa Schwarzkopf, Kai Förster, Yvonne Kraus, Andreas W. Flemmer, Georg Hansmann, Hannes Sallmon, Ursula Felderhoff-Müser, Sabine Witt, Lars Schwettmann, Anne Hilgendorff
Pulmonary hypertension (PH) is the most severe complication in preterm infants with bronchopulmonary dysplasia (BPD) and associated with significant mortality. Diagnostic and treatment strategies, however, still lack standardization. By the use of a survey study (PH in BPD), we assessed clinical practice (diagnosis, treatment, follow-up) in preterm infants with early postnatal persistent pulmonary
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Identification of monocyte-associated pathways participated in the pathogenesis of pulmonary arterial hypertension based on omics-data Pulm. Circ. (IF 2.6) Pub Date : 2023-12-20 Caiming Zhong, Yachen Si, Huanhuan Yang, Chao Zhou, Yang Chen, Chen Wang, Yalong Liu, Cheng Chen, Hui Shi, Xueli Lai, Hao Tang
Pulmonary arterial hypertension (PAH) is one kind of chronic and uncurable diseases that can cause heart failure. Immune microenvironment plays a significant role in PAH. The aim of this study was to assess the role of immune cell infiltration in the pathogenesis of PAH. Differentially expressed genes based on microarray data were enriched in several immune-related pathways. To evaluate the immune
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Pulmonary embolism induces pneumonia-like lung injury beyond pulmonary infarction Pulm. Circ. (IF 2.6) Pub Date : 2023-12-17 Yue Wang, Bo Liu, Chuming Zhou, Yuan Wang, Jianing Miao, Li Zhao
Patients with pulmonary embolism (PE) commonly manifest concomitant “pneumonia,” which is generally believed to be either a cause (infection) or a consequence (infarction) of PE. This study aimed to clarify the relationship between PE and “pneumonia-like” lesions beyond pulmonary infection and infarction. Chest computed tomography (CT) images of patients with PE and deep vein thrombosis (DVT) were
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Quantitative CT measures of pulmonary vascular volume distribution in pulmonary hypertension associated with COPD: Association with clinical characteristics and outcomes Pulm. Circ. (IF 2.6) Pub Date : 2023-12-13 Hector R. Cajigas, Ben Lavon, William Harmsen, Patrick Muchmore, Joana Costa, Charles Mussche, Sydney Pulsifer, Jan De Backer
To determine whether quantitative computed tomography (qCT)-derived metrics of pulmonary vascular volume distribution could distinguish chronic obstructive pulmonary disease (COPD) subjects with associated pulmonary hypertension (PH) from those without and to characterize associations of these measurements with clinical and physiological characteristics and outcomes. We collected retrospective CT,
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Connective tissue disease-associated pulmonary hypertension: A comprehensive review Pulm. Circ. (IF 2.6) Pub Date : 2023-12-11 Vikramjit Khangoora, Elana J. Bernstein, Christopher S. King, Oksana A. Shlobin
Connective tissue diseases (CTDs) can be associated with various forms of pulmonary hypertension, including pulmonary arterial hypertension (PAH), pulmonary veno-occlusive disease, pulmonary venous hypertension, interstitial lung disease-associated pulmonary hypertension, chronic thromboembolic pulmonary hypertension, and sometimes a combination of several processes. The prevalence of PAH varies among
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PERT era, race-based healthcare disparities in a large urban safety net hospital Pulm. Circ. (IF 2.6) Pub Date : 2023-12-05 Veena H. Dronamraju, Ka U. Lio, Rohan Badlani, Ke Cheng, Parth Rali
Pulmonary embolism (PE) is the third leading cause of cardiovascular death in the United States. Black Americans have higher incidence, greater clot severity, and worse outcomes than White Americans. This disparity is not fully understood, especially in the context of the advent of PE response teams (PERT), which aim to standardize PE-related care. This retrospective single-center cohort study compared
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Pulmonary artery dilatation in different causes of pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-12-06 Qunying Xi, Zhihong Liu, Changming Xiong, Qin Luo, Zhihui Zhao, Qing Zhao, Tao Yang, Qixian Zeng, Pingwei Li, Luwen Qiu
Pulmonary artery (PA) dilatation is commonly observed in patients with pulmonary hypertension (PH). However, the clinical aspects of PA dilatation in various etiology of PH remain unknown. In this study, we investigated the clinical and imaging characteristics of 1018 patients with different subtypes of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)
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Risk assessment in pulmonary arterial hypertension patients with multiple comorbidities and/or advanced age—Where do we stand and what's next? Pulm. Circ. (IF 2.6) Pub Date : 2023-11-30 Abdulla Ahmed, Salaheldin Ahmed, Göran Rådegran
The 2022 ESC/ERS pulmonary hypertension (PH) guidelines evaluated the evidence and clinical role of risk stratification, by answering key narrative question 4 -“Should a risk-stratification strategy be used to guide treatment in patients with pulmonary arterial hypertension (PAH)?” The guidelines concluded that therapeutic decision-making in PAH should be based on two prognostic determinators, that
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Left ventricular underfilling in PAH: A potential indicator for adaptive-to-maladaptive transition Pulm. Circ. (IF 2.6) Pub Date : 2023-11-30 Jiajun Guo, Jiaqi Wang, Lili Wang, Yangjie Li, Yuanwei Xu, Weihao Li, Chen Chen, Juan He, Lidan Yin, Shoufang Pu, Bi Wen, Yuchi Han, Yucheng Chen
Pulmonary arterial hypertension (PAH) still remains a life-threatening disorder with poor prognosis. The right ventricle (RV) adapts to the increased afterload by a series of prognostically significant morphological and functional changes, the adaptive nature should also be understood in the context of ventricular interdependence. We hypothesized that left ventricle (LV) underfilling could serve as
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A 5-year survivor of endarterectomy for sclerosing undifferentiated intimal sarcoma of the pulmonary artery: Importance of clinical suspicion and careful histologic evaluation Pulm. Circ. (IF 2.6) Pub Date : 2023-11-29 Kisaki Amemiya, Morikazu Nishihira, Hatsue Ishibashi-Ueda, Keiko Ohta-Ogo, Takeshi Ogo, Yoshihiko Ikeda, Kinta Hatakeyama, Hiroaki Sasaki, Hitoshi Ogino
We present a diagnostically challenging case of intimal sarcoma of the pulmonary artery (PA) due to the histologic finding of a sclerosing appearance with no appreciable spindle/pleomorphic cell proliferation. Initial endarterectomy specimens were composed of sclerosing extracellular matrix with a few bland cells, some recanalization, and fibrin thrombi, impeding the confirmation of intimal sarcoma
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Premorbid weight in pulmonary arterial hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-11-21 Laura J. Oppegard, Lia M. Barros, Hongyang Pi, James Kornfield, Catherine L. Hough, Samuel G. Rayner, Jeffrey C. Robinson, Peter J. Leary
Relationships between obesity and outcomes in pulmonary arterial hypertension (PAH) are complex. Previous work suggested obesity, occurring alongside PAH, may be associated with better survival. In our work, we suggest obesity prior to PAH development is associated with worse survival. This may add a novel temporal element to the “obesity-paradox.”
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Balloon pulmonary angioplasty in the current era of CTEPH treatment: How did we get here? Pulm. Circ. (IF 2.6) Pub Date : 2023-11-20 Jenny Z. Yang, David S. Poch, Lawrence Ang, Ehtisham Mahmud, Nick H. Kim
Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by persistent organized thromboembolic obstruction of the pulmonary arteries from incompletely resolved pulmonary embolism. The treatment of choice is pulmonary thromboendarterectomy (PTE) surgery and all patients should be evaluated for operability candidacy. Despite advancements in PTE technique allowing more segmental–subsegmental surgeries
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4D-Flow MRI intracardiac flow analysis considering different subtypes of pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-11-06 Michael T. Cain, Michal Schäfer, Lexie K. Ross, David D. Ivy, Max B. Mitchell, Brett E. Fenster, Todd M. Bull, Alex J. Barker, Daniel Vargas, Jordan R. H. Hoffman
Intracardiac flow hemodynamic patterns have been considered to be an early sign of diastolic dysfunction. In this study we investigated right ventricular (RV) diastolic dysfunction between patients with pulmonary arterial hypertension (PAH) and pulmonary hypertension with chronic lung disease (PH-CLD) via 4D-Flow cardiac MRI (CMR). Patients underwent prospective, comprehensive CMR for function and
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Management of pulmonary hypertension associated with valvular heart disease with angiotensin-receptor neprilysin inhibitor Pulm. Circ. (IF 2.6) Pub Date : 2023-11-05 Hyeon-Ju Ryoo Ali, Jerome Thomas, Sandeep Sahay, Ashrith Guha
Pulmonary hypertension secondary to left-sided valvular disease (VHD-PH) is associated with high morbidity and mortality. Angiotensin-receptor neprilysin inhibitor (ARNI) is a novel pharmacotherapy, which reduces afterload with natriuresis and peripheral vasodilation. Our cases demonstrate that ARNI may also have a role in the treatment of combined pre- and postcapillary pulmonary hypertension that
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Long-term sequelae following acute pulmonary embolism: A nationwide follow-up study regarding the incidence of CTEPH, dyspnea, echocardiographic and V/Q scan abnormalities Pulm. Circ. (IF 2.6) Pub Date : 2023-11-02 Therese Andersson, Lars Nilsson, Flemming Larsen, Bo Carlberg, Stefan Söderberg
We aimed to follow a nationwide cohort of patients with pulmonary embolism (PE) without any exclusions to generate information regarding long-term symptoms, investigational findings and to determine the prevalence of chronic thromboembolic pulmonary hypertension (CTEPH). We hypothesized that this approach would yield generalizable estimates of CTEPH prevalence and incidence. All individuals diagnosed
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Differential drug response in pulmonary arterial hypertension: The potential for precision medicine Pulm. Circ. (IF 2.6) Pub Date : 2023-11-02 Elise Miller, Chinwuwanuju Ugo-Obi Sampson, Ankit A. Desai, Jason H. Karnes
Pulmonary arterial hypertension (PAH) is a rare, complex, and deadly cardiopulmonary disease. It is characterized by changes in endothelial cell function and smooth muscle cell proliferation in the pulmonary arteries, causing persistent vasoconstriction, resulting in right heart hypertrophy and failure. There are multiple drug classes specific to PAH treatment, but variation between patients may impact
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Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-10-31 Joseph M. Collaco, Steven H. Abman, Eric D. Austin, Catherine M. Avitabile, Angela Bates, Jeffrey R. Fineman, Grace A. Freire, Stephanie S. Handler, Dunbar D. Ivy, Usha S. Krishnan, Mary P. Mullen, Nidhy P. Varghese, Delphine Yung, Melanie K. Nies, Allen D. Everett, Kanecia O. Zimmerman, William Simmons, Hrishikesh Chakraborty, Gayane Yenokyan, Allison Newell-Sturdivant, Eric Christensen, Lindsay M
Pulmonary hypertension (PH) is a significant health problem that contributes to high morbidity and mortality in diverse cardiac, pulmonary, and systemic diseases in children. Evidence-based advances in PH care have been challenged by a paucity of quality endpoints for assessing clinical course and the lack of robust clinical trial data to guide pharmacologic therapies in children. While the landmark
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Persistence of pulmonary hypertension in patients undergoing ventricular assist devices and orthotopic heart transplantation Pulm. Circ. (IF 2.6) Pub Date : 2023-10-30 Arun Rajaratnam, Ameen El-Swais, Charles McTiernan, Floyd W. Thoma, Moaaz O. Baghal, Kristen Raffensperger, Chung-Chou H. Chang, Gavin W. Hickey, Faraaz A. Shah, Imad Al Ghouleh
Pulmonary hypertension (PH) is common in advanced heart failure and often improves quickly after left ventricular assist device (VAD) implantation or orthotopic heart transplantation (OHT), but long-term effects and outcomes are not well-described. This study evaluated PH persistence after VAD as destination therapy (VAD-DT), bridge to transplant (VAD-OHT), or OHT-alone. The study constituted a retrospective
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A very rare cause of pre-capillary pulmonary hypertension: The PAMI syndrome Pulm. Circ. (IF 2.6) Pub Date : 2023-10-22 Manuela Iseppi, Giulio Savonitto, Alberto Tommasini, Alessia Pin, Gianfranco Sinagra, Davide Stolfo
We report the first known case of PAMI syndrome associated with pulmonary arterial hypertension (PAH) with a positive response to cyclophosphamide and pulmonary vasodilators. The patient's history began at 7 months with severe pancytopenia and fever. As time progressed, migrating arthritis, hepatosplenomegaly, and a growth deficit manifested without a plausible explanation. At the age of 17, worsening
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Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia Pulm. Circ. (IF 2.6) Pub Date : 2023-10-20 Melissa K. Webb, Milenka Cuevas Guaman, S. Kristen Sexson Tejtel, Neil Cambronero, Ryan D. Coleman, Corey A. Chartan, Betul Yilmaz Furtun, Shaine A. Morris, Nidhy P. Varghese, Natalie M. Villafranco
Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD-ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD-ASD
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Whole-process respiratory management strategies based on electrical impedance tomography in a pregnant woman with diffuse alveolar hemorrhage induced by systemic lupus erythematosus under veno-venous extracorporeal membrane oxygenation Pulm. Circ. (IF 2.6) Pub Date : 2023-10-20 Yu Zhao, Longxiang Su, Huaiwu He, Dawei Liu, Yun Long
Electrical impedance tomography (EIT) as a bedside, noninvasive, radiation-free technology, could quantify alveolar collapse and over-distension and provide real-time ventilation images of lungs. Clinical studies have shown potential benefit in reducing lung injury by EIT to guide mechanical ventilation setting in acute respiratory distress syndrome (ARDS). The respiratory management of ARDS with venous−venous
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Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review Pulm. Circ. (IF 2.6) Pub Date : 2023-10-19 Akash Mathavan, Akshay Mathavan, Renuka Reddy, Kirk Jones, Christina Eagan, Hassan Alnuaimat, Ali Ataya
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disorder characterized by recurrent spontaneous epistaxis, mucocutaneous telangiectasias, and solid organ arteriovenous malformations (AVMs). Pulmonary hypertension (PH) is an increasingly recognized complication in patients with HHT, most often precipitated by high-output heart failure in the presence of hepatic AVMs
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Quantifying 4D flow cardiovascular magnetic resonance vortices in patients with pulmonary hypertension: A pilot study Pulm. Circ. (IF 2.6) Pub Date : 2023-10-18 Ali Borhani, Kristin K. Porter, Muhammad Umair, Linda C. Chu, Stephen C. Mathai, Todd M. Kolb, Rachel L. Damico, Paul M. Hassoun, Ihab R. Kamel, Stefan L. Zimmerman
In this 4D flow cardiovascular magnetic resonance (CMR) study, vortical blood flow in the main pulmonary artery (MPA) is quantified using circulation (ᴦ), a metric used in fluid dynamics to quantify the rotational components of flow. Circulation (ᴦ) is a 4D flow CMR metric that quantifies the vortical blood flow pattern in the MPA of patients with pulmonary hypertension (PH), distinguishes them from
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Pulmonary hemodynamics and transplant-free survival in sarcoidosis-associated pulmonary hypertension: Results from an international registry Pulm. Circ. (IF 2.6) Pub Date : 2023-10-11 Shameek K. Gayen, Robert P. Baughman, Steven D. Nathan, Athol U. Wells, Vasilis Kouranos, Esam H. Alhamad, Daniel A. Culver, Joseph Barney, Eva M. Carmoma, Francis C. Cordova, Marloes Huitema, Mary Beth Scholand, Marlies Wijsenbeek, Sivagini Ganesh, Surinder S. Birring, Laura C. Price, Stephen J. Wort, Oksana A. Shlobin, Rohit Gupta
Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac index (CI) ≤ 2 L/min/m2. However, there is no clear definition denoting severity of sarcoidosis-associated PH (SAPH). We aimed to determine pulmonary hemodynamic cut-off
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Assessing the optimal MAP target in pre-capillary PH patients with RV failure: A retrospective analysis Pulm. Circ. (IF 2.6) Pub Date : 2023-10-08 Niala Moallem, Garrett Fiscus, David M. O'Sullivan, Michael Perkins, Andrew Scatola, Raj Parikh
Right ventricular failure (RVF) in pre-capillary pulmonary hypertension (PH) is associated with high morbidity and mortality. While mean arterial pressure (MAP) goals have been well established in critical care literature, the optimal MAP target for patients with RVF secondary to pre-capillary PH remains unknown. The objective of this study was to evaluate the difference in outcomes between patients
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Lianhua Qingke ameliorates lipopolysaccharide-induced lung injury by inhibiting neutrophil extracellular traps formation and pyroptosis Pulm. Circ. (IF 2.6) Pub Date : 2023-10-06 Wenjun Peng, Hui Qi, Wensi Zhu, Lin Tong, Ainiwaer Rouzi, Yuanyuan Wu, Linxiao Han, Ludan He, Yu Yan, Ting Pan, Jie Liu, Qin Wang, Zhenhua Jia, Yuanlin Song, Qiaoliang Zhu, Jian Zhou
LHQK is a patented Traditional Chinese Medicine (TCM) which is clinically used for acute tracheobronchitis, cough, and other respiratory diseases. Recent studies have proved that LHQK exhibits excellent clinical efficacy in the treatment of acute lung injury (ALI). However, the corresponding mechanisms remain largely unexplored. In this study, we investigated the effects and the underlying mechanisms
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The role of vitamin D in chronic obstructive pulmonary disease with pulmonary hypertension Pulm. Circ. (IF 2.6) Pub Date : 2023-10-04 Mengxi Li
Hypoxia pulmonary hypertension (PH) belongs to the third major category in PH classification. Chronic obstructive pulmonary disease (COPD) is a common cause of hypoxia PH. Low serum vitamin D concentration is considered to be a possible risk factor for chronic lung disease; epidemiological studies have found that vitamin D deficiency increases pulmonary artery pressure. Therefore, this study aimed
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Pulmonary arterial hypertension drugs can partially restore altered angiogenic capacities in bmpr2-silenced human lung microvascular endothelial cells Pulm. Circ. (IF 2.6) Pub Date : 2023-10-02 Birger Tielemans, Allard Wagenaar, Catharina Belge, Marion Delcroix, Rozenn Quarck
Mutations in the bone morphogenetic protein receptor type 2 (bmpr2) gene and signaling pathway impairment are observed in heritable and idiopathic pulmonary arterial hypertension (PAH). In PAH, endothelial dysfunction is currently handled by drugs targeting the endothelin-1 (ET-1), nitric oxide (NO), and prostacyclin (PGI2) pathways. The role of angiogenesis in the disease process and the effect of