Peng Zhang, Denielli Da Silva Goncalves Bos, Alexander Vang, Julia Feord, Danielle J. McCullough, Alexsandra Zimmer, Natalie D'Silva, Richard T. Clements, Gaurav Choudhary
{"title":"Reduced exercise capacity occurs before intrinsic skeletal muscle dysfunction in experimental rat models of pulmonary hypertension","authors":"Peng Zhang, Denielli Da Silva Goncalves Bos, Alexander Vang, Julia Feord, Danielle J. McCullough, Alexsandra Zimmer, Natalie D'Silva, Richard T. Clements, Gaurav Choudhary","doi":"10.1002/pul2.12358","DOIUrl":"https://doi.org/10.1002/pul2.12358","url":null,"abstract":"Reduced exercise capacity in pulmonary hypertension (PH) significantly impacts quality of life. However, the cause of reduced exercise capacity in PH remains unclear. The objective of this study was to investigate whether intrinsic skeletal muscle changes are causative in reduced exercise capacity in PH using preclinical PH rat models with different PH severity. PH was induced in adult Sprague–Dawley (SD) or Fischer (CDF) rats with one dose of SU5416 (20 mg/kg) injection, followed by 3 weeks of hypoxia and additional 0–4 weeks of normoxia exposure. Control\u0000<span style=\"text-decoration:line-through\">s</span> rats were injected with vehicle and housed in normoxia. Echocardiography was performed to assess cardiac function. Exercise capacity was assessed by VO<sub>2</sub> max. Skeletal muscle structural changes (atrophy, fiber type switching, and capillary density), mitochondrial function, isometric force, and fatigue profile were assessed. In SD rats, right ventricular systolic dysfunction is associated with reduced exercise capacity in PH rats at 7-week timepoint in comparison to control rats, while no changes were observed in skeletal muscle structure, mitochondrial function, isometric force, or fatigue profile. CDF rats at 4-week timepoint developed a more severe PH and, in addition to right ventricular dysfunction, the reduced exercise capacity in these rats is associated with skeletal muscle atrophy; however, mitochondrial function, isometric force, and fatigue profile in skeletal muscle remain unchanged. Our data suggest that cardiopulmonary impairments in PH are the primary cause of reduced exercise capacity, which occurs before intrinsic skeletal muscle dysfunction.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"33 1","pages":""},"PeriodicalIF":2.6,"publicationDate":"2024-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140560257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel J. Lachant, Michael D. Lachant, Deborah Haight, R. James White
{"title":"Cardiac effort and 6-min walk distance correlate with stroke volume measured by cardiac magnetic resonance imaging","authors":"Daniel J. Lachant, Michael D. Lachant, Deborah Haight, R. James White","doi":"10.1002/pul2.12355","DOIUrl":"https://doi.org/10.1002/pul2.12355","url":null,"abstract":"Right ventricular (RV) dysfunction in pulmonary arterial hypertension (PAH) is associated with poor outcomes. Cardiac magnetic resonance imaging (cMRI) is the gold standard for volumetric assessment, and few reports have correlated 6-min walk distance (6MWD) and cMRI parameters in PAH. Cardiac Effort, (the number of heart beats used during 6-min walk test)/(6MWD), incorporates physiologic changes into walk distance and has been associated with stroke volume (SV) measured by nuclear imaging and indirect Fick. Here, we aimed to interrogate the relationship of Cardiac Effort and 6MWD with SV measured by the gold standard, cMRI. This was a single-center, observational, prospective study in Group 1 PAH patients. Subjects completed 6-min walk with heart rate monitoring (Cardiac Effort) and cMRI within 24 h. cMRI was correlated to Cardiac Effort and 6MWD using Spearman Correlation Coefficient. Twenty-five participants with a wide range of RV function completed both cMRI and Cardiac Effort. There was a strong correlation between left ventricle SV index and both Cardiac Effort (<i>r</i> = −0.70, <i>p</i> = 0.0001) and 6MWD (<i>r</i> = 0.67, <i>p</i> = 0.0002). Cardiac Effort and 6MWD were statistically separated in patients at prognostically significant thresholds of left ventricle SV index (>31 ml/m<sup>2</sup>), RV Ejection Fraction (>35%), and SV/End Systolic Volume ( > 0.53). Cardiac Effort and 6MWD are noninvasive ways to gain insight into those with impaired SV. 6MWD may correlate better with SV than previously thought and heart rate monitoring provides physiologic context to the walk distance obtained.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"5 1","pages":""},"PeriodicalIF":2.6,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140560363","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pulmonary tumor thrombotic microangiopathy due to early gastric carcinoma in a patient with no antemortem findings suggestive of primary malignancy.","authors":"Jun-Ichi Noiri, Yu Taniguchi, Yu Izawa, Nobuyuki Saga, Kaori Kusakabe, Yu-Ichiro Koma, Ken-Ichi Hirata","doi":"10.1002/pul2.12359","DOIUrl":"10.1002/pul2.12359","url":null,"abstract":"<p><p>Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare and critical malignancy-related disease characterized by acute progressive pulmonary hypertension (PH). In most cases of PTTM, the cancer can be diagnosed in advance. Identification of the primary cancer is valuable for PTTM diagnosis. Here, we present the case of a patient with PTTM due to early gastric carcinoma in whom the diagnosis of malignant cancer was not revealed until macroscopic autopsy findings. This case highlights the importance of recognizing causative occult early gastric cancer leading to PTTM in cases of acute progressive PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12359"},"PeriodicalIF":2.2,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10973772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140319113","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}
Pulmonary CirculationPub Date : 2024-03-25eCollection Date: 2024-01-01DOI: 10.1002/pul2.12352
Hunter Gillies, Murali M Chakinala, Benjamin T Dake, Jeremy P Feldman, Marius M Hoeper, Marc Humbert, Zhi-Cheng Jing, Jonathan Langley, Vallerie V McLaughlin, Ralph W Niven, Stephan Rosenkranz, Xiaosha Zhang, Nicholas S Hill
{"title":"IMPAHCT: A randomized phase 2b/3 study of inhaled imatinib for pulmonary arterial hypertension.","authors":"Hunter Gillies, Murali M Chakinala, Benjamin T Dake, Jeremy P Feldman, Marius M Hoeper, Marc Humbert, Zhi-Cheng Jing, Jonathan Langley, Vallerie V McLaughlin, Ralph W Niven, Stephan Rosenkranz, Xiaosha Zhang, Nicholas S Hill","doi":"10.1002/pul2.12352","DOIUrl":"10.1002/pul2.12352","url":null,"abstract":"<p><p>AV-101 (imatinib) powder for inhalation, an investigational dry powder inhaled formulation of imatinib designed to target the underlying pathobiology of pulmonary arterial hypertension, was generally well tolerated in healthy adults in a phase 1 single and multiple ascending dose study. <i>I</i>nhaled I<i>m</i>atinib <i>P</i>ulmonary <i>A</i>rterial <i>H</i>ypertension <i>C</i>linical <i>T</i>rial (IMPAHCT; NCT05036135) is a phase 2b/3, randomized, double-blind, placebo-controlled, dose-ranging, and confirmatory study. IMPAHCT is designed to identify an optimal AV-101 dose (phase 2b primary endpoint: pulmonary vascular resistance) and assess the efficacy (phase 3 primary endpoint: 6-min walk distance), safety, and tolerability of AV-101 dose levels in subjects with pulmonary arterial hypertension using background therapies. The study has an operationally seamless, adaptive design allowing for continuous recruitment. It includes three parts; subjects enrolled in Part 1 (phase 2b dose-response portion) or Part 2 (phase 3 intermediate portion) will be randomized 1:1:1:1 to 10, 35, 70 mg AV-101, or placebo (twice daily), respectively. Subjects enrolled in Part 3 (phase 3 optimal dose portion) will be randomized 1:1 to the optimal dose of AV-101 and placebo (twice daily), respectively. All study parts include a screening period, a 24-week treatment period, and a 30-day safety follow-up period; the total duration is ∼32 weeks. Participation is possible in only one study part. IMPAHCT has the potential to advance therapies for patients with pulmonary arterial hypertension by assessing the efficacy and safety of a novel investigational drug-device combination (AV-101) using an improved study design that has the potential to save 6-12 months of development time. ClinicalTrials.gov Identifier: NCT05036135.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12352"},"PeriodicalIF":2.2,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10963589/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140294406","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}
Pulmonary CirculationPub Date : 2024-03-18eCollection Date: 2024-01-01DOI: 10.1002/pul2.12356
George A Alba, Iris Y Zhou, Molly Mascia, Michael Magaletta, Jehan W Alladina, Francesca L Giacona, Leo C Ginns, Peter Caravan, Bradley A Maron, Sydney B Montesi
{"title":"Plasma NEDD9 is increased following SARS-CoV-2 infection and associates with indices of pulmonary vascular dysfunction.","authors":"George A Alba, Iris Y Zhou, Molly Mascia, Michael Magaletta, Jehan W Alladina, Francesca L Giacona, Leo C Ginns, Peter Caravan, Bradley A Maron, Sydney B Montesi","doi":"10.1002/pul2.12356","DOIUrl":"10.1002/pul2.12356","url":null,"abstract":"<p><p>Compared to healthy volunteers, participants with post-acute sequelae of SARS-CoV-2 infection (PASC) demonstrated increased plasma levels of the prothrombotic protein NEDD9, which associated inversely with indices of pulmonary vascular function. This suggests persistent pulmonary vascular dysfunction may play a role in the pathobiology of PASC.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12356"},"PeriodicalIF":2.2,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10946282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140158902","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}
Pulmonary CirculationPub Date : 2024-03-17eCollection Date: 2024-01-01DOI: 10.1002/pul2.12337
Frances Varian, Jennifer Dick, Christian Battersby, Stefan Roman, Jenna Ablott, Lisa Watson, Sarah Binmahfooz, Hamza Zafar, Gerry Colgan, John Cannon, Jay Suntharalingam, Jim Lordan, Luke Howard, Colm McCabe, John Wort, Laura Price, Colin Church, Neil Hamilton, Iain Armstrong, Abdul Hameed, Judith Hurdman, Charlie Elliot, Robin Condliffe, Martin Wilkins, Alastair Webb, David Adlam, Ray L Benza, Kazem Rahimi, Mohadeseh Shojaei-Shahrokhabadi, Nan X Lin, James M S Wason, Alasdair McIntosh, Alex McConnachie, Jennifer T Middleton, Roger Thompson, David G Kiely, Mark Toshner, Alexander Rothman
{"title":"Pulmonary Hypertension: Intensification and Personalization of Combination Rx (PHoenix): A phase IV randomized trial for the evaluation of dose-response and clinical efficacy of riociguat and selexipag using implanted technologies.","authors":"Frances Varian, Jennifer Dick, Christian Battersby, Stefan Roman, Jenna Ablott, Lisa Watson, Sarah Binmahfooz, Hamza Zafar, Gerry Colgan, John Cannon, Jay Suntharalingam, Jim Lordan, Luke Howard, Colm McCabe, John Wort, Laura Price, Colin Church, Neil Hamilton, Iain Armstrong, Abdul Hameed, Judith Hurdman, Charlie Elliot, Robin Condliffe, Martin Wilkins, Alastair Webb, David Adlam, Ray L Benza, Kazem Rahimi, Mohadeseh Shojaei-Shahrokhabadi, Nan X Lin, James M S Wason, Alasdair McIntosh, Alex McConnachie, Jennifer T Middleton, Roger Thompson, David G Kiely, Mark Toshner, Alexander Rothman","doi":"10.1002/pul2.12337","DOIUrl":"10.1002/pul2.12337","url":null,"abstract":"<p><p>Approved therapies for the treatment of patients with pulmonary arterial hypertension (PAH) mediate pulmonary vascular vasodilatation by targeting distinct biological pathways. International guidelines recommend that patients with an inadequate response to dual therapy with a phosphodiesterase type-5 inhibitor (PDE5i) and endothelin receptor antagonist (ERA), are recommended to either intensify oral therapy by adding a selective prostacyclin receptor (IP) agonist (selexipag), or switching from PDE5i to a soluble guanylate-cyclase stimulator (sGCS; riociguat). The clinical equipoise between these therapeutic choices provides the opportunity for evaluation of individualized therapeutic effects. Traditionally, invasive/hospital-based investigations are required to comprehensively assess disease severity and demonstrate treatment benefits. Regulatory-approved, minimally invasive monitors enable equivalent measurements to be obtained while patients are at home. In this 2 × 2 randomized crossover trial, patients with PAH established on guideline-recommended dual therapy and implanted with CardioMEMS™ (a wireless pulmonary artery sensor) and ConfirmRx™ (an insertable cardiac rhythm monitor), will receive ERA + sGCS, or PDEi + ERA + IP agonist. The study will evaluate clinical efficacy via established clinical investigations and remote monitoring technologies, with remote data relayed through regulatory-approved online clinical portals. The primary aim will be the change in right ventricular systolic volume measured by magnetic resonance imaging (MRI) from baseline to maximal tolerated dose with each therapy. Using data from MRI and other outcomes, including hemodynamics, physical activity, physiological measurements, quality of life, and side effect reporting, we will determine whether remote technology facilitates early evaluation of clinical efficacy, and investigate intra-patient efficacy of the two treatment approaches.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12337"},"PeriodicalIF":2.2,"publicationDate":"2024-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10945040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140159888","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":"Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension with bronchial obstruction by a carcinoid tumor.","authors":"Yuki Monden, Dai Une, Sho Mitsumune, Hiroto Shimokawahara, Hirofumi Okada, Kenji Yoshida, Shutaro Kato, Suzuka Kamaguchi, Mikizo Nakai, Motomi Ando","doi":"10.1002/pul2.12354","DOIUrl":"10.1002/pul2.12354","url":null,"abstract":"<p><p>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 carcinoid tumor in a 75-year-old man. On-site evaluation and removal of the obstructive tumor were performed bronchoscopically, increasing the effectiveness of subsequent PEA for all affected pulmonary segments. This report illustrates a PEA strategy to treat CTEPH with bronchial tumor obstruction.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12354"},"PeriodicalIF":2.2,"publicationDate":"2024-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10938258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140132435","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}
Pulmonary CirculationPub Date : 2024-03-12eCollection Date: 2024-01-01DOI: 10.1002/pul2.12353
Emilia M Swietlik, Michaela Fay, Nicholas W Morrell
{"title":"Unlocking the potential of genetic research in pulmonary arterial hypertension: Insights from clinicians, researchers, and study team.","authors":"Emilia M Swietlik, Michaela Fay, Nicholas W Morrell","doi":"10.1002/pul2.12353","DOIUrl":"10.1002/pul2.12353","url":null,"abstract":"<p><p>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, and researchers from nine UK Pulmonary Hypertension centers, who had genetic research experience. Transcripts were thematically coded using inductive analysis. In this study, we focus on the researchers', clinicians', and study team's perspectives. From the interview data, several key themes emerged, ranging from study design, recruitment, and consent procedures to the return of individual genetic results. Additionally, participants reflected on both the successes of these studies and the future directions of genetic research. The analysis highlighted the critical importance of fostering collaborative networks firmly rooted in existing clinical and research infrastructure in rare disease study setups. Furthermore, the significance of trust-building, personalized communication, and transparency among stakeholders was underscored. The study offered valuable insights into the motivating and hindering factors to participant recruitment and consent procedures. Lastly, the findings gathered from processes surrounding the return of individual genetic results, genetic counselling, and the recruitment of relatives provided invaluable lessons regarding the integration of genetics into clinical practice. This in-depth analysis yields a crucial understanding of attitudes to genetic research among various stakeholders and sheds light on the complexities of genetic research and the evidence-practice gap.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 1","pages":"e12353"},"PeriodicalIF":2.2,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10933531/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140120514","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}
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
{"title":"Evaluating the efficacy and safety of oral triple sequential combination therapy for treating patients with pulmonary arterial hypertension: A multicenter retrospective study","authors":"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","doi":"10.1002/pul2.12351","DOIUrl":"https://doi.org/10.1002/pul2.12351","url":null,"abstract":"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 on dual-combination therapy with ERA and PDE5i/riociguat for a minimum of 3 months. Clinical data were collected at baseline and after 6 months of treatment. The study analyzed the event-free survival at 6 months and all-cause death over 2 years. At baseline, the distribution of patients among the risk groups was as follows: 22 in the low-risk group, 35 in the intermediate-low-risk group, 91 in the intermediate-high-risk group, and 44 in the high-risk group. After 6 months of treatment, the oral sequential triple combination therapy resulted in reduced NT-proBNP levels (media from 1604 to 678 pg/mL), a decline in the percentage of WHO-FC III/IV (from 79.2% to 60.4%), an increased in the 6MWD (from 325 ± 147 to 378 ± 143 m) and a rise in the percentage of patients with three low-risk criteria (from 5.7% to 13.5%). Among the low-risk group, there was an improvement in the right heart remodeling, marked by a decrease in right atrium area and eccentricity index. The intermediate-low-risk group exhibited significant enhancements in WHO-FC and tricuspid annular plane systolic excursion. For those in the intermediate-high and high-risk groups, there were marked improvements in activity tolerance, as reflected by WHO-FC and 6MWD. The event-free survival rate at 6 months stood at 88%. Over the long-term follow-up, the survival rates at 1 and 2 years were 86.5% and 86.0%, respectively. In conclusion, the oral sequential triple combination therapy enhanced both exercise capacity and cardiac remodeling across PAH patients of different risk stratifications.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"283 1","pages":""},"PeriodicalIF":2.6,"publicationDate":"2024-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140098403","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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
{"title":"Safety of inhaled nitric oxide withdrawal in severe chronic pulmonary hypertension","authors":"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","doi":"10.1002/pul2.12344","DOIUrl":"https://doi.org/10.1002/pul2.12344","url":null,"abstract":"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 assessed hemodynamic, echocardiographic, and other noninvasive variables to evaluate safety and efficacy of iNO. We performed an iNO withdrawal test during right heart catheterization and after 3 days of iNO treatment. iNO significantly improved all variables examined in 22 patients with PAH and 11 with nCTEPH. Two patterns of response were observed after sudden iNO withdrawal. Twenty-nine patients (88%) showed minimal hemodynamic, oxygenation and clinical changes. Four patients (12%) had a reduction in cardiac index ≥20% and PaO<sub>2</sub> ≥ 5%, three patients did not show clinical deterioration, and one patient developed hemodynamic collapse that needed iNO administration. This retrospective study suggests that short-term iNO improves hemodynamics and clinical conditions in some patients with PAH an nCTPEH. However, pulsed iNO withdrawal PH rebound could be a serious concern in these patients. Given the lack of evidence, we do not recommend the use of pulsed iNO in the treatment of patients with chronic PH.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"21 1","pages":""},"PeriodicalIF":2.6,"publicationDate":"2024-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140098373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}