Pulmonary CirculationPub Date : 2024-07-08eCollection Date: 2024-07-01DOI: 10.1002/pul2.12399
Allan Lawrie, Neil Hamilton, Steven Wood, Fernando Exposto, Ruvimbo Muzwidzwa, Louise Raiteri, Amélie Beaudet, Audrey Muller, Rafael Sauter, Nadia Pillai, David G Kiely
{"title":"Association of risk assessment at diagnosis with healthcare resource utilization and health-related quality of life outcomes in pulmonary arterial hypertension.","authors":"Allan Lawrie, Neil Hamilton, Steven Wood, Fernando Exposto, Ruvimbo Muzwidzwa, Louise Raiteri, Amélie Beaudet, Audrey Muller, Rafael Sauter, Nadia Pillai, David G Kiely","doi":"10.1002/pul2.12399","DOIUrl":"10.1002/pul2.12399","url":null,"abstract":"<p><p>We aimed to describe the clinical characteristics, healthcare resource utilization (HCRU) and costs, health-related quality of life (HRQoL), and survival for patients with pulmonary arterial hypertension (PAH), stratified by 1-year mortality risk at diagnosis. Adults diagnosed with PAH at the Sheffield Pulmonary Vascular Disease Unit between 2012 and 2019 were included. Patients were categorized as low, intermediate, or high risk for 1-year mortality at diagnosis. Demographics, clinical characteristics, comorbidities, HCRU, costs, HRQoL, and survival were analyzed. Overall, 1717 patients were included: 72 (5%) at low risk, 941 (62%) at intermediate risk, and 496 (33%) at high risk. Low-risk patients had lower HCRU prediagnosis and 1-year postdiagnosis than intermediate- or high-risk patients. Postdiagnosis, there were significant changes in HCRU, particularly inpatient hospitalizations and accident and emergency (A&E) visits among high-risk patients. At 3 years postdiagnosis, HCRU for all measures was similar across risk groups. Low-risk patients had lower EmPHasis-10 scores (indicating better HRQoL) at diagnosis and at 1-year follow-up compared with intermediate- and high-risk patients; only the score in the high-risk group improved. Median overall survival decreased as risk category increased in analyzed subgroups. Low-risk status was associated with better 1-year survival and HRQoL compared with intermediate- and high-risk patients. HCRU decreased in high-risk patients postdiagnosis, with the most marked reduction in A&E admissions. The pattern of decreased per-patient inpatient hospitalizations and A&E visits at 3 years postdiagnosis suggests that a diagnosis of PAH helps to decrease HCRU in areas that are key drivers of costs.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12399"},"PeriodicalIF":2.2,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11229026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141559587","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 : 2024-07-05eCollection Date: 2024-07-01DOI: 10.1002/pul2.12404
Rodrigo Hoyos, Mona Lichtblau, Elizabeth Cajamarca, Laura Mayer, Esther Irene Schwarz, Silvia Ulrich
{"title":"Characteristics and risk profiles of patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension living permanently at >2500 m of high altitude in Ecuador.","authors":"Rodrigo Hoyos, Mona Lichtblau, Elizabeth Cajamarca, Laura Mayer, Esther Irene Schwarz, Silvia Ulrich","doi":"10.1002/pul2.12404","DOIUrl":"10.1002/pul2.12404","url":null,"abstract":"<p><p>Over 80 Mio people worldwide live >2500 m, including at least as many patients with pulmonary vascular disease (PVD), defined as pulmonary arterial or chronic thromboembolic pulmonary hypertension (PAH/CTEPH), as elsewhere (estimated 0.1‰). Whether PVD patients living at high altitude have altered disease characteristics due to hypobaric hypoxia is unknown. In a cross-sectional study conducted at the Hospital Carlos Andrade Marin in Quito, Ecuador, located at 2840 m, we included 36 outpatients with PAH or CTEPH visiting the clinic from January 2022 to July 2023. We collected data on diagnostic right heart catheterization, treatment, and risk factors, including NYHA functional class (FC), 6-min walk distance (6MWD), and NT-brain natriuretic peptide (BNP) at baseline and at last follow-up. Thirty-six PVD patients (83% women, 32 PAH, 4 CTEPH, mean ± SD age 44 ± 13 years, living altitude 2831 ± 58 m) were included and had the following baseline values: PaO<sub>2</sub> 8.2 ± 1.6 kPa, PaCO<sub>2</sub> 3.9 ± 0.5 kPa, SaO<sub>2</sub> 91 ± 3%, mean pulmonary artery pressure 53 ± 16 mmHg, pulmonary vascular resistance 16 ± 4 WU, 50% FC II, 50% FC III, 6MWD 472 ± 118 m, BNP 490 ± 823 ng/L. Patients were treated for 1628 ± 1186 days with sildenafil (100%), bosentan (33%), calcium channel blockers (33%), diuretics (69%), and oxygen (nocturnal 53%, daytime 11%). Values at last visit were: FC (II 75%, III 25%), 6MWD of 496 ± 108 m, BNP of 576 ± 5774 ng/L. Compared to European PVD registries, ambulatory PVD patients living >2500 m revealed similar blood gases and relatively low and stable risk factor profiles despite severe hemodynamic compromise, suggesting that favorable outcomes are achievable for altitude residents with PVD. Future studies should focus on long-term outcomes in PVD patients dwelling >2500 m.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12404"},"PeriodicalIF":2.2,"publicationDate":"2024-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11224915/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141555366","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 : 2024-07-03eCollection Date: 2024-07-01DOI: 10.1002/pul2.12379
Sean E DeWolf, Alana A Hawkes, Sunil M Kurian, Diana E Gorial, Mark L Hepokoski, Stephanie S Almeida, Isabella R Posner, Dianne B McKay
{"title":"Human pulmonary microvascular endothelial cells respond to DAMPs from injured renal tubular cells.","authors":"Sean E DeWolf, Alana A Hawkes, Sunil M Kurian, Diana E Gorial, Mark L Hepokoski, Stephanie S Almeida, Isabella R Posner, Dianne B McKay","doi":"10.1002/pul2.12379","DOIUrl":"10.1002/pul2.12379","url":null,"abstract":"<p><p>Acute kidney injury (AKI) causes distant organ dysfunction through yet unknown mechanisms, leading to multiorgan failure and death. The lungs are one of the most common extrarenal organs affected by AKI, and combined lung and kidney injury has a mortality as high as 60%-80%. One mechanism that has been implicated in lung injury after AKI involves molecules released from injured kidney cells (DAMPs, or damage-associated molecular patterns) that promote a noninfectious inflammatory response by binding to pattern recognition receptors (PRRs) constitutively expressed on the pulmonary endothelium. To date there are limited data investigating the role of PRRs and DAMPs in the pulmonary endothelial response to AKI. Understanding these mechanisms holds great promise for therapeutics aimed at ameliorating the devastating effects of AKI. In this study, we stimulate primary human microvascular endothelial cells with DAMPs derived from injured primary renal tubular epithelial cells (RTECs) as an ex-vivo model of lung injury following AKI. We show that DAMPs derived from injured RTECs cause activation of Toll-Like Receptor and NOD-Like Receptor signaling pathways as well as increase human primary pulmonary microvascular endothelial cell (HMVEC) cytokine production, cell signaling activation, and permeability. We further show that cytokine production in HMVECs in response to DAMPs derived from RTECs is reduced by the inhibition of NOD1 and NOD2, which may have implications for future therapeutics. This paper adds to our understanding of PRR expression and function in pulmonary HMVECs and provides a foundation for future work aimed at developing therapeutic strategies to prevent lung injury following AKI.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12379"},"PeriodicalIF":2.2,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11220341/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498800","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 : 2024-07-02eCollection Date: 2024-07-01DOI: 10.1002/pul2.12402
Catherine M Avitabile, Jena P Mota, Kiley M Yeaman, Sybil J Andrieux, Lara Lechtenberg, Emma Escobar, John Chuo, Melissa S Xanthopoulos, Walter Faig, Shannon M O'Malley, Elizabeth Ford, Michael G McBride, Stephen M Paridon, Jonathan A Mitchell, Babette S Zemel
{"title":"Creating a digital approach for promoting physical activity in pediatric pulmonary hypertension: A framework for future interventions.","authors":"Catherine M Avitabile, Jena P Mota, Kiley M Yeaman, Sybil J Andrieux, Lara Lechtenberg, Emma Escobar, John Chuo, Melissa S Xanthopoulos, Walter Faig, Shannon M O'Malley, Elizabeth Ford, Michael G McBride, Stephen M Paridon, Jonathan A Mitchell, Babette S Zemel","doi":"10.1002/pul2.12402","DOIUrl":"10.1002/pul2.12402","url":null,"abstract":"<p><p>Children with pulmonary hypertension (PH) often demonstrate limited exercise capacity. Data support exercise as an effective nonpharmacologic intervention among adults with PH. However, data on exercise training in children and adolescents are limited, and characteristics of the optimal exercise program in pediatric PH have not been identified. Exercise programs may have multiple targets, including muscle deficits which are associated with exercise limitations in both adult and pediatric PH. Wearable accelerometer sensors measure physical activity volume and intensity in the naturalistic setting and can facilitate near continuous data transfer and bidirectional communication between patients and the study team when paired with informatics tools during exercise interventions. To address the knowledge gaps in exercise training in pediatric PH, we designed a prospective, single arm, nonrandomized pilot study to determine feasibility and preliminary estimates of efficacy of a 16-week home exercise intervention, targeting lower extremity muscle mass and enriched by wearable mobile health technology. The exercIse Training in pulmONary hypertEnsion (iTONE) trial includes (1) semistructured exercise prescriptions tailored to the participant's baseline level of activity and access to resources; (2) interval goal setting fostering self-efficacy; (3) real time monitoring of activity via wearable devices; (4) a digital platform enabling communication and feedback between participant and study team; (5) multiple avenues to assess participant safety. This pilot intervention will provide information on the digital infrastructure needed to conduct home-based exercise interventions in PH and will generate important preliminary data on the effect of exercise interventions in youth with chronic cardiorespiratory conditions to power larger studies in the future.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12402"},"PeriodicalIF":2.2,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11219287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498799","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":"Balloon pulmonary angioplasty for chronic thromboembolic pulmonary disease without pulmonary hypertension.","authors":"Takatoyo Kiko, Ryotaro Asano, Hiroyuki Endo, Naruhiro Nishi, Hiroya Hayashi, Akiyuki Kotoku, Hiroki Horinouchi, Jin Ueda, Tatsuo Aoki, Akihiro Tsuji, Tetsuya Fukuda, Takeshi Ogo","doi":"10.1002/pul2.12409","DOIUrl":"10.1002/pul2.12409","url":null,"abstract":"<p><p>Balloon pulmonary angioplasty (BPA) is beneficial for patients with chronic thromboembolic pulmonary disease (CTEPD) with pulmonary hypertension (PH). However, the clinical benefit of BPA for the patients with CTEPD without PH remains unknown. In this study, we aimed to evaluate the efficacy, safety, and long-term outcomes of BPA in patients with CTEPD without PH. We retrospectively analyzed the data from 84 CTEPD patients with mean pulmonary artery pressure (mPAP) < 25 mmHg and 39 CTEPD patients with mPAP ≤ 20 mmHg (without PH). Among the 39 patients with CTEPD without PH, 14 underwent BPA (BPA-treated group), and the remaining 25 received no treatment (untreated group). In the patients with CTEPD without PH, BPA led to improvements in symptoms, pulmonary vascular resistance (3.6 ± 1.6 to 2.6 ± 1.1 Wood units, <i>p</i> < 0.001), peak oxygen consumption (16.1 ± 4.0 to 18.8 ± 4.3 mL/kg/min, <i>p</i> = 0.033), minute ventilation versus carbon dioxide production slope (41.4 ± 12.2 to 35.1 ± 6.7, <i>p</i> = 0.026), and mPAP/cardiac output slope (7.0 ± 2.6 to 4.4 ± 2.0 mmHg/L/min, <i>p</i> = 0.004) and facilitated the discontinuation of home oxygenation therapy, with no serious complications. Kaplan-Meier analysis showed no significant difference in all-cause mortality between the untreated and BPA-treated groups. BPA may be a safe treatment option for the patients with CTEPD without PH that can alleviate symptoms, improve exercise capacity, and facilitate weaning from home oxygen therapy. Further prospective randomized trials are needed to confirm these findings.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12409"},"PeriodicalIF":2.2,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11219285/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498798","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":"A systematic review of the impact of pulmonary thromboendarterectomy on health-related quality of life.","authors":"Aarohanan Raguragavan, Dujinthan Jayabalan, Sugam Dhakal, Akshat Saxena","doi":"10.1002/pul2.12407","DOIUrl":"10.1002/pul2.12407","url":null,"abstract":"<p><p>Pulmonary thromboendarterectomy (PTE) is the current gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH) and is a viable treatment option for chronic thromboembolic pulmonary disease (CTEPD). The progressive nature of both diseases severely impacts health-related quality of life (HRQoL) across a variety of domains. This systematic review was performed to evaluate the impact of PTE on short- and long-term HRQoL. A literature search was conducted on PubMed for studies matching the eligibility criteria between January 2000 and September 2022. OVID (MEDLINE), Google Scholar, EBSCOhost (EMBASE), and bibliographies of included studies were reviewed. Inclusion of studies was based on predetermined eligibility criteria. Quality appraisal and data tabulation were performed using predetermined forms. Results were synthesized by narrative review. The structure of this systematic review follows the PRISMA guidelines. This systematic review was prospectively registered in the PROSPERO register (CRD42022342144). Thirteen studies (2184 patients) were included. Within 3 months post-PTE, HRQoL improved in both CTEPD and CTEPH as measured by disease-specific and generic questionnaires. HRQoL improvements were sustained up to 5 years postoperatively in patients with CTEPH post-PTE. PTE remains the gold standard for treating CTEPH and improving HRQoL. Residual pulmonary hypertension and comorbidities such as COPD and coronary artery disease decrement HRQoL over time. The impact of mPAP and PVR on HRQoL outcomes postoperatively remain ambiguous. Pulmonary thromboendarterectomy remains the gold standard for treating CTEPH and has shown to improve HRQoL outcomes at 3-month sustaining improvements up to 5-year postoperatively. Residual pulmonary hypertension and comorbidities hinder HRQoL outcomes post-PTE.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 3","pages":"e12407"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11217019/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498786","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 : 2024-06-30eCollection Date: 2024-04-01DOI: 10.1002/pul2.12406
Nika Skoro-Sajer, Karen Sheares, Paul Forfia, Gustavo A Heresi, Mitja Jevnikar, Grzegorz Kopeć, Olga Moiseeva, Mario Terra-Filho, Helen Whitford, Zhenguo Zhai, Amélie Beaudet, Virginie Gressin, Catherina Meijer, Yan Zhi Tan, Kohtaro Abe
{"title":"Treatment and management of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross-sectional scientific survey (CLARITY).","authors":"Nika Skoro-Sajer, Karen Sheares, Paul Forfia, Gustavo A Heresi, Mitja Jevnikar, Grzegorz Kopeć, Olga Moiseeva, Mario Terra-Filho, Helen Whitford, Zhenguo Zhai, Amélie Beaudet, Virginie Gressin, Catherina Meijer, Yan Zhi Tan, Kohtaro Abe","doi":"10.1002/pul2.12406","DOIUrl":"10.1002/pul2.12406","url":null,"abstract":"<p><p>Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 2","pages":"e12406"},"PeriodicalIF":2.2,"publicationDate":"2024-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11214874/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470433","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 : 2024-06-26eCollection Date: 2024-04-01DOI: 10.1002/pul2.12394
Mona Lichtblau, Lena Reimann, Lucilla Piccari
{"title":"Pulmonary vascular disease, environmental pollution, and climate change.","authors":"Mona Lichtblau, Lena Reimann, Lucilla Piccari","doi":"10.1002/pul2.12394","DOIUrl":"10.1002/pul2.12394","url":null,"abstract":"<p><p>Pollution and climate change constitute a combined, grave and pervasive threat to humans and to the life-support systems on which they depend. Evidence shows a strong association between pollution and climate change on cardiovascular and respiratory diseases, and pulmonary vascular disease (PVD) is no exception. An increasing number of studies has documented the impact of environmental pollution and extreme temperatures on pulmonary circulation and the right heart, on the severity and outcomes of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH), on the incidence of pulmonary embolism, and the prevalence and severity of diseases associated with PH. Furthermore, the downstream consequences of climate change impair health care systems' accessibility, which could pose unique obstacles in the case of PVD patients, who require a complex and sophisticated network of health interventions. Patients, caretakers and health care professionals should thus be included in the design of policies aimed at adaptation to and mitigation of current challenges, and prevention of further climate change. The purpose of this review is to summarize the available evidence concerning the impact of environmental pollution and climate change on the pulmonary circulation, and to propose measures at the individual, healthcare and community levels directed at protecting patients with PVD.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 2","pages":"e12394"},"PeriodicalIF":2.2,"publicationDate":"2024-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11205889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458967","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 : 2024-06-25eCollection Date: 2024-04-01DOI: 10.1002/pul2.12392
Melody Walker, Helen Moore, Ali Ataya, Ann Pham, Paul A Corris, Reinhard Laubenbacher, Andrew J Bryant
{"title":"A perfectly imperfect engine: Utilizing the digital twin paradigm in pulmonary hypertension.","authors":"Melody Walker, Helen Moore, Ali Ataya, Ann Pham, Paul A Corris, Reinhard Laubenbacher, Andrew J Bryant","doi":"10.1002/pul2.12392","DOIUrl":"10.1002/pul2.12392","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a severe medical condition with a number of treatment options, the majority of which are introduced without consideration of the underlying mechanisms driving it within an individual and thus a lack of tailored approach to treatment. The one exception is a patient presenting with apparent pulmonary arterial hypertension and shown to have vaso-responsive disease, whose clinical course and prognosis is significantly improved by high dose calcium channel blockers. PH is however characterized by a relative abundance of available data from patient cohorts, ranging from molecular data characterizing gene and protein expression in different tissues to physiological data at the organ level and clinical information. Integrating available data with mechanistic information at the different scales into computational models suggests an approach to a more personalized treatment of the disease using model-based optimization of interventions for individual patients. That is, constructing digital twins of the disease, customized to a patient, promises to be a key technology for personalized medicine, with the aim of optimizing use of existing treatments and developing novel interventions, such as new drugs. This article presents a perspective on this approach in the context of a review of existing computational models for different aspects of the disease, and it lays out a roadmap for a path to realizing it.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 2","pages":"e12392"},"PeriodicalIF":2.2,"publicationDate":"2024-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11199193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458966","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}