Pulmonary Circulation最新文献

{"title":"Using PAH-SYMPACT to assess quality of life in patients with pulmonary hypertension associated with chronic lung disease.","authors":"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","doi":"10.1002/pul2.12343","DOIUrl":"https://doi.org/10.1002/pul2.12343","url":null,"abstract":"<p><p>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 aimed at assessing the symptoms and impact of PH on overall function and well-being. We performed a single-center prospective cohort study using PAH-SYMPACT scores to compare symptoms, exercise capacity and HRQOL in patients with PAH and PH-CLD. One hundred and twenty-five patients (99 patients with idiopathic/heritable PAH and 26 with PH-CLD) completed the PAH-SYMPACT questionnaire which consists of 22 questions that assess HRQOL across four domains: cardiopulmonary (CP) symptoms, cardiovascular (CV) symptoms, physical impact (PI), and cognitive/emotional (CE) impact. Higher scores indicate worse HRQOL. We compared patients with PAH and PH-CLD using a Wilcoxon rank sum or chi-squared test as appropriate. Multivariate linear regression analysis was used to assess the relationship between PH classification and SYMPACT scores. Compared to PAH, patients with PH-CLD were older, more likely to use oxygen and had worse functional class and exercise capacity. While there was no significant difference between the two groups in CP, CV, or CE domain scores, patients with PH-CLD had significantly worse PI scores by univariate (1.79 vs. 1.13, <i>p</i> < 0.001) and multivariate analysis (1.61 vs. 1.17, <i>p</i> = 0.02) and overall worse SYMPACT scores (1.19 vs. 0.91, <i>p</i> = 0.03). In conclusion, patients with PH-CLD have worse HRQOL as assessed by the PAH-SYMPACT questionnaire versus patients with PAH. Although PAH-SYMPACT has not been validated in PH-CLD, the results of this study can guide clinicians in understanding the symptoms and impact of PH-CLD relative to PAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10858736/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139723886","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":"Hemodynamic response to inhaled nitric oxide in patients with pulmonary hypertension and chronic kidney disease: A retrospective cohort study.","authors":"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","doi":"10.1002/pul2.12341","DOIUrl":"https://doi.org/10.1002/pul2.12341","url":null,"abstract":"<p><p>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 in PH-CKD. We sought to improve understanding of the clinical significance of the NO pathway in patients with PH-CKD by assessing the hemodynamic response to inhaled NO (iNO) during right heart catheterization (RHC). In this retrospective cohort study, patients with diagnosis codes of PH and stage IV/V CKD or end-stage renal disease and estimated glomerular filtration rate < 60 mL/min/body surface area who underwent RHC and hemodynamic drug study between July 2011 and June 2021 were eligible. Patients with mean pulmonary artery pressure (mPAP) > 20 mmHg and pulmonary vascular resistance (PVR) > 3 Wood units were included. The final cohort included 37 patients (45.9% female, mean age 72.5 ± 9.7 years). A total of 56.7% of the cohort (21/37) had precapillary PH, while 43.2% (16/37) had combined precapillary postcapillary PH (Cpc-PH). Median survival was 3.1 years after RHC. iNO was associated with a significant decrease in both mPAP and PVR. Hemodynamic changes in mPAP and PVR were similar in precapillary and Cpc-PH groups. Among a small subset (<i>n</i> = 14) who were subsequently treated with PAH-targeted therapy, treatment response was mixed and did not reveal significant benefit. Further studies are warranted to better define the potential role of PAH therapy in PH-CKD.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10858735/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139723884","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":"Clinical evaluation of code-based algorithms to identify patients with pulmonary arterial hypertension in healthcare databases.","authors":"Eva-Maria Didden, Di Lu, Andrew Hsi, Monika Brand, Haley Hedlin, Roham T Zamanian","doi":"10.1002/pul2.12333","DOIUrl":"10.1002/pul2.12333","url":null,"abstract":"<p><p>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 catheterization (RHC). This study evaluated algorithms in patients who were recorded in two linkable data assets: the Stanford Healthcare administrative electronic health record database and the Stanford Vera Moulton Wall Center clinical PH database (which records each patient's RHC diagnosis). We assessed the sensitivity and specificity achieved by 16 algorithms (six published). In total, 720 PH patients with linked data available were included and 558 (78%) of these were PAH patients. Algorithms consisting solely of a P(A)H-specific diagnostic code classed all or almost all PH patients as PAH (sensitivity >97%, specificity <12%) while multicomponent algorithms with well-defined temporal sequences of procedure, diagnosis and treatment codes achieved a better balance of sensitivity and specificity. Specificity increased and sensitivity decreased with increasing algorithm complexity. The best-performing algorithms, in terms of fewest misclassified patients, included multiple components (e.g., PH diagnosis, PAH treatment, continuous enrollment for ≥6 months before and ≥12 months following index date) and achieved sensitivities and specificities of around 95% and 38%, respectively. Our findings help researchers tailor their choice and design of code-based PAH algorithms to their research question and demonstrate the importance of including well-defined temporal components in the algorithms.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10851026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139707695","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":"Structured evaluation of unclear dyspnea-An attempt to shorten the diagnostic delay in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.","authors":"Salaheldin Ahmed, Abdulla Ahmed, Göran Rådegran","doi":"10.1002/pul2.12340","DOIUrl":"10.1002/pul2.12340","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10839287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139692833","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":"Experimental <i>Schistosoma haematobium</i> pulmonary hypertension.","authors":"Biruk Kassa, Dara C Fonseca-Balladares, Rahul Kumar, Michael H Lee, Claudia Mickael, Linda Sanders, Kevin Nolan, Brian B Graham","doi":"10.1002/pul2.12336","DOIUrl":"10.1002/pul2.12336","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10835079/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139681487","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 hypertension patient perspectives toward pulmonary rehabilitation","authors":"Morgan Bailey, Hilary M. DuBrock","doi":"10.1002/pul2.12338","DOIUrl":"https://doi.org/10.1002/pul2.12338","url":null,"abstract":"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. We performed a cross-sectional survey of patients with self-reported PH who attended the Pulmonary Hypertension Association (PHA) conference in June 2022 in Atlanta, Georgia, and patients within the PHA listserv. A total of 429 participants completed the survey and were enrolled in the study. The average age of participants was 61 ± 14 years with 83% of participants identifying as female, 51% of patients self-reported as having group 1 PH. Among patients who completed the survey, 41% had previously attended a PR program. Of those who had completed a PR program, 83% reported being satisfied or very satisfied with the program and 86% reported that they would recommend PR to other PH patients. After completion of a PR program, 76% of patients reported an improvement in their quality of life and 88% reported improvement in exercise capacity. Among the patients who had not previously participated in PR (<i>n</i> = 254), 63% reported an interest in participation while 64% cited a lack of discussion from their treatment team as the primary reason for the lack of PR participation. Limitations of the study include sampling and response bias. According to this cross-sectional survey, the majority of PH patients who have participated in PR report improvement in both quality of life and exercise capacity and would recommend PR to other PH patients. The majority of PH patients who have not participated in PR were interested in participation and cited a lack of discussion with their treatment team as one of the primary reasons for the lack of participation. PR is associated with self-reported improvements in quality of life and exercise capacity but remains underutilized among patients with PH.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139560753","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":"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","authors":"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","doi":"10.1002/pul2.12316","DOIUrl":"https://doi.org/10.1002/pul2.12316","url":null,"abstract":"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 capacity. Therefore, we aimed to assess the effects of PEA on hemodynamics and exercise physiology in mild and severe CTEPH patients. We retrospectively studied 18 CTEPH patients with a mild hemodynamic profile (mean pulmonary arterial pressure [mPAP] between 25 and 30 mmHg at rest) and CTEPH patients with a more severe hemodynamic profile (mPAP &gt; 30 mmHg), matched by age, gender, and PVO. Cardiopulmonary exercise testing parameters were evaluated at baseline and 18 months following PEA. At baseline, exercise capacity, defined as oxygen uptake, was less severely impaired in the mild CTEPH group compared to the severe CTEPH group. After PEA, in the mild CTEPH group, ventilatory efficiency and oxygen pulse improved significantly (<i>p</i> &lt; 0.05), however, the change in ventilatory efficiency and oxygen pulse was smaller compared to the severe CTEPH group. Only in the severe CTEPH group exercise capacity improved significantly (<i>p</i> &lt; 0.001). Hence, in the present study, postoperative hemodynamic outcome and the CPET-determined recovery of exercise capacity in mild CTEPH patients did not differ from a matched group of severe CTEPH patients.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139560866","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":"Exercise and pulsatile pulmonary vascular loading in chronic thromboembolic pulmonary disease.","authors":"Sinan Osman, Natasha R Girdharry, Elizabeth Karvasarski, Robert F Bentley, Stephen P Wright, Nadia Sharif, Micheal McInnis, John T Granton, Marc dePerrot, Susanna Mak","doi":"10.1002/pul2.12331","DOIUrl":"10.1002/pul2.12331","url":null,"abstract":"<p><p>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 elevations of pulsatile vascular afterload than resistive elevations in patients with CTEPD without PH. The interdependent physiology of pulmonary venous and PA hemodynamics was also evaluated. Consecutive patients with CTEPD without PH (resting mean PA pressure ≤20 mmHg) undergoing an exercise RHC were identified. Latent resistive and pulsatile abnormalities of pulmonary vascular afterload were defined as an exercise mean PA pressure/cardiac output >3 WU, and PA pulse pressure to PA wedge pressure (PA PP/PAWP) ratio >2.5, respectively. Forty-five patients (29% female, 53 ± 14 years) with CTEPD without PH were analyzed. With exercise, 19 patients had no abnormalities (ExNOR), 26 patients had abnormalities (ExABN) of pulsatile (20), resistive (2), or both (4) elements of pulmonary vascular afterload. Exercise elicited elevations of pulsatile afterload (53%) more commonly than resistive afterload (13%) (<i>p</i> < 0.001). ExABN patients had lower PA compliance and higher pulmonary vascular resistance at rest and exercise and prolonged resistance-compliance time product at rest. The physiological relationship between changes in PA pressures relative to PAWP was disrupted in the ExABN group. In CTEPD without PH, exercise RHC revealed latent pulmonary vascular afterload elevations in 58% of patients with more frequent augmentation of pulsatile than resistive pulmonary vascular afterload.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10799664/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139513428","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":"Impact of body position on hemodynamic measurements during exercise: A tale of two bikes","authors":"Pradhab Kirupaharan, James Lane, Celia Melillo, Deborah Paul, Alla Amoushref, Sami Al Abdi, Adriano R. Tonelli","doi":"10.1002/pul2.12334","DOIUrl":"https://doi.org/10.1002/pul2.12334","url":null,"abstract":"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 due to dyspnea who were referred for exercise RHC at the Cleveland Clinic. Patients were randomized (1:1) to exercise in seated or supine position to a goal of 60 W followed by maximal exercise in the alternate position. We analyzed 17 patients aged 60.3 ± 10.9 years, including 13 females. At rest in the sitting position, patients had significantly lower right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), pulmonary artery wedge pressure (PAWP) and cardiac index (CI). In every stage of exercise (20, 40, and 60 W), the RAP, mPAP, and PAWP were lower in the sitting position. Exercise in the sitting position allowed the identification of preload insufficiency in nine patients. Exercise in either position increased the identification of postcapillary pulmonary hypertension (PH). Body position significantly influences hemodynamics at rest and with exercise; however, mPAP/CO and PAWP/CO were not positionally affected. Hemodynamic measurements in the seated position allowed the detection of preload insufficiency, a condition that was predominantly identified as no PH during supine exercise.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139462219","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":"BMPR2 mutation and clinical response to imatinib in a case of heritable pulmonary arterial hypertension.","authors":"Shine Kumar, Lalitha Biswas, Anju Choorakottayil Pushkaran, Raman Krishna Kumar","doi":"10.1002/pul2.12335","DOIUrl":"10.1002/pul2.12335","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782117/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139425334","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}