39 results on '"Elwing J"'
Search Results
2. REVEAL Score performance in patients with WHO Group 2 pulmonary hypertension
- Author
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Sharp Dimitri, D, primary, Gomez-Arroyo, J, additional, Zhou, C, additional, Mai, E, additional, Pourriahi, M, additional, Cook, J L, additional, and Elwing, J M, additional
- Published
- 2023
- Full Text
- View/download PDF
3. Efficacy and dose-response relationship of oral treprostinil in PAH patients on monotherapy or dual background therapy
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Barbera, J A, primary, Kiely, D, additional, Gruenig, E, additional, Balasubramanian, V, additional, Vizza, C D, additional, Elwing, J, additional, Sood, N, additional, Rao, Y, additional, Holdstock, L, additional, Seaman, S, additional, Broderick, M, additional, and White, R J, additional
- Published
- 2022
- Full Text
- View/download PDF
4. CT-derived vascular pruning assessment in portopulmonary hypertension
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Mai, E, primary, Gomez-Arroyo, J, additional, Pillai, A, additional, Zhou, C, additional, Snapp, K, additional, Demetrio, S, additional, Kay, D, additional, Raouf, A, additional, Elwing, J, additional, Jose, A, additional, and Goutham, M, additional
- Published
- 2022
- Full Text
- View/download PDF
5. Efficacy of oral treprostinil as an add-on therapy for PAH
- Author
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Kiely, D, primary, Grünig, E, additional, Balasubramanian, V, additional, Barberà, J, additional, Vizza, C D, additional, Elwing, J, additional, Sood, N, additional, Rao, Y, additional, Holdstock, L, additional, Seaman, S, additional, Broderick, M, additional, and White, R J, additional
- Published
- 2022
- Full Text
- View/download PDF
6. Mining the Plasma Proteome for Insights into the Molecular Pathology of Pulmonary Arterial Hypertension
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Harbaum, Lars, primary, Rhodes, Christopher J., additional, Wharton, John, additional, Lawrie, Allan, additional, Karnes, Jason H., additional, Desai, Ankit A., additional, Nichols, William C., additional, Humbert, Marc, additional, Montani, David, additional, Girerd, Barbara, additional, Sitbon, Olivier, additional, Boehm, Mario, additional, Novoyatleva, Tatyana, additional, Schermuly, Ralph T., additional, Ghofrani, H. Ardeschir, additional, Toshner, Mark, additional, Kiely, David G., additional, Howard, Luke S., additional, Swietlik, Emilia M., additional, Gräf, Stefan, additional, Pietzner, Maik, additional, Morrell, Nicholas W., additional, Wilkins, Martin R., additional, Southgate, L, additional, Machado, RD, additional, Martin, J, additional, Ouwehand, WH, additional, Pauciulo, MW, additional, Arora, A, additional, Lutz, K, additional, Ahmad, F, additional, Archer, SL, additional, Argula, R, additional, Austin, ED, additional, Badesch, D, additional, Bakshi, S, additional, Barnett, C, additional, Benza, R, additional, Bhatt, N, additional, Burger, CD, additional, Chakinala, M, additional, Elwing, J, additional, Fortin, T, additional, Frantz, RP, additional, Frost, A, additional, Garcia, JGN, additional, Harley, J, additional, He, H, additional, Hill, NS, additional, Hirsch, R, additional, Ivy, D, additional, Klinger, J, additional, Lahm, T, additional, Marsolo, K, additional, Martin, LJ, additional, Nathan, SD, additional, Oudiz, RJ, additional, Rehman, Z, additional, Robbins, I, additional, Roden, DM, additional, Rosenzweig, EB, additional, Saydain, G, additional, Schilz, R, additional, Simms, RW, additional, Simon, M, additional, Tang, H, additional, Tchourbanov, AY, additional, Thenappan, T, additional, Torres, F, additional, Walsworth, AK, additional, Walter, RE, additional, White, RJ, additional, Wilt, J, additional, Yung, D, additional, Kittles, R, additional, Aman, J, additional, Knight, J, additional, Hanscombe, KB, additional, Gall, H, additional, Ulrich, A, additional, Bogaard, HJ, additional, Church, C, additional, Coghlan, JG, additional, Condliffe, R, additional, Corris, PA, additional, Danesino, C, additional, Elliott, CG, additional, Franke, A, additional, Ghio, S, additional, Gibbs, JSR, additional, Houweling, AC, additional, Kovacs, G, additional, Laudes, M, additional, MacKenzie Ross, RV, additional, Moledina, S, additional, Newnham, M, additional, Olschewski, A, additional, Olschewski, H, additional, Peacock, AJ, additional, Pepke-Zaba, J, additional, Scelsi, L, additional, Seeger, W, additional, Shaffer, CM, additional, Sitbon, O, additional, Suntharalingam, J, additional, Treacy, C, additional, Vonk Noordegraaf, A, additional, Waisfisz, Q, additional, Wort, SJ, additional, Trembath, RC, additional, Germain, M, additional, Cebola, I, additional, Ferrer, J, additional, Amouyel, P, additional, Debette, S, additional, Eyries, M, additional, Soubrier, F, additional, and Trégouët, DA, additional
- Published
- 2022
- Full Text
- View/download PDF
7. Corrigendum for health‐related quality of life and hospitalizations in chronic thromboembolic pulmonary hypertension versus idiopathic pulmonary arterial hypertension: And analysis from the Pulmonary Hypertension Association Registry
- Author
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Minhas, J., primary, Narasimmal, S. P., additional, Bull, T. M., additional, De Marco, T., additional, Mc Connell, J. W., additional, Lammi, M. R., additional, Thenappan, T., additional, Feldman, J. P., additional, Sager, J. S., additional, Badesch, D. B., additional, Ryan, J. J., additional, Grinnan, D. C., additional, Zwicke, D., additional, Horn, E. M., additional, Elwing, J. M., additional, Moss, J. E., additional, Eggert, M., additional, Shlobin, O. A., additional, Frantz, R. P., additional, Bartolome, S. D., additional, Mathai, S. C., additional, Mazimba, S., additional, Pugliese, S. C., additional, and Al‐Naamani, N., additional
- Published
- 2022
- Full Text
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8. (653) - Heart Rate Variability (HRV) as a Marker of Treatment Response in PAH
- Author
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Moseley, A.D., O'Donnell, R.E., and Elwing, J.
- Published
- 2024
- Full Text
- View/download PDF
9. Pulmonary Hypertension in Scleroderma ILD Is Associated with Vascular Pruning: Non Invasive Assessment Using High Resolution CT
- Author
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Gomez-Arroyo, J., primary, Stephens, M., additional, Gupta, N., additional, McCormack, F.X., additional, Elwing, J., additional, Amin, R.S., additional, and Mylavarapu, G., additional
- Published
- 2021
- Full Text
- View/download PDF
10. Genetic determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis
- Author
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Rhodes, CJ, Batai, K, Bleda, M, Haimel, M, Southgate, L, Germain, M, Pauciulo, MW, Hadinnapola, C, Aman, J, Girerd, B, Arora, A, Knight, J, Hanscombe, KB, Karnes, JH, Kaakinen, M, Gall, H, Ulrich, A, Harbaum, L, Cebola, I, Ferrer, J, Lutz, K, Swietlik, EM, Ahmad, F, Amouyel, P, Archer, SL, Argula, R, Austin, ED, Badesch, D, Bakshi, S, Barnett, C, Benza, R, Bhatt, N, Bogaard, HJ, Burger, CD, Chakinala, M, Church, C, Coghlan, JG, Condliffe, R, Corris, PA, Danesino, C, Debette, S, Elliott, CG, Elwing, J, Eyries, M, Fortin, T, Franke, A, Frantz, RP, Frost, A, Garcia, JGN, Ghio, S, Ghofrani, H-A, Gibbs, JSR, Harley, J, He, H, Hill, NS, Hirsch, R, Houweling, AC, Howard, LS, Ivy, D, Kiely, DG, Klinger, J, Kovacs, G, Lahm, T, Laudes, M, Machado, RD, Ross, RV, Marsolo, K, Martin, LJ, Moledina, S, Montani, D, Nathan, SD, Newnham, M, Olschewski, A, Olschewski, H, Oudiz, RJ, Ouwehand, WH, Peacock, AJ, Pepke-Zaba, J, Rehman, Z, Robbins, I, Roden, DM, Rosenzweig, EB, Saydain, G, Scelsi, L, Schilz, R, Seeger, W, Shaffer, CM, Simms, RW, Simon, M, Sitbon, O, Suntharalingam, J, Tang, H, Tchourbanov, AY, Thenappan, T, Torres, F, Toshner, MR, Treacy, CM, Noordegraaf, A, Waisfisz, Q, Walsworth, AK, Walter, RE, Wharton, J, White, RJ, Wilt, J, Wort, SJ, Yung, D, Lawrie, A, Humbert, M, Soubrier, F, Trégouët, D-A, Prokopenko, I, Kittles, R, Gräf, S, Nichols, WC, Trembath, RC, Desai, AA, Morrell, NW, Wilkins, MR, Consortium, UK NIHR Bioresource Rare Diseases, Consortium, UK PAH Cohort Study, Consortium, US PAH Biobank, McCarthy, M, Sorbonne Université (SU), Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), Pulmonary medicine, ACS - Pulmonary hypertension & thrombosis, Human genetics, ACS - Atherosclerosis & ischemic syndromes, APH - Quality of Care, and ACS - Microcirculation
- Subjects
Male ,Pulmonary Arterial Hypertension ,Genotyping Techniques ,[SDV]Life Sciences [q-bio] ,Genetic Variation ,HLA-DP alpha-Chains ,Middle Aged ,Polymorphism, Single Nucleotide ,Risk Assessment ,Survival Analysis ,Article ,SOXF Transcription Factors ,Humans ,Female ,Genetic Predisposition to Disease ,HLA-DP beta-Chains ,Genome-Wide Association Study ,Signal Transduction - Abstract
Background Raregenetic variantscause pulmonary arterial hypertension, but the contribution of commongenetic variationto disease risk and natural history is poorly characterised. We tested for genome-wide association for pulmonary arterial hypertension in large international cohorts and assessed the contribution of associated regions to outcomes. Methods We did two separate genome-wide association studies (GWAS) and a meta-analysis of pulmonary arterial hypertension. These GWAS used data from four international case-control studies across 11 744 individuals with European ancestry (including 2085 patients). One GWAS usedgenotypesfrom 5895 whole-genome sequences and the other GWAS used genotyping array data from an additional 5849 individuals.Cross-validationof loci reaching genome-wide significance was sought by meta-analysis. Conditional analysis corrected for the most significant variants at each locus was used to resolve signals for multiple associations. We functionally annotated associated variants and tested associations with duration ofsurvival.All-cause mortalitywas the primary endpoint in survival analyses. Findings A locus nearSOX17(rs10103692, odds ratio 1·80 [95% CI 1·55–2·08], p=5·13 × 10–15) and a second locus inHLA-DPA1andHLA-DPB1(collectively referred to asHLA-DPA1/DPB1here; rs2856830, 1·56 [1·42–1·71], p=7·65 × 10–20) within the class II MHC region were associated with pulmonary arterial hypertension. TheSOX17locus had two independent signals associated with pulmonary arterial hypertension (rs13266183, 1·36 [1·25–1·48], p=1·69 × 10–12; and rs10103692). Functional andepigenomicdata indicate that the risk variants nearSOX17altergene regulationvia anenhanceractive inendothelial cells. Pulmonary arterial hypertension risk variants determined haplotype-specific enhancer activity, and CRISPR-mediated inhibition of the enhancer reducedSOX17expression. TheHLA-DPA1/DPB1rs2856830 genotype was strongly associated with survival. Median survival fromdiagnosisin patients with pulmonary arterial hypertension with the C/C homozygous genotype was double (13·50 years [95% CI 12·07 to >13·50]) that of those with the T/T genotype (6·97 years [6·02–8·05]), despite similar baselinedisease severity. Interpretation This is the first study to report that common genetic variation at loci in an enhancer nearSOX17and inHLA-DPA1/DPB1is associated with pulmonary arterial hypertension. Impairment of SOX17 function might be more common in pulmonary arterial hypertension than suggested by raremutationsinSOX17. Further studies are needed to confirm the association betweenHLA typingor rs2856830 genotyping and survival, and to determine whether HLA typing or rs2856830 genotyping improvesrisk stratificationin clinical practice or trials.
- Published
- 2019
- Full Text
- View/download PDF
11. Genetic determinants of risk in pulmonary arterial hypertension: international case-control studies and meta-analysis
- Author
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Rhodes, CJ, Batai, K, Bleda, M, Haimel, M, Southgate, L, Germain, M, Pauciulo, MW, Hadinnapola, C, Aman, J, Girerd, B, Arora, A, Robbins, I, Roden, DM, Rosenzweig, EB, Saydain, G, Scelsi, L, Schilz, R, Seeger, W, Shaffer, CM, Simms, RW, Simon, M, Walter, RE, Sitbon, O, Suntharalingam, J, Tang, H, Tchourbanov, AY, Thenappan, T, Torres, F, Toshner, MR, Treacy, CM, Noordegraaf, AV, Waisfisz, Q, Wharton, J, Walsworth, AK, White, RJ, Wilt, J, Wort, SJ, Yung, D, Lawrie, A, Humbert, M, Soubrier, F, Trégouët, D-A, Knight, J, Prokopenko, I, Kittles, R, Gräf, S, Nichols, WC, Trembath, RC, Desai, AA, Morrell, NW, Wilkins, MR, UK NIHR BioResource Rare Diseases Consortium, UK PAH Cohort Study Consortium, Hanscombe, KB, US PAH Biobank Consortium, Karnes, JH, Kaakinen, M, Gall, H, Ulrich, A, Harbaum, L, Cebola, I, Ferrer, J, Lutz, K, Swietlik, EM, Ahmad, F, Amouyel, P, Archer, SL, Argula, R, Austin, ED, Badesch, D, Bakshi, S, Barnett, C, Benza, R, Bhatt, N, Bogaard, HJ, Burger, CD, Chakinala, M, Church, C, Coghlan, JG, Condliffe, R, Corris, PA, Danesino, C, Debette, S, Elliott, CG, Elwing, J, Eyries, M, Fortin, T, Franke, A, Frantz, RP, Frost, A, Garcia, JGN, Ghio, S, Ghofrani, H-A, Gibbs, JSR, Harley, J, He, H, Hill, NS, Hirsch, R, Houweling, AC, Howard, LS, Ivy, D, Kiely, DG, Klinger, J, Kovacs, G, Lahm, T, Laudes, M, Machado, RD, Ross, RVM, Marsolo, K, Martin, LJ, Moledina, S, Montani, D, Nathan, SD, Newnham, M, Olschewski, A, Olschewski, H, Oudiz, RJ, Ouwehand, WH, Peacock, AJ, Pepke-Zaba, J, Rehman, Z, British Heart Foundation, Wellcome Trust, and Medical Research Council (MRC)
- Subjects
ALPHA ,Science & Technology ,US PAH Biobank Consortium ,Critical Care Medicine ,ENDODERM FORMATION ,General & Internal Medicine ,UK NIHR BioResource Rare Diseases Consortium ,Respiratory System ,UK PAH Cohort Study Consortium ,SUSCEPTIBILITY ,SOX17 ,Life Sciences & Biomedicine ,DISEASE - Abstract
Background Rare genetic variants cause pulmonary arterial hypertension, but the contribution of common genetic variation to disease risk and natural history is poorly characterised. We tested for genome-wide association for pulmonary arterial hypertension in large international cohorts and assessed the contribution of associated regions to outcomes. Methods We did two separate genome-wide association studies (GWAS) and a meta-analysis of pulmonary arterial hypertension. These GWAS used data from four international case-control studies across 11 744 individuals with European ancestry (including 2085 patients). One GWAS used genotypes from 5895 whole-genome sequences and the other GWAS used genotyping array data from an additional 5849 individuals. Cross-validation of loci reaching genome-wide significance was sought by meta-analysis. Conditional analysis corrected for the most significant variants at each locus was used to resolve signals for multiple associations. We functionally annotated associated variants and tested associations with duration of survival. All-cause mortality was the primary endpoint in survival analyses. Findings A locus near SOX17 (rs10103692, odds ratio 1·80 [95% CI 1·55–2·08], p=5·13 × 10–15) and a second locus in HLA-DPA1 and HLA-DPB1 (collectively referred to as HLA-DPA1/DPB1 here; rs2856830, 1·56 [1·42–1·71], p=7·65 × 10–20) within the class II MHC region were associated with pulmonary arterial hypertension. The SOX17 locus had two independent signals associated with pulmonary arterial hypertension (rs13266183, 1·36 [1·25–1·48], p=1·69 × 10–12; and rs10103692). Functional and epigenomic data indicate that the risk variants near SOX17 alter gene regulation via an enhancer active in endothelial cells. Pulmonary arterial hypertension risk variants determined haplotype-specific enhancer activity, and CRISPR-mediated inhibition of the enhancer reduced SOX17 expression. The HLA-DPA1/DPB1 rs2856830 genotype was strongly associated with survival. Median survival from diagnosis in patients with pulmonary arterial hypertension with the C/C homozygous genotype was double (13·50 years [95% CI 12·07 to >13·50]) that of those with the T/T genotype (6·97 years [6·02–8·05]), despite similar baseline disease severity. Interpretation This is the first study to report that common genetic variation at loci in an enhancer near SOX17 and in HLA-DPA1/DPB1 is associated with pulmonary arterial hypertension. Impairment of SOX17 function might be more common in pulmonary arterial hypertension than suggested by rare mutations in SOX17. Further studies are needed to confirm the association between HLA typing or rs2856830 genotyping and survival, and to determine whether HLA typing or rs2856830 genotyping improves risk stratification in clinical practice or trials.
- Published
- 2018
12. 1741: CHEMOTHERAPY: A THERAPEUTIC OPTION IN SEPSIS?
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Medepalli K, Lebeck-Lee C, Elwing J, and Benninger L
- Subjects
Sepsis ,Chemotherapy ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,medicine ,Critical Care and Intensive Care Medicine ,Intensive care medicine ,medicine.disease ,business - Published
- 2016
- Full Text
- View/download PDF
13. (653) - Heart Rate Variability (HRV) as a Marker of Treatment Response in PAH.
- Author
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Jose, A., Moseley, A.D., O'Donnell, R.E., and Elwing, J.
- Subjects
- *
HEART beat - Published
- 2024
- Full Text
- View/download PDF
14. Utilization of risk assessment tools in management of PAH: A PAH provider survey
- Author
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Sandeep Sahay, Vijay Balasubramanian, Humna Memon, Abby Poms, Eduardo Bossone, Kristine Highland, Dana Kay, Deborah J Levine, Christopher J Mullin, Lana Melendres‐Groves, Stephen C Mathai, Francisco J Soto, Oksana Shlobin, Jean M Elwing, Sahay, S., Balasubramanian, V., Memon, H., Poms, A., Bossone, E., Highland, K., Kay, D., Levine, D. J., Mullin, C. J., Melendres-Groves, L., Mathai, S. C., Soto, F. J., Shlobin, O., and Elwing, J. M.
- Subjects
Pulmonary and Respiratory Medicine ,pulmonary arterial hypertension ,risk assessment ,REVEAL 2.0 ,survival ,quality improvement - Abstract
Pulmonary arterial hypertension (PAH) is a chronically progressive fatal disease. A goal-oriented approach to achieve low risk status has been associated with improved survival. A variety of risk stratification tools are available, but use is low. We conducted a survey to assess potential reasons for under-utilization. We conducted a survey-based study of global PAH disease specialists with a goal of assessing risk assessment utilization and identifying modifiable barriers to use. The survey was designed by the American College of Chest Physicians’ Pulmonary Vascular Diseases (PVD) NetWork. Respondents were global members of the PVD NetWork and Pulmonary Hypertension Association. Survey invitations were sent electronically to all members. Participation was anonymous and no provider or patient level data was collected. Participants from four countries responded with the majority (84%) being from the United States. Our survey found suboptimal use of any risk stratification tool with 71/112 (63%) reporting use. A total of 85% of the respondents had more than 5 years of experience in managing PAH. REVEAL 2.0 and European Society of Cardiology/European Respiratory Society risk tools were the most commonly used. A total of 44 (65%) surveyed felt that use of risk tools led to change in PAH therapies. Only 6 (9%) felt they prompted additional testing or changed the frequency of follow-up. A total of 5 (7%) reported they prompted goals of care/palliative care discussions and 2 (3%) that they triggered lung transplant referral. The vast majority indicated that incorporation of risk tools into electronic medical records (EMR) would improve utilization. PAH risk assessment tools remain under-utilized. Most respondents were experienced PAH clinicians. More than one-third were not routinely using risk tools. Most felt that risk tools led to PAH therapy changes but few reported impacts on other aspects of care. The most commonly identified barriers to use were time constraints and lack of integration with EMR.
- Published
- 2021
15. Treatment with Oral or Inhaled Treprostinil in Patients with Pulmonary Arterial Hypertension and Cardiovascular Comorbidities.
- Author
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White RJ, El-Kersh K, Rosenkranz S, Franco V, Vizza CD, Badagliacca R, Pepke-Zaba J, Elwing J, Argula RG, Shapiro S, Kim H, Seaman S, Shen E, Das M, Broderick M, and McLaughlin V
- Abstract
Background: An increasing number of patients with pulmonary arterial hypertension (PAH) have cardiovascular comorbidities. However, the effects of comorbidities on responses to PAH treatment are not well understood., Research Question: Do cardiovascular comorbidities in patients with PAH influence the efficacy and tolerability of inhaled or oral treprostinil?, Study Design and Methods: All patients from phase 3 studies TRIUMPH (N = 235) and FREEDOM-EV (N = 690) were included in this post hoc analysis and were classified as having 0, ≥1, or ≥2 cardiovascular comorbidities of interest based on patients' medical histories. The mean difference in 6-minute walk distance (6MWD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) from baseline to Week 12 was assessed for TRIUMPH and the risk and incidence of clinical worsening was assessed for patients in FREEDOM-EV. Adverse events (AEs) were summarized for each comorbidity grouping for TRIUMPH and FREEDOM-EV., Results: In TRIUMPH, there were 79, 156, and 88 patients with 0, ≥1, and ≥2 comorbidities, respectively. Patients on inhaled treprostinil had improvements in 6MWD, with numerically similar improvements for comorbidity subgroups (0: 26 m, P = 0.020; ≥1: 22 m, P = 0.006; ≥2: 21.6 m, P = 0.043). Significant reductions in NT-proBNP were also seen in all subgroups. In FREEDOM-EV, there were 375, 315, and 166 patients with 0, ≥1, and ≥2 comorbidities, respectively. Regardless of comorbidities, patients on oral treprostinil had a significantly reduced risk of clinical worsening compared with placebo (0: 36% reduction, P = 0.034; ≥1: 41% reduction, P = 0.014; ≥2: 45% reduction, P = 0.026). In TRIUMPH and FREEDOM-EV, AE profiles were typical for this class of medication regardless of the number of comorbidities. A sensitivity analysis using a subset of comorbidities confirmed the findings of our primary analysis., Interpretation: This post hoc analysis suggests that patients with PAH and cardiovascular comorbidities can benefit from combination therapy with inhaled or oral treprostinil., (Copyright © 2024. Published by Elsevier Inc.)
- Published
- 2024
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16. Pulmonary Vascular Disease in Chronic Obstructive Pulmonary Disease.
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Kay D, Bernardo R, and Elwing J
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- Humans, Hemodynamics, Heart Failure physiopathology, Heart Failure etiology, Pulmonary Disease, Chronic Obstructive physiopathology, Pulmonary Disease, Chronic Obstructive complications, Hypertension, Pulmonary physiopathology, Hypertension, Pulmonary etiology, Vascular Resistance, Pulmonary Artery physiopathology
- Abstract
Pulmonary hypertension (PH) is a vascular disease characterized by pulmonary artery remodeling and right heart failure. PH related to COPD is a precapillary form of the disease, with hemodynamic measurements including a mean pulmonary artery pressure of greater than 20 mm Hg, a wedge pressure of less than 15 mm Hg, and a pulmonary vascular resistance of greater than 3 WU (Woods units), categorized under the World Health Organization classification as group 3. The presence of PH in COPD has been known to increase morbidity and mortality. Limited studies have evaluated treatment options for PH related to COPD., Competing Interests: None declared., (Thieme. All rights reserved.)
- Published
- 2024
- Full Text
- View/download PDF
17. Mortality Risk Assessment Using the REVEAL 2.0 Score in Pulmonary Hypertension Secondary to Left Heart Disease.
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Sharp-Dimitri D, Pourriahi M, Zhou C, Jandarov R, Kay D, Jose A, Cook J, Elwing J, and Gomez-Arroyo J
- Abstract
Background: Pulmonary hypertension (PH) frequently complicates the course of patients with left heart disease (PH-LHD) and is associated with worse clinical outcomes. Mortality calculators for PH-LHD are lacking, and it is unclear whether any risk prediction tools originally derived from other forms of PH can accurately predict outcomes in patients with PH-LHD., Methods: We retrospectively analyzed data from 161 patients diagnosed with PH-LHD referred to our pulmonary hypertension center from 2016 to 2022. We calculated the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL 2.0) risk score and categorized patients as low, intermediate, or high-risk. We assessed survival at 1 and 3 years using Kaplan-Meier and Cox proportional hazards, as well as classification performance using a concordance index., Results: At the first outpatient visit, 15% of patients were stratified as low-risk, 27% as intermediate, and 57% as high-risk. Cumulative 1-year survival rates were 100%, 94%, and 91% for the low, intermediate, and high-risk strata, respectively. Cumulative 3-year survival rates were 96%, 89%, and 70% for the low, intermediate, and high-risk strata, respectively. We found no difference in outcomes at 1 year between risk groups. High-risk patients had an increased risk of death at 3 years using REVEAL 2.0 (HR 5.32, p < 0.001). However, while REVEAL 2.0 accurately discriminated high-risk patients, the hazard ratio was not statistically different between patients classified as intermediate-risk compared to low-risk., Conclusion: REVEAL 2.0 accurately predicted 3-year survival in PH-LHD patients with high-risk features. However, the mortality risk between patients classified as intermediate-risk was not different from the low-risk stratum, suggesting inaccurate classification for this group of patients., Competing Interests: Disclosures: No commercial disclosures. None of the authors have any conflict of interest.
- Published
- 2024
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18. Semaglutide improves metabolic dysfunction-associated steatohepatitis: A 10-year retrospective study.
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Shah P, White M, Sievert A, Conway A, Kneepkens A, Sayuk G, Lisker-Melman M, and Elwing J
- Abstract
Background and Aims: Semaglutide has been studied in patients with metabolic dysfunction-associated steatohepatitis (MASH) due to potential benefit from weight loss on liver inflammation. However, preclinical studies suggest that MASH improvement may be independent of weight loss. We aim to assess the impact of semaglutide on MASH in relation to weight loss., Methods: This retrospective study included 420 patients with diabetes on semaglutide for at least 12 months between 2011 and 2022. Exclusion criteria were liver disease other than MASH, decompensated cirrhosis, malignancy, and bariatric surgery. Primary endpoints were clinically significant improvements in AST or ALT (mean difference > 6.3 U/L and > 10.6 U/L respectively). Statistical analysis included Student's t-test/ANOVA, Wilcoxon signed-rank test/Friedman test as appropriate, and binary logistic regression., Results: Median duration of semaglutide was 22.5 months and 80% of patients received 1 mg/week. BMI improved by a mean (SD) of 1.9 points (2.8), weight by 13.3 lbs. (19.1), AST by 4.1 U/L (11.5), and ALT by 5.3 U/L (14.2). In 28% and 22% of patients respectively, AST and ALT had a clinically significant improvement. MASH scores (NFS, FIB4, APRI) improved after semaglutide ( p < 0.001). No statistically significant differences in AST or ALT improvement were found when patients were stratified by BMI prior to semaglutide or when stratified by percentage of weight loss. On logistic regression, the duration of semaglutide and pretreatment APRI score increased the odds of clinically significant improvements of AST and ALT., Conclusion: Semaglutide treatment was associated with improvement in transaminases and MASH scores. Higher odds of positive semaglutide effects were observed with longer treatment duration and were independent of weight loss., (© 2024 The Authors. JGH Open published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)
- Published
- 2024
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19. Oral Treprostinil is Associated with Improved Survival in FREEDOM-EV and its Open-Label Extension.
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Grünig E, Rahaghi F, Elwing J, Vizza CD, Pepke-Zaba J, Shen J, Yao H, Hage A, Rosenkranz S, Vonk M, Balasubramanian V, Yuanhua Y, Yu Z, Lordan J, Cadaret L, Grover R, Ousmanou A, Seaman S, Deng C, Broderick M, and White RJ
- Subjects
- Humans, Treatment Outcome, Epoprostenol adverse effects, Antihypertensive Agents, Hypertension, Pulmonary drug therapy
- Abstract
Introduction: In the event-driven FREEDOM-EV trial, oral treprostinil delayed clinical worsening in patients with pulmonary arterial hypertension (PAH). Open-label extension studies offer additional data about tolerability, efficacy, and survival, especially for those initially assigned placebo. The aim of the current study was to determine if oral treprostinil changed survival when considering the parent and extension study, if treprostinil provides functional benefits for participants initially assigned to placebo, and if the benefits observed for those treated with treprostinil were durable., Methods: Both active and placebo participants from FREEDOM-EV could enroll in the FREEDOM-EV open-label extension (OLE) study after experiencing an investigator-assessed clinical worsening event or after parent study closure. All participants in the OLE were offered open-label oral treprostinil. Previously assigned placebo participants titrated to maximally tolerated doses; previously assigned treprostinil participants continued dose titration. We repeated assessments including functional class and 6-min walk distance (6MWD) at 12-week intervals and measured N-terminal pro-brain natriuretic peptide (NT-proBNP) at week 48. Survival was estimated by Kaplan-Meier analysis, and we estimated hazard ratio (HR) using Cox proportional hazards., Results: Of 690 FREEDOM-EV participants, 470 enrolled in the OLE; vital status was available for 89% of initial Freedom-EV participants. When considering the combined parent and open-label data, initial assignment to oral treprostinil reduced mortality (HR 0.64, 95% confidence interval 0.46-0.91, p = 0.013); absolute risk reduction was 9%. Participants randomized to placebo who initiated oral treprostinil after clinical worsening and tolerated treatment through week 48 demonstrated favorable shifts in functional class (p < 0.0001), 6MWD improvements of + 84 m (p < 0.0001), and a reduction in NT-proBNP of - 778 pg/mL (p = 0.02), compared to OLE baseline. Modest trends toward benefit were measured for those initially assigned placebo who did not have clinical worsening, and 132/144 (92%) of treprostinil assigned participants without clinical worsening remained on drug at week 48 in the OLE study. Adverse events were consistent with FREEDOM-EV., Conclusion: Initial treprostinil assignment improved survival in the entire data set; those who began treprostinil after a clinical worsening in the placebo arm and tolerated drug to week 48 enjoyed substantial functional gains., Clinical Trial Registration: ClinicalTrials.gov identifier NCT01560637., (© 2023. The Author(s).)
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- 2024
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20. 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.
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Morland K, Gerges C, Elwing J, Visovatti SH, Weatherald J, Gillmeyer KR, Sahay S, Mathai SC, Boucly A, Williams PG, Harikrishnan S, Minty EP, Hobohm L, Jose A, Badagliacca R, Lau EMT, Jing ZC, Vanderpool RR, Fauvel C, Leonidas Alves J Jr, Strange G, Pulido T, Qian J, Li M, Mercurio V, Zelt JGE, Moles VM, Cirulis MM, Nikkho SM, Benza RL, and Elliott CG
- Abstract
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, we review real-world data (RWD) sources, discuss challenges and opportunities when using RWD sources to study PH populations, and identify resources needed to support the generation of meaningful RWE for the global PH community., Competing Interests: Boucly reports consulting fees from AOP Orphan; payment, honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from AOP Orphan, MSD, and Janssen; and support for attending meetings/travel from MSD and Janssen. Moles reports grants or contracts from Acceleron Pharma, Janssen, and CVS Caremark; and leadership or fiduciary role in other board, society, committee, or advocacy group, paid or unpaid, for Gossamer Bio. Elwing reports grants or contracts from United Therapeutics Corporation, Gossamer Bio, Bayer, Acceleron/Merck, Altavant, Aerovate, Tenax, Pharmosa, Actelion/Janssen, Arena, Lung, Liquidia, and Phase Bio; consulting fees for United Therapeutics Corporation; support for attending meetings and/or travel for United Therapeutics, Altavant, Aerovate, Bayer, Gossamer Bio, Liquida, Acceleron/Merck, Janssen/Actelion, and Insmed; and other financial or nonfinancial interests from Bayer as a grant reviewer. Jose reports grants or contracts from Team Phenomenal Hope Charity, Francis Family Foundation, United Therapeutics, and Janssen/Johnson & Johnson. Alves Jr. reports payment or honoraria from Bayer and Janssen; and support for attending meetings and/or travel from Janssen. Minty reports consulting fees from Janssen Pharmaceuticals and Orpyx Medical Technologies; and stock or stock options from Orpyx Medical Technologies. Weatherald reports grants or contracts from Janssen, Bayer, Merck, Canadian Institutes for Health Research, and the Heart & Stroke Foundation of Canada; consulting fees from Janssen and Merck; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Janssen and Merck; payment for expert testimony from Sprigings Intellectual Property Law; support for attending meetings and/or travel from Janssen; participation on a data safety monitoring board or advisory board from Janssen, Acceleron, and Université Laval; and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the Pulmonary Hypertension Association of Canada. Mathai reports grants or contracts from NIH/NHLBI (HL125175) and DOD (W81XWH‐20‐1‐0768); consulting fees from Actelion/Janssen, Aerovate, Merck, United Therapeutics Corporation, and Theravance; and participation on a data safety monitoring board or advisory board from Bayer. Sahay reports grants or contracts from United Therapeutics Corporation, Janssen, Bayer, Merck, Liquidia, and Gossamer; consulting fees from Merck, Liquidia, and Gossamer; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from United Therapeutics Corporation (received by hospital rather than self); patents planned, issued, or pending with Janssen; and participation on a data safety monitoring board or advisory board with GSK, Bayer, United Therapeutics, and Janssen. Pulido reports grants or contracts from Janssen, MSD, and United Therapeutics; consulting fees from Janssen, Bayer, Gossamer, and MSD; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Bayer, Janssen, and Pfizer. Badagliacca reports grants or contracts from United Therapeutics Corporation, Ferrer, and Janssen; consulting fees from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP; and support for attending meetings and/or travel from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP. Cirulis reports grants or contracts from Entelligence (now ATS); and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, with ATS Web Committee Co‐Director and ATS Program Committee. Gerges reports grants or contracts from AOP Health; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from AOP Health, Actelion/Janssen, and Ferrer; and support for attending meetings and/or travel from AOP Health, Actelion/Janssen, Ferrer, Daiichi Sankyo, and MSD. Gillmeyer reports grants or contracts from Parker B. Francis Fellowship Award, Doris Duke Charitable Foundation Award, and National Center for Advancing Translational Sciences, National Institutes of Health (BU‐CTSI Grant Number 1UL1TR001430); and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, for the American Thoracic Society Program Committee (unpaid). Hobohm reports consulting fees for Janssen, Boston Consulting, and MSD. Lau reports grants or contracts from Janssen and GSK; consulting fees for Janssen; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Janssen. Morland reports grants or contracts from United Therapeutics Corporation; leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the PVRI Innovative Drug Development Initiative (unpaid); and stock or stock options from United Therapeutics Corporation. Nikkho reports grants or contracts from Bayer AG; and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the PVRI Innovative Drug Development Initiative (unpaid). The remaining authors declare no conflict of interest., (© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)
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- 2023
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21. A Percutaneous Endoscopic Colostomy Tube to the Rescue.
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Numan L, Brotherton T, Baliss M, Ghosh S, Lamm V, Klos C, Sayuk G, Presti M, and Elwing J
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Percutaneous endoscopic colostomy (PEC) tube placement is a minimally invasive procedure used to treat recurrent colonic pseudo-obstruction, sigmoid volvulus, chronic intractable constipation, and neurogenic bowel. PEC is a viable treatment alternative for patients who have failed conservative therapies and are deemed high risk for surgical management. We present a case of acute colonic pseudo-obstruction after Clostridioides difficile infection that was unresponsive to medical treatment or endoscopic decompression. A PEC tube was placed into the transverse colon with successful resolution of the colonic distension., (© 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)
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- 2023
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22. Baseline Features and Reasons for Nonparticipation in the Colonoscopy Versus Fecal Immunochemical Test in Reducing Mortality From Colorectal Cancer (CONFIRM) Study, a Colorectal Cancer Screening Trial.
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Robertson DJ, Dominitz JA, Beed A, Boardman KD, Del Curto BJ, Guarino PD, Imperiale TF, LaCasse A, Larson MF, Gupta S, Lieberman D, Planeta B, Shaukat A, Sultan S, Menees SB, Saini SD, Schoenfeld P, Goebel S, von Rosenvinge EC, Baffy G, Halasz I, Pedrosa MC, Kahng LS, Cassim R, Greer KB, Kinnard MF, Bhatt DB, Dunbar KB, Harford WV Jr, Mengshol JA, Olson JE, Patel SG, Antaki F, Fisher DA, Sullivan BA, Lenza C, Prajapati DN, Wong H, Beyth R, Lieb JG 2nd, Manlolo J, Ona FV, Cole RA, Khalaf N, Kahi CJ, Kohli DR, Rai T, Sharma P, Anastasiou J, Hagedorn C, Fernando RS, Jackson CS, Jamal MM, Lee RH, Merchant F, May FP, Pisegna JR, Omer E, Parajuli D, Said A, Nguyen TD, Tombazzi CR, Feldman PA, Jacob L, Koppelman RN, Lehenbauer KP, Desai DS, Madhoun MF, Tierney WM, Ho MQ, Hockman HJ, Lopez C, Carter Paulson E, Tobi M, Pinillos HL, Young M, Ho NC, Mascarenhas R, Promrat K, Mutha PR, Pandak WM Jr, Shah T, Schubert M, Pancotto FS, Gawron AJ, Underwood AE, Ho SB, Magno-Pagatzaurtundua P, Toro DH, Beymer CH, Kaz AM, Elwing J, Gill JA, Goldsmith SF, Yao MD, Protiva P, Pohl H, and Kyriakides T
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- Adult, Humans, Female, Male, Middle Aged, Occult Blood, Cross-Sectional Studies, Colonoscopy, Early Detection of Cancer, Neoplasms
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Importance: The Colonoscopy Versus Fecal Immunochemical Test in Reducing Mortality From Colorectal Cancer (CONFIRM) randomized clinical trial sought to recruit 50 000 adults into a study comparing colorectal cancer (CRC) mortality outcomes after randomization to either an annual fecal immunochemical test (FIT) or colonoscopy., Objective: To (1) describe study participant characteristics and (2) examine who declined participation because of a preference for colonoscopy or stool testing (ie, fecal occult blood test [FOBT]/FIT) and assess that preference's association with geographic and temporal factors., Design, Setting, and Participants: This cross-sectional study within CONFIRM, which completed enrollment through 46 Department of Veterans Affairs medical centers between May 22, 2012, and December 1, 2017, with follow-up planned through 2028, comprised veterans aged 50 to 75 years with an average CRC risk and due for screening. Data were analyzed between March 7 and December 5, 2022., Exposure: Case report forms were used to capture enrolled participant data and reasons for declining participation among otherwise eligible individuals., Main Outcomes and Measures: Descriptive statistics were used to characterize the cohort overall and by intervention. Among individuals declining participation, logistic regression was used to compare preference for FOBT/FIT or colonoscopy by recruitment region and year., Results: A total of 50 126 participants were recruited (mean [SD] age, 59.1 [6.9] years; 46 618 [93.0%] male and 3508 [7.0%] female). The cohort was racially and ethnically diverse, with 748 (1.5%) identifying as Asian, 12 021 (24.0%) as Black, 415 (0.8%) as Native American or Alaska Native, 34 629 (69.1%) as White, and 1877 (3.7%) as other race, including multiracial; and 5734 (11.4%) as having Hispanic ethnicity. Of the 11 109 eligible individuals who declined participation (18.0%), 4824 (43.4%) declined due to a stated preference for a specific screening test, with FOBT/FIT being the most preferred method (2820 [58.5%]) vs colonoscopy (1958 [40.6%]; P < .001) or other screening tests (46 [1.0%] P < .001). Preference for FOBT/FIT was strongest in the West (963 of 1472 [65.4%]) and modest elsewhere, ranging from 199 of 371 (53.6%) in the Northeast to 884 of 1543 (57.3%) in the Midwest (P = .001). Adjusting for region, the preference for FOBT/FIT increased by 19% per recruitment year (odds ratio, 1.19; 95% CI, 1.14-1.25)., Conclusions and Relevance: In this cross-sectional analysis of veterans choosing nonenrollment in the CONFIRM study, those who declined participation more often preferred FOBT or FIT over colonoscopy. This preference increased over time and was strongest in the western US and may provide insight into trends in CRC screening preferences.
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- 2023
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23. Impact of the COVID-19 pandemic on chronic disease management and patient reported outcomes in patients with pulmonary hypertension: The Pulmonary Hypertension Association Registry.
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Mayer M, Badesch DB, Nielsen KH, Kawut S, Bull T, Ryan JJ, Sager J, Mazimba S, Hemnes A, Klinger J, Runo J, McConnell JW, De Marco T, Chakinala MM, Yung D, Elwing J, Kaplan A, Argula R, Pomponio R, Peterson R, and Hountras P
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To better understand the impact of the COVID-19 pandemic on the care of patients with pulmonary hypertension, we conducted a retrospective cohort study evaluating health insurance status, healthcare access, disease severity, and patient reported outcomes in this population. Using the Pulmonary Hypertension Association Registry (PHAR), we defined and extracted a longitudinal cohort of pulmonary arterial hypertension (PAH) patients from the PHAR's inception in 2015 until March 2022. We used generalized estimating equations to model the impact of the COVID-19 pandemic on patient outcomes, adjusting for demographic confounders. We assessed whether insurance status modified these effects via covariate interactions. PAH patients were more likely to be on publicly-sponsored insurance during the COVID-19 pandemic compared with prior, and did not experience statistically significant delays in access to medications, increased emergency room visits or nights in the hospital, or worsening of mental health metrics. Patients on publicly-sponsored insurance had higher healthcare utilization and worse objective measures of disease severity compared with privately insured individuals irrespective of the COVID-19 pandemic. The relatively small impact of the COVID-19 pandemic on pulmonary hypertension-related outcomes was unexpected but may be due to pre-established access to high quality care at pulmonary hypertension comprehensive care centers. Irrespective of the COVID-19 pandemic, patients who were on publicly-sponsored insurance seemed to do worse, consistent with prior studies highlighting outcomes in this population. We speculate that previously established care relationships may lessen the impact of an acute event, such as a pandemic, on patients with chronic illness., Competing Interests: The authors declare no conflict of interest., (© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)
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- 2023
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24. Inhaled Treprostinil Dosage in Pulmonary Hypertension Associated With Interstitial Lung Disease and Its Effects on Clinical Outcomes.
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Nathan SD, Deng C, King CS, DuBrock HM, Elwing J, Rajagopal S, Rischard F, Sahay S, Broderick M, Shen E, Smith P, Tapson VF, and Waxman AB
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- Humans, Antihypertensive Agents therapeutic use, Treatment Outcome, Epoprostenol therapeutic use, Double-Blind Method, Hypertension, Pulmonary drug therapy, Hypertension, Pulmonary etiology, Lung Diseases, Interstitial complications, Lung Diseases, Interstitial drug therapy, Lung Diseases, Interstitial chemically induced
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Background: Pulmonary hypertension (PH) complicates the course of many patients with fibrotic interstitial lung disease (ILD). Inhaled treprostinil (iTre) has been shown to improve functional ability and to delay clinical worsening in patients with PH resulting from ILD., Research Question: Do higher dosages of iTre have greater benefits in preventing clinical worsening and achieving clinical improvement?, Study Design and Methods: Post hoc analysis of the INCREASE study, a 16-week double-blind, randomized, placebo-controlled trial of iTre in patients with PH resulting from ILD. Four groups were identified based on the number of breaths per session (bps; < 9 and ≥ 9 bps) of active drug or placebo attained at 4 weeks. Patients were evaluated for clinical worsening (15% decrease in 6-min walkdistance, cardiopulmonary hospitalization, lung transplantation, or death) or clinical improvement (15% increase in the six-minute walk distance with a concomitant 30% reduction in N-terminal prohormone of brain natriuretic peptide without any clinical worsening event)., Results: At 4 weeks, 70 patients were at a dose of ≥ 9 bps (high-dosage group) and 79 patients were at a dose of < 9 bps (low-dosage group) in the iTre arm vs 86 patients in the high-dose group and 67 patients in the low-dose group in the placebo arm. Between weeks 4 and 16, 17.1% of patients in the high-dose treprostinil group and 22.8% in the low-dose treatment group experienced a clinical worsening event vs 33.7% and 34.3% of patients in the two placebo arms, respectively (P = .006). By week 16, 15.7% and 12.7% of patients in the high- and low-dose iTre groups, respectively, demonstrated clinical improvement vs 7% and 1.5% patients in the placebo arms (P = .003) INTERPRETATION: Higher dosages of iTre overall show greater benefit in terms of preventing clinical worsening and achieving clinical improvement. These data support the early initiation and uptitration of therapy to a dosage of at least 9 bps four times daily in patients with PH resulting from ILD., Trial Registry: ClinicalTrials.gov; No.: NCT02630316; URL: www., Clinicaltrials: gov., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)
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- 2023
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25. Pulmonary Vascular Research Institute GoDeep: A meta-registry merging deep phenotyping datafrom international PH reference centers.
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Majeed RW, Wilkins MR, Howard L, Hassoun PM, Anthi A, Cajigas HR, Cannon J, Chan SY, Damonte V, Elwing J, Förster K, Frantz R, Ghio S, Al Ghouleh I, Hilgendorff A, Jose A, Juaneda E, Kiely DG, Lawrie A, Orfanos SE, Pepe A, Pepke-Zaba J, Sirenko Y, Swett AJ, Torbas O, Zamanian RT, Marquardt K, Michel-Backofen A, Antoine T, Wilhelm J, Barwick S, Krieb P, Fuenderich M, Fischer P, Gall H, Ghofrani HA, Grimminger F, Tello K, Richter MJ, and Seeger W
- Abstract
The Pulmonary Vascular Research Institute GoDeep meta-registry is a collaboration of pulmonary hypertension (PH) reference centers across the globe. Merging worldwide PH data in a central meta-registry to allow advanced analysis of the heterogeneity of PH and its groups/subgroups on a worldwide geographical, ethnical, and etiological landscape (ClinTrial. gov NCT05329714). Retrospective and prospective PH patient data (diagnosis based on catheterization; individuals with exclusion of PH are included as a comparator group) are mapped to a common clinical parameter set of more than 350 items, anonymized and electronically exported to a central server. Use and access is decided by the GoDeep steering board, where each center has one vote. As of April 2022, GoDeep comprised 15,742 individuals with 1.9 million data points from eight PH centers. Geographic distribution comprises 3990 enrollees (25%) from America and 11,752 (75%) from Europe. Eighty-nine perecent were diagnosed with PH and 11% were classified as not PH and provided a comparator group. The retrospective observation period is an average of 3.5 years (standard error of the mean 0.04), with 1159 PH patients followed for over 10 years. Pulmonary arterial hypertension represents the largest PH group (42.6%), followed by Group 2 (21.7%), Group 3 (17.3%), Group 4 (15.2%), and Group 5 (3.3%). The age distribution spans several decades, with patients 60 years or older comprising 60%. The majority of patients met an intermediate risk profile upon diagnosis. Data entry from a further six centers is ongoing, and negotiations with >10 centers worldwide have commenced. Using electronic interface-based automated retrospective and prospective data transfer, GoDeep aims to provide in-depth epidemiological and etiological understanding of PH and its various groups/subgroups on a global scale, offering insights for improved management., Competing Interests: AL has received support and fees from Janssen, Bayer, Novartis, GSK, Alexion, and IQVIA Ltd. DK has received personal fees for consultancy work giving education talks and participation in steering committees from Acceleron, Ferrer, GSK, and Janssen. His department has received grants for research from GSK and Janssen. EJ is GSL Investigator. HAG has received fees from Actelion, AstraZeneca, Bayer, GSK, Janssen‐Cilag, Lilly, Novartis, OMT, Pfizer, and United Therapeutics. HG has received fees from Actelion, AstraZeneca, Bayer, GSK, Janssen‐Cilag, Lilly, Novartis, OMT, Pfizer, and United Therapeutics. IAG has held research grants from the United Therapeutics and Gilead Sciences Research Scholars Programs. JC has served as a consultant for Acceleron Pharma, Actelion, GSK, and Merck and has received speaker fees from Actelion and GSK. JME has served as a consultant for United Therapeutics, Altavant, Aerovate, Bayer, Gossamer Bio, and Liquida. She has participated in clinical research funded by Janssen, United Therapeutics, Liquidia, Phase Bio, Gossamer Bio, Bayer, Acceleron, Altavant, and Aerovate. KT has received speaker fees from Janssen. MJR has received support from Janssen Pharmaceutica and Bayer Pharma AG, as well as speaker fees from Janssen Pharmaceutica and OMT. Stephen Y. Chan has served as a consultant for Acceleron Pharma and United Therapeutics; SYC (Stephen Y. Chan) has held research grants from Actelion, Bayer, and Pfizer. SYC has filed patent applications regarding metabolic targeting in pulmonary hypertension. SYC is a director, officer, and shareholder of Synhale Therapeutics. WS has received consultancy fees from Abivax, Lung Biotechnology, Liquidia, Medspray, Pieris, United Therapeutics, and Vectura. The remaining authors declare no conflict of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)
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- 2022
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26. Segmental Colitis Associated With Diverticulosis Masquerading as Polyploid-Appearing Mucosa in the Rectosigmoid Area on Endoscopy and as Focal Thickening on Imaging.
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Nwankwo EC Jr, Khneizer G, Sayuk G, Elwing J, Havlioglu N, and Presti M
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Segmental colitis associated with diverticulosis (SCAD) is an inflammatory disease affecting segments of the large bowel with diverticular disease. SCAD presents several challenges in diagnoses and treatment because it often mimics a range of disorders including inflammatory bowel disease and malignancy. Here, we present the case of a 72-year-old man with lower abdominal pain and bloody stools whose initial abdominal workup showed nonspecific large bowel thickening and concerns for malignancy. Ultimately, the patient was diagnosed with mild SCAD and treated conservatively with a resolution of symptoms. He had no symptoms at the three-month and 1-year follow-ups. This case highlights the importance of including SCAD in the initial differential diagnosis to allow accurate identification and treatment., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Nwankwo et al.)
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- 2022
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27. Efficacy of Inhaled Treprostinil on Multiple Disease Progression Events in Patients with Pulmonary Hypertension due to Parenchymal Lung Disease in the INCREASE Trial.
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Nathan SD, Tapson VF, Elwing J, Rischard F, Mehta J, Shapiro S, Shen E, Deng C, Smith P, and Waxman A
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- Administration, Inhalation, Aged, Epoprostenol administration & dosage, Female, Healthy Volunteers, Humans, Hypertension, Pulmonary physiopathology, Longitudinal Studies, Male, Middle Aged, Treatment Outcome, Antihypertensive Agents therapeutic use, Disease Progression, Epoprostenol analogs & derivatives, Epoprostenol therapeutic use, Hypertension, Pulmonary drug therapy, Hypertension, Pulmonary etiology, Lung Diseases, Interstitial complications
- Abstract
Rationale: The INCREASE study of inhaled treprostinil met its primary endpoint of change in 6-minute-walk distance at Week 16. In addition, there were significantly fewer clinical worsening events in patients receiving inhaled treprostinil. However, the incidence of multiple events in the same patient is unknown. Objectives: This post hoc analysis evaluated the effect of continued treatment with inhaled treprostinil on the frequency and impact of multiple disease progression events. Methods: Patients enrolled in INCREASE were analyzed for disease progression events, defined as at least 15% decline in 6-minute-walk distance, exacerbation of underlying lung disease, cardiopulmonary hospitalization, lung transplantation, at least 10% decline in forced vital capacity, or death during the duration of the 16-week study. Measurements and Main Results: In total, 147 disease progression events occurred in the inhaled treprostinil group (89/163 patients, 55%) compared with 215 events (109/163 patients, 67%) in the placebo group ( P = 0.018). There was a lower incidence of each disease progression component in the inhaled treprostinil group: 6-minute-walk distance decline (45 vs. 64 events), lung disease exacerbation (48 vs. 72 events), FVC decline (19 vs. 33), cardiopulmonary hospitalization (23 vs. 33 events), and death (10 vs. 12). Fewer patients receiving inhaled treprostinil had multiple progression events compared with those receiving the placebo (35 vs. 58, 22% vs. 36%; P = 0.005). Conclusions: Patients who received inhaled treprostinil were significantly less likely to experience further disease progression events after an initial event compared with patients receiving placebo. These results support the continuation of inhaled treprostinil despite the occurrence of disease progression in clinical practice.
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- 2022
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28. Colonoscopy Following COVID-19 Delays in Procedures: Risk Stratification for Procedures Is Critical.
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Fritz CDL, Sayuk GS, Elwing JE, Wilgus NC, Dieckgraefe BK, and Presti ME
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- 2022
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29. Symptomatic Pneumoperitoneum After Gastrostomy Tube Placement Managed by Pneumocentesis.
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Brotherton T, Chhaparia A, Presti M, Sayuk G, and Elwing J
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Pneumoperitoneum is a known complication of percutaneous endoscopic gastrostomy tube placement that typically resolves spontaneously with conservative management. We describe the case of a 72-year-old man who developed abdominal pain and distention after percutaneous endoscopic gastrostomy tube placement who was subsequently found to have a moderate-sized pneumoperitoneum. Despite supportive care, his abdominal pain failed to improve. We report paracentesis with air aspiration as an intervention for benign pneumoperitoneum resulting in rapid and durable resolution of abdominal complaints., (© 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)
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- 2021
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30. Successful use of intravenous treprostinil as a bridge to pulmonary thromboendarterectomy.
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Cattran A and Elwing J
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- Chronic Disease, Endarterectomy, Epoprostenol analogs & derivatives, Humans, Hypertension, Pulmonary drug therapy, Pulmonary Embolism drug therapy, Pulmonary Embolism surgery
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Chronic thromboembolic pulmonary hypertension (CTEPH) involves non-resolving thromboemboli in the pulmonary arteries. Treatment for CTEPH includes lifelong anticoagulation and determination of patients who have disease which is operable versus inoperable. Pulmonary arterial hypertension (PAH) targeted therapies are oftentimes used as a bridge to pulmonary thromboendarterectomy (PTE), though riociguat is the only Food and Drug Administration (FDA)-approved therapy for CTEPH. There is a paucity of data regarding the efficacy of other PAH therapies, particularly as a bridge to PTE. Here, we present a case report of severe CTEPH related to ventriculoatrial shunt in which intravenous treprostinil was used as a bridge to PTE., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2021
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31. Prediction of Health-related Quality of Life and Hospitalization in Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry.
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Min J, Badesch D, Chakinala M, Elwing J, Frantz R, Horn E, Klinger J, Lammi M, Mazimba S, Sager J, Shlobin O, Simon M, Thenappan T, Grinnan D, Ventetuolo C, and Al-Naamani N
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- Adult, Aged, Aged, 80 and over, Female, Forecasting, Hospitalization trends, Humans, Male, Middle Aged, Pulmonary Arterial Hypertension epidemiology, Risk Assessment trends, United States epidemiology, Hospitalization statistics & numerical data, Pulmonary Arterial Hypertension mortality, Pulmonary Arterial Hypertension physiopathology, Quality of Life psychology, Registries statistics & numerical data, Risk Assessment methods
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- 2021
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32. Raynaud Phenomenon in Antisynthetase Syndrome Treated With Epoprostenol.
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Vick E, Sharma D, and Elwing J
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- Epoprostenol, Humans, Myositis diagnosis, Myositis drug therapy, Raynaud Disease diagnosis, Raynaud Disease drug therapy
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Competing Interests: The authors have no financial disclosures. It should be noted that this represents an off-label use of epoprostenol. Currently, it is only Food and Drug Administration approved in the United States for the treatment of pulmonary artery hypertension, and not as a treatment for Raynaud phenomenon. The authors have no conflicts of interest related to this topic.
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- 2021
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33. Genetic determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis.
- Author
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Rhodes CJ, Batai K, Bleda M, Haimel M, Southgate L, Germain M, Pauciulo MW, Hadinnapola C, Aman J, Girerd B, Arora A, Knight J, Hanscombe KB, Karnes JH, Kaakinen M, Gall H, Ulrich A, Harbaum L, Cebola I, Ferrer J, Lutz K, Swietlik EM, Ahmad F, Amouyel P, Archer SL, Argula R, Austin ED, Badesch D, Bakshi S, Barnett C, Benza R, Bhatt N, Bogaard HJ, Burger CD, Chakinala M, Church C, Coghlan JG, Condliffe R, Corris PA, Danesino C, Debette S, Elliott CG, Elwing J, Eyries M, Fortin T, Franke A, Frantz RP, Frost A, Garcia JGN, Ghio S, Ghofrani HA, Gibbs JSR, Harley J, He H, Hill NS, Hirsch R, Houweling AC, Howard LS, Ivy D, Kiely DG, Klinger J, Kovacs G, Lahm T, Laudes M, Machado RD, MacKenzie Ross RV, Marsolo K, Martin LJ, Moledina S, Montani D, Nathan SD, Newnham M, Olschewski A, Olschewski H, Oudiz RJ, Ouwehand WH, Peacock AJ, Pepke-Zaba J, Rehman Z, Robbins I, Roden DM, Rosenzweig EB, Saydain G, Scelsi L, Schilz R, Seeger W, Shaffer CM, Simms RW, Simon M, Sitbon O, Suntharalingam J, Tang H, Tchourbanov AY, Thenappan T, Torres F, Toshner MR, Treacy CM, Vonk Noordegraaf A, Waisfisz Q, Walsworth AK, Walter RE, Wharton J, White RJ, Wilt J, Wort SJ, Yung D, Lawrie A, Humbert M, Soubrier F, Trégouët DA, Prokopenko I, Kittles R, Gräf S, Nichols WC, Trembath RC, Desai AA, Morrell NW, and Wilkins MR
- Subjects
- Female, Genetic Predisposition to Disease, Genetic Variation, Genome-Wide Association Study, Genotyping Techniques methods, Humans, Male, Middle Aged, Polymorphism, Single Nucleotide, Risk Assessment, Signal Transduction genetics, Survival Analysis, HLA-DP alpha-Chains genetics, HLA-DP beta-Chains genetics, Pulmonary Arterial Hypertension diagnosis, Pulmonary Arterial Hypertension genetics, Pulmonary Arterial Hypertension metabolism, Pulmonary Arterial Hypertension mortality, SOXF Transcription Factors genetics
- Abstract
Background: Rare genetic variants cause pulmonary arterial hypertension, but the contribution of common genetic variation to disease risk and natural history is poorly characterised. We tested for genome-wide association for pulmonary arterial hypertension in large international cohorts and assessed the contribution of associated regions to outcomes., Methods: We did two separate genome-wide association studies (GWAS) and a meta-analysis of pulmonary arterial hypertension. These GWAS used data from four international case-control studies across 11 744 individuals with European ancestry (including 2085 patients). One GWAS used genotypes from 5895 whole-genome sequences and the other GWAS used genotyping array data from an additional 5849 individuals. Cross-validation of loci reaching genome-wide significance was sought by meta-analysis. Conditional analysis corrected for the most significant variants at each locus was used to resolve signals for multiple associations. We functionally annotated associated variants and tested associations with duration of survival. All-cause mortality was the primary endpoint in survival analyses., Findings: A locus near SOX17 (rs10103692, odds ratio 1·80 [95% CI 1·55-2·08], p=5·13 × 10
-15 ) and a second locus in HLA-DPA1 and HLA-DPB1 (collectively referred to as HLA-DPA1/DPB1 here; rs2856830, 1·56 [1·42-1·71], p=7·65 × 10-20 ) within the class II MHC region were associated with pulmonary arterial hypertension. The SOX17 locus had two independent signals associated with pulmonary arterial hypertension (rs13266183, 1·36 [1·25-1·48], p=1·69 × 10-12 ; and rs10103692). Functional and epigenomic data indicate that the risk variants near SOX17 alter gene regulation via an enhancer active in endothelial cells. Pulmonary arterial hypertension risk variants determined haplotype-specific enhancer activity, and CRISPR-mediated inhibition of the enhancer reduced SOX17 expression. The HLA-DPA1/DPB1 rs2856830 genotype was strongly associated with survival. Median survival from diagnosis in patients with pulmonary arterial hypertension with the C/C homozygous genotype was double (13·50 years [95% CI 12·07 to >13·50]) that of those with the T/T genotype (6·97 years [6·02-8·05]), despite similar baseline disease severity., Interpretation: This is the first study to report that common genetic variation at loci in an enhancer near SOX17 and in HLA-DPA1/DPB1 is associated with pulmonary arterial hypertension. Impairment of SOX17 function might be more common in pulmonary arterial hypertension than suggested by rare mutations in SOX17. Further studies are needed to confirm the association between HLA typing or rs2856830 genotyping and survival, and to determine whether HLA typing or rs2856830 genotyping improves risk stratification in clinical practice or trials., Funding: UK NIHR, BHF, UK MRC, Dinosaur Trust, NIH/NHLBI, ERS, EMBO, Wellcome Trust, EU, AHA, ACClinPharm, Netherlands CVRI, Dutch Heart Foundation, Dutch Federation of UMC, Netherlands OHRD and RNAS, German DFG, German BMBF, APH Paris, INSERM, Université Paris-Sud, and French ANR., (Copyright © 2019 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license. Published by Elsevier Ltd.. All rights reserved.)- Published
- 2019
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34. Pulse dose steroids in severe pulmonary arterial hypertension secondary to systemic lupus erythematosus.
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Lee C and Elwing J
- Abstract
Objective: The pulmonary vascular targeted treatment for systemic lupus erythematosus-associated pulmonary arterial hypertension is similar to other connective tissue disease-associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject. Our objective is to highlight the importance of early immunosuppression in systemic lupus erythematosus-associated pulmonary arterial hypertension and the role of pulse dose steroids in management., Methods: This case describes a 23-year-old woman who presented with pulmonary arterial hypertension diagnosed by right heart catheterization with mean pulmonary artery pressure of 74 mmHg, pulmonary capillary wedge pressure of 12 mmHg, and a pulmonary vascular resistance of 1908 dyne s cm
-5 . Due to the aggressive nature of her disease, she declined despite management with epoprostenol and sildenafil. Because of coexisting systemic lupus erythematosus with hemolytic anemia and worsening pulmonary arterial hypertension, intensive immunosuppressive therapy with pulse dose steroids was initiated., Results: Shortly after initiation of pulse dose steroids and maintenance immunosuppression, she had a dramatic symptomatic and hemodynamic response with a decrease in her pulmonary vascular resistance from 1908 to 136 dyne sec cm-5 and improvement in her mean pulmonary artery pressure from 74 to 27 mmHg on repeat right heart catheterization., Conclusion: Early immunosuppression is important to consider in those with systemic lupus erythematosus-associated pulmonary arterial hypertension. Limited studies are available, but most have focused on the use of cyclophosphamide. Pulse dose steroids may be a potentially less toxic but equally effective manner to aid in the treatment of systemic lupus erythematosus-pulmonary arterial hypertension when intensive immunosuppression is being considered., Competing Interests: Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.- Published
- 2017
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35. Predictors of Poor Adherence of US Gastroenterologists with Colonoscopy Screening and Surveillance Guidelines.
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Iskandar H, Yan Y, Elwing J, Early D, Colditz GA, and Wang JS
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- Gastroenterology statistics & numerical data, Humans, Population Surveillance, Colonoscopy standards, Gastroenterology standards, Guideline Adherence statistics & numerical data
- Abstract
Background: The US Multi-Society Task Force on Colorectal Cancer published guidelines for colonoscopy screening and surveillance in 2008 and affirmed them in 2012. Characteristics associated with guideline adherence among US gastroenterologists have not been assessed., Aim: Assess awareness and adherence of US gastroenterologists with national guidelines for colonoscopy screening and surveillance and predictors of adherence to guidelines., Methods: A Web-based survey was administered to gastroenterologists in various practice settings across the USA., Results: A total of 306 gastroenterologists completed the survey; 86 % reported awareness of the guidelines. Low-volume colonoscopists (<20/month) were less likely to be aware of the guidelines (OR 0.26, p = 0.03) compared to high-volume colonoscopists (>100/month). Those completing training before 1990 were less likely to report following guidelines (OR 0.37, p = 0.01). Adherence with guidelines was then assessed via clinical scenarios. Compared to physicians finishing training in 1991-2010, less adherence was seen in those finishing before 1990 (OR 0.75, p < 0.001) or currently in training (OR 0.72, p = 0.004). Compared to the Western USA, less adherence was seen in the Midwest (OR 0.69, p = 0.001), Northeast (OR 0.63, p < 0.001), and South (OR 0.59, p < 0.001). Lower adherence was seen among non-academic physicians (OR 0.72, p = 0.001) and low-volume colonoscopists (OR 0.52, p < 0.001)., Conclusions: There is poor adherence with colonoscopy screening and surveillance guidelines among US gastroenterologists. Poor adherence was associated with being in training or finishing training before 1990, practicing in the South, non-academic settings, and low colonoscopy volume. These findings can target interventions for quality improvement in colorectal cancer screening and surveillance.
- Published
- 2015
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36. Worsening gradient of aortic stenosis with treatment of pulmonary arterial hypertension in scleroderma.
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Pietras DA, Lopez FR, Peréz R, López-Candales A, and Elwing J
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- Aortic Valve Stenosis physiopathology, Echocardiography, Follow-Up Studies, Humans, Hypertension, Pulmonary drug therapy, Male, Middle Aged, Vasodilator Agents adverse effects, Ventricular Function, Right physiology, Aortic Valve Stenosis etiology, Hypertension, Pulmonary etiology, Scleroderma, Systemic complications, Vasodilator Agents therapeutic use
- Abstract
Systemic sclerosis (SSc) can cause interstitial lung and pulmonary vascular disease that can induce pulmonary arterial hypertension (PAH). It is well known that severe PAH may reduce left ventricluar preload and decrease diastolic filling with the potential of reducing forward flow. We present a case in which a patient with SSc and symptomatic PAH required direct pulmonary vasodilator therapy for treatment of elevated pulmonary pressures. On follow-up echocardiogram, while improvement in right ventricular function and reduction in estimated pulmonary pressures were noted; worsening of aortic valve gradients was also found. Cardiac hemodynamics of pulmonary vasodilator therapy is discussed and the literature is reviewed.
- Published
- 2015
37. Objective measures of right ventricular function during exercise: results of a pilot study.
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Anjak A, López-Candales A, Lopez FR, Harris D, and Elwing J
- Subjects
- Adult, Age Factors, Analysis of Variance, Anthropometry, Body Mass Index, Case-Control Studies, Confidence Intervals, Echocardiography methods, Female, Humans, Male, Middle Aged, Observer Variation, Pilot Projects, Retrospective Studies, Stroke Volume physiology, Echocardiography, Stress methods, Exercise Test, Exercise Tolerance physiology, Ventricular Function, Right physiology
- Abstract
Background: A growing body of evidence seems to suggest that reduction in right ventricular (RV) systolic function is not only associated with both development of symptoms associated with pulmonary arterial hypertension (PAH) but also increased morbidity and adverse clinical outcomes. Recent data suggest that supine bicycle stress echocardiography (sBEE) is feasible and provides realistic resistance and compliance estimations., Methods: The study design was a retrospective analysis of sBEE obtained for clinical indications of dyspnea on exertion or unexplained exercise intolerance. A total of 30 sBEE studies were included. The first 10 sBEE studies served as a calibration cohort not only to establish objective measures of RV performance but also to determine feasibility and reproducibility. The following 20 sBEE served as a validation cohort., Results: Our results demonstrate that tricuspid annular plane systolic excursion and tricuspid annular systolic velocity, even when measured by an untrained reader, are reliable and accurate measures of the RV response to exercise during a sBEE protocol. Furthermore, measurements of RV myocardial performance (Tei index) or peak RV strain during exercise not only are time consuming but also simply unreliable and should not be utilized to evaluate RV function during sBEE., Conclusion: The results of our pilot study suggest that sBEE might be promising for detecting RV abnormalities during exercise. A larger prospective study is now needed to determine if TAPSE and tricuspid annular systolic velocity recorded during sBEE might be useful in assessing patients presenting with dyspnea during exercise or suspected of having PAH., (© 2013, Wiley Periodicals, Inc.)
- Published
- 2014
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38. Right ventricular ejection efficiency: a new echocardiographic measure of mechanical performance in chronic pulmonary hypertension.
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López-Candales A, Lopez FR, Trivedi S, and Elwing J
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- Adult, Aged, Aged, 80 and over, Chronic Disease, Cohort Studies, Female, Humans, Hypertension, Pulmonary physiopathology, Logistic Models, Male, Middle Aged, Multivariate Analysis, Observer Variation, Prospective Studies, Severity of Illness Index, Vascular Resistance physiology, Ventricular Dysfunction, Right diagnostic imaging, Ventricular Dysfunction, Right physiopathology, Young Adult, Echocardiography methods, Hypertension, Pulmonary diagnostic imaging, Stroke Volume, Ventricular Function, Right physiology
- Abstract
Background: The severity of pulmonary vascular resistance (PVR) is known to be a critical determinant of right ventricular (RV) systolic function; this relationship remains poorly characterized. We therefore, designed a study to examine the relationship that exists between echocardiographically measured PVR and maximal tricuspid annular plane systolic excursion (TAPSE) to gain some insight regarding RV ejection efficiency (RVEe) in patients with chronic pulmonary hypertension (cPH)., Methods: Standard echocardiographic measures of RV size and systolic performance were recorded from 95 patients (age 54 ± 15 years and pulmonary artery systolic pressures [PASP] that range from 20 to 125 mmHg). For this study, RVEe was defined as TAPSE/Echocardiographic PVR., Results: A strong negative correlation (R(2) = -0.51, P < 0.001) was seen between TAPSE and PASP; however, a power curve trend line fit the relationship between RVEe and PASP (R(2) = 0.77; P < 0.01). In a multiple regression analysis, abnormal pulmonary pressures were better identified when RVEe (P < 0.0001) was used., Conclusions: Based on these results, it appears that measurement of RVEe might be extremely useful for the assessment of RV mechanics and plasticity. The power curve relationship clearly demonstrates that minimal changes in PASP (up to 50 mmHg) result in dramatic reductions in RVEe. A steady decline in RVEe, though at a lower rate, continues to occur with increasing PASP. Additional studies are required using RVEe into a functional RV imaging algorithm and determine if RVEe correlates with development of symptoms, response to therapy and overall clinical outcomes., (© 2013, Wiley Periodicals, Inc.)
- Published
- 2014
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39. Pulmonary hypertension associated with COPD.
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Elwing J and Panos RJ
- Subjects
- Humans, Hypertension, Pulmonary therapy, Pulmonary Disease, Chronic Obstructive pathology, Pulmonary Disease, Chronic Obstructive therapy, Hypertension, Pulmonary diagnosis, Hypertension, Pulmonary etiology, Pulmonary Disease, Chronic Obstructive complications
- Abstract
Although the prevalence of pulmonary hypertension (PH) in individuals with chronic obstructive pulmonary disease (COPD) is not known precisely, approximately 10%-30% of patients with moderate to severe COPD have elevated pulmonary pressures. The vast majority of PH associated with COPD is mild to moderate and severe PH occurs in < 5% of patients. When COPD is associated with PH, both mortality and morbidity are increased. There are no clinical or physical examination findings that accurately identify patients with underlying PH. Radiographic imaging findings are specific but not sensitive indicators of PH. Echocardiography is the principle noninvasive diagnostic test but may be technically limited in a significant proportion of patients with COPD. Right heart catheterization is required for accurate measurement of pulmonary pressures. The combined effects of inflammation, endothelial cell dysfunction, and angiogenesis appear to contribute to the development of PH associated with COPD. Systemic vasodilators have not been found to be effective therapy. Selective pulmonary vasodilators including inhaled nitric oxide and phosphodiesterase inhibitors are promising treatments for patients with COPD associated PH but further evaluation of these medications is needed prior to their routine use.
- Published
- 2008
- Full Text
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