The DOI matches its Crossref record; 27 of the 27 references it cites were found in a registry or an index.
Sotatercept for the Treatment of Pulmonary Arterial Hypertension
Open accessAbstract
BACKGROUND: Pulmonary arterial hypertension is characterized by pulmonary vascular remodeling, cellular proliferation, and poor long-term outcomes. Dysfunctional bone morphogenetic protein pathway signaling is associated with both hereditary and idiopathic subtypes. Sotatercept, a novel fusion protein, binds activins and growth differentiation factors in the attempt to restore balance between growth-promoting and growth-inhibiting signaling pathways. METHODS: In this 24-week multicenter trial, we randomly assigned 106 adults who were receiving background therapy for pulmonary arterial hypertension to receive subcutaneous sotatercept at a dose of 0.3 mg per kilogram of body weight every 3 weeks or 0.7 mg per kilogram every 3 weeks or placebo. The primary end point was the change from baseline to week 24 in pulmonary vascular resistance. RESULTS: (95% CI, -329.3 to -149.7; P<0.001). At 24 weeks, the least-squares mean difference between the sotatercept 0.3-mg group and the placebo group in the change from baseline in 6-minute walk distance was 29.4 m (95% CI, 3.8 to 55.0). The least-squares mean difference between the sotatercept 0.7-mg group and the placebo group was 21.4 m (95% CI, -2.8 to 45.7). Sotatercept was also associated with a decrease in N-terminal pro-B-type natriuretic peptide levels. Thrombocytopenia and an increased hemoglobin level were the most common hematologic adverse events. One patient in the sotatercept 0.7-mg group died from cardiac arrest. CONCLUSIONS: Treatment with sotatercept resulted in a reduction in pulmonary vascular resistance in patients receiving background therapy for pulmonary arterial hypertension. (Funded by Acceleron Pharma; PULSAR ClinicalTrials.gov number, NCT03496207.).
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Cites
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
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Self-citationValidating the SF-36 health survey questionnaire: new outcome measure for primary care.
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Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives
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Five-Year Outcomes of Patients Enrolled in the REVEAL Registry
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Self-citationGenetics and genomics of pulmonary arterial hypertension
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Cited by
Volkmann, E. R., Andréasson, K., & Smith, V. (2023) · The Lancet · doi:10.1016/s0140-6736(22)01692-0 · Cited by 616
Houston, B. A., Brittain, E. L., & Tedford, R. J. (2023) · New England Journal of Medicine · doi:10.1056/nejmra2207410 · Cited by 280
Insights into bone morphogenetic proteins in cardiovascular diseases
Ye, D., Liu, Y., Pan, H., Feng, Y., Lu, X., Gan, L., Wan, J., & Ye, J. (2023) · Frontiers in Pharmacology · doi:10.3389/fphar.2023.1125642 · Cited by 37
Goebel, E. J., Aykul, S., Hom, W. W., Saotome, K., Economides, A. N., Franklin, M. C., & Idone, V. J. (2025) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2502788122 · Cited by 2
Metadata
- Authors
- Humbert, M., McLaughlin, V., Gibbs, J. S. R., Gomberg-Maitland, M., Hoeper, M. M., Preston, I. R., Souza, R., Waxman, A., Escribano Subias, P., Feldman, J., Meyer, G., Montani, D., Olsson, K. M., Manimaran, S., Barnes, J., Linde, P. G., de Oliveira Pena, J., & Badesch, D. B.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2021-04
- Volume
- 384
- Issue
- 13
- Pages
- 1204-1215
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W3152050105
- Cited by
- 608
- References
- 27
- FWCI
- 50.21
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Humbert, M., McLaughlin, V., Gibbs, J. S. R., Gomberg-Maitland, M., Hoeper, M. M., Preston, I. R., Souza, R., Waxman, A., Escribano Subias, P., Feldman, J., Meyer, G., Montani, D., Olsson, K. M., Manimaran, S., Barnes, J., Linde, P. G., de Oliveira Pena, J., & Badesch, D. B. (2021). Sotatercept for the Treatment of Pulmonary Arterial Hypertension. New England Journal of Medicine. https://doi.org/10.1056/nejmoa2024277
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
