ArticlePulmonary circulation
Identifying "super responders" in pulmonary arterial hypertension.
Article in Pulmonary circulation. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Heterogeneity of treatment effects by risk in pulmonary arterial hypertension.The European respiratory journal · 2023Pooled it
- Variation inCirculation research · 2026Article
- Acute pulmonary vasoreactivity: a simple test revisited in the contemporary era - a narrative review.International journal of cardiology. Heart & vasculature · 2026Review
- Shifting Paradigms in the Management of Pulmonary Hypertension.European cardiology · 2024Review
- Lung Transplantation for Pulmonary Arterial Hypertension: Optimized Referral and Listing Based on an Evolving Disease Concept.Journal of cardiovascular development and disease · 2023Review
- Evolution and optimization of clinical trial endpoints and design in pulmonary arterial hypertension.Pulmonary circulation · 2023Review
- Circulating markers of inflammation and angiogenesis and clinical outcomes across subtypes of pulmonary arterial hypertension.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2023Observational
- Pharmacogenomics in the Management of Pulmonary Arterial Hypertension: Current Perspectives.Pharmacogenomics and personalized medicine · 2023Review
- A Semi-Automated Term Harmonization Pipeline Applied to Pulmonary Arterial Hypertension Clinical Trials.Methods of information in medicine · 2022Article
- Article
- Pulmonary Vasculature Responsiveness to Phosphodiesterase-5A Inhibition in Heart Failure With Reduced Ejection Fraction: Possible Role of Plasma Potassium.Frontiers in cardiovascular medicine · 2022Article
- Advances in the management of pulmonary arterial hypertension.Journal of investigative medicine : the official publication of the American Federation for Clinical Research · 2021Review
- Using Omics to Understand and Treat Pulmonary Vascular Disease.Frontiers in medicine · 2018Review
- A pilot study of oral treprostinil pharmacogenomics and treatment persistence in patients with pulmonary arterial hypertension.Therapeutic advances in respiratory diseaseArticle
- Role of biomarkers in evaluation, treatment and clinical studies of pulmonary arterial hypertension.Pulmonary circulationArticle
- Apoptosis signal-regulating kinase 1 inhibition inPulmonary circulationArticle
- Clinical and genetic associations with prostacyclin response in pulmonary arterial hypertension.Pulmonary circulationArticle
Corrections and comments
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Authors and funding
2 authors.
Funding
Abstract
Pharmacotherapeutic options for pulmonary arterial hypertension (PAH) have increased dramatically in the last two decades and along with this have been substantial improvements in survival. Despite these advances, however, PAH remains a progressive and ultimately fatal disease for most patients and only epoprostenol has been shown to improve survival in a randomized control trial. Clinical observations of the heterogeneity of treatment response to different classes of medications across the phenotypically diverse PAH population has led to the identification of patients who derive significantly more benefit from certain medications than the population mean, the so-called "super responders." This was first recognized among PAH patients with acute vasodilator response during invasive hemodynamic testing, a subset of whom have dramatically improved survival when treated with calcium channel blocker (CCB) therapy. Retrospective studies have now suggested a sex discrepancy in response to endothelin receptor antagonists (ERA) and phosphodiesterase inhibitors, and more recently a few studies have found genomic associations with response to CCBs and ERAs. With increasing availability of "omics" technologies, recognition of these "super responders," combined with careful clinical and molecular phenotyping, will lead to advances in pharmacogenomics, precision medicine, and continued improvements in survival among PAH patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.