ArticlePulmonary circulation2015
Use of pulmonary arterial hypertension-approved therapy in the treatment of non-group 1 pulmonary hypertension at US referral centers.
Article in Pulmonary circulation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 2 of them syntheses that pooled it.
What it found
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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
29 citing papers in PubMed, 2 syntheses or guidelines pooled it, 47 citations in OpenAlex.
- Effects of pulmonary arterial hypertension therapies on arterial oxygenation in patients with pulmonary hypertension associated with lung disease: a systematic review and meta-analysis.BMJ open respiratory research · 2025Pooled it
- Pulmonary hypertension-targeted therapies in heart failure: A systematic review and meta-analysis.PloS one · 2018Pooled it
- Connective tissue disease-related interstitial lung disease and pulmonary hypertension.Breathe (Sheffield, England) · 2025Review
- Shining a spotlight on pulmonary hypertension associated with interstitial lung disease care: The latest advances in diagnosis and treatment.Journal of managed care & specialty pharmacy · 2025Review
- Evaluation of patients with severe pulmonary hypertension and a range of comorbidities prescribed inhaled treprostinil.JHLT open · 2024Article
- Cluster analysis identifies novel real-world lung disease-pulmonary hypertension subphenotypes: implications for treatment response.ERJ open research · 2024Article
- Pulmonary hypertension secondary to valvular heart disease: a state-of-the-art review.Heart failure reviews · 2024Review
- Using PAH-SYMPACT to assess quality of life in patients with pulmonary hypertension associated with chronic lung disease.Pulmonary circulation · 2024Article
- Pulmonary hypertension associated mortality in the United States from 2003 to 2020: an observational analysis of time trends and disparities.Journal of thoracic disease · 2023Article
- Variable Monitoring of Veterans with Group 3 Pulmonary Hypertension Treated with Off-Label Pulmonary Vasodilator Therapy.Annals of the American Thoracic Society · 2022Article
- Real-world use of inhaled treprostinil for lung disease-pulmonary hypertension: A protocol for patient evaluation and prescribing.Pulmonary circulation · 2022Article
- Parenteral prostanoids for severe Group 3 pulmonary hypertension with right ventricular dysfunction.Journal of thoracic disease · 2021Article
- Prescription Patterns for Pulmonary Vasodilators in the Treatment of Pulmonary Hypertension Associated With Chronic Lung Diseases: Insights From a Clinician Survey.Frontiers in medicine · 2021Article
- Factors Associated With Potentially Inappropriate Phosphodiesterase-5 Inhibitor Use for Pulmonary Hypertension in the United States, 2006 to 2015.Circulation. Cardiovascular quality and outcomes · 2020Article
- Histological hallmarks and role of Slug/PIP axis in pulmonary hypertension secondary to pulmonary fibrosis.EMBO molecular medicine · 2019Article
- Inflammasomes: a novel therapeutic target in pulmonary hypertension?British journal of pharmacology · 2019Review
- Survival in pulmonary hypertension due to chronic lung disease: Influence of low diffusion capacity of the lungs for carbon monoxide.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2019Article
- Study on the clinical efficacy of specific phosphodiesterase inhibitor in patients with pulmonary hypertension due to left heart disease.Experimental and therapeutic medicine · 2018Article
- Challenges in Pulmonary Hypertension: Controversies in Treating the Tip of the Iceberg. A Joint National Institutes of Health Clinical Center and Pulmonary Hypertension Association Symposium Report.American journal of respiratory and critical care medicine · 2018Article
- Healthcare burden of pulmonary hypertension owing to lung disease and/or hypoxia.BMC pulmonary medicine · 2017Observational
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
Pulmonary hypertension (PH) is a frequent complication of left heart disease and parenchymal lung disease, and it portends increased mortality. A growing number of medications are approved for the treatment of World Health Organization (WHO) group 1 pulmonary arterial hypertension (PAH). However, they are not well studied in PH of other etiologies (WHO groups 2-5). We sought to assess treatment approaches used by PAH referral centers in this diverse group of patients. We developed a semiquantitative online survey designed to evaluate the use of PAH-approved therapy by pulmonary vascular disease centers in the United States for management of non-group 1 PH. Thirty of 50 centers completed the survey. Almost all centers (93%) reported using PAH therapy for patients with non-group 1 PH, including 77% with group 2 PH and 80% with group 3 PH. Elevated transpulmonary gradient or pulmonary vascular resistance and the presence of right ventricular (RV) dysfunction were commonly cited as supporting use of PAH therapy in patients with PH secondary to left heart disease. For patients with PH and concomitant parenchymal lung disease, degree of pulmonary function impairment and RV dysfunction were most important in influencing use of PAH therapy. In conclusion, pulmonary vascular disease treatment centers use PAH-approved therapy for patients with WHO group 2-5 PH, mostly relying on hemodynamics and assessment of RV function to identify candidates for therapy. Clinical trials designed to test the efficacy of PAH therapy in PH due to left heart and lung disease are needed, as clinical practice has extended beyond the evidence for these etiologies of PH.
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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.