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Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension.

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Date
2019-03-11
Authors
Parikh, Kishan S
Dahhan, Talal
Nicholl, Leigh
Ruopp, Nicole
Pomann, Gina-Maria
Fortin, Terry
Tapson, Victor F
Rajagopal, Sudarshan
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Abstract
The presence of pulmonary hypertension (PH) significantly worsens outcomes in patients with advanced sarcoidosis, but its optimal management is unknown. We aimed to characterize a large sarcoidosis-associated pulmonary hypertension (SAPH) cohort to better understand patient characteristics, clinical outcomes, and management strategies including treatment with PH therapies. Patients at Duke University Medical Center with biopsy-proven sarcoidosis and SAPH confirmed by right heart catheterization (RHC) were identified from 1990-2010. Subjects were followed for up to 11 years and assessed for differences by treatment strategy for their SAPH, including those who were not treated with PH-specific therapies. Our primary outcomes of interest were change in 6-minute walk distance (6MWD) and change in N-terminal pro-brain natriuretic peptide (NT-proBNP) by after therapy. We included 95 patients (76% women, 86% African American) with SAPH. Overall, 70% of patients had stage IV pulmonary sarcoidosis, and 77% had functional class III/IV symptoms. Median NT-proBNP value was elevated (910 pg/mL), and right ventricular dysfunction was moderate/severe in 55% of patients. Median values for mean pulmonary artery pressure (49 mmHg) and pulmonary vascular resistance (8.5 Woods units) were consistent with severe pulmonary hypertension. The mortality rate over median 3-year follow-up was 32%. Those who experienced a clinical event and those who did not had similar overall echocardiographic findings, hemodynamics, 6MWD and NT-proBNP at baseline, and unadjusted analysis showed that only follow-up NT-proBNP was associated with all-cause hospitalization or mortality. A sign test to evaluate the difference between NT-Pro-BNP before and after PH therapy produced evidence that a significant difference existed between the median pre- and post-NT-Pro-BNP (-387.0 (IQR: -1373.0-109), p = 0.0495). Use of PH-specific therapy may be helpful in selected patients with SAPH and pre-capillary pulmonary vascular disease. Prospective trials are needed to characterize responses to PH-specific therapy in this subset of patients with SAPH.
Type
Journal article
Subject
Science & Technology
Multidisciplinary Sciences
Science & Technology - Other Topics
SURVIVAL
THERAPY
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https://hdl.handle.net/10161/18198
Published Version (Please cite this version)
10.1038/s41598-019-40030-w
Publication Info
Parikh, Kishan S; Dahhan, Talal; Nicholl, Leigh; Ruopp, Nicole; Pomann, Gina-Maria; Fortin, Terry; ... Rajagopal, Sudarshan (2019). Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension. Scientific reports, 9(1). pp. 4061. 10.1038/s41598-019-40030-w. Retrieved from https://hdl.handle.net/10161/18198.
This is constructed from limited available data and may be imprecise. To cite this article, please review & use the official citation provided by the journal.
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Scholars@Duke

Dahhan

Talal I Dahhan

Assistant Professor of Medicine
An Internist, Pulmonologist and Critical Care Medicine physician with great interest in diagnosis and management of pulmonary vascular disease, as well as innovations in critical care curricular designs and graduate medical education.
Fortin

Terry Ann Fortin

Associate Professor of Medicine
Parikh

Kishan S Parikh

Assistant Professor of Medicine
Duke University Medical CenterDuke Clinical Research Institute
Pomann

Gina-Maria Pomann

Assistant Professor of Biostatistics & Bioinformatics
My primary research focuses on methods related to the development of biostatistics, bioinformatics, and data science units within academic health centers. An essential element of my work is to improve and diversify the workforce of collaborative biostatisticians and data scientists. I develop training programs, operational processes, and organizational infrastructure that foster efficient and effective collaborations between clinical and translational scientists and quantitative scientists.<b
Rajagopal

Sudarshan Rajagopal

Associate Professor of Medicine
I am a physician-scientist with a research focus on G protein-coupled receptor signaling in inflammation and vascular disease and a clinical focus on pulmonary vascular disease, as I serve as Co-Director of the Duke Pulmonary Vascular Disease Center. My research spans the spectrum from clinical research in pulmonary vascular disease, to translational research in cardiovascular disease, to the basic science of receptor signaling.  Our basic science resesarch focuses on understandin
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