Overview
The ABCs of CTD-PAH: (A)lgorithms, Team - (B)ased Approach, and (C)ombination Therapy
CTD-PAH Expert Interview
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John Ryan, MD discusses screening protocols, diagnostic and treatment algorithms, clinical trial evidence, and the vital role of multidisciplinary teams for the management of patients with CTD-PAH in this four-chapter series
This activity is intended for pulmonologists, cardiopulmonologists, cardiologists, rheumatologists, and other AHPs treating patients with PAH
Pulmonary arterial hypertension (PAH) is a progressive disorder of the small pulmonary arteries characterized by endothelial dysfunction and vascular remodeling that results in increased pulmonary vascular resistance and pulmonary pressures, and is a highly challenging condition to both diagnose and manage. Connective tissue disease-associated PAH (CTD-PAH) is the second most common cause of PAH, accounting for approximately 25% of cases, and is associated with worse outcomes than idiopathic PAH.
Given the impact on patient outcomes, screening of PAH in CTD patients, including those with systemic sclerosis (scleroderma), systemic lupus erythematosus, and mixed CTD, is of critical importance. However, even with appropriate screening to identify suspected cases of CTD-PAH, the diagnosis of PAH in these patients is challenging due to the complex differential diagnosis. In addition, treatment selection is complex due to comorbidities, relatively little clinical trial data in this patient population, and evidence suggesting attenuated responses to PAH therapies in CTD-PAH patients.
Therefore, clinicians require education on the detection, diagnosis, and treatment of CTD-PAH, taking into account the particular clinical nuances relevant to this patient population. Clinicians must be prepared to adapt their practices to include better screening practices, selection of appropriate treatments, and multidisciplinary collaboration to ensure effective management of CTD-PAH patients to improve outcomes.
Upon completion of this activity, participants will be able to:
• Discuss the impact that various screening protocols have on CTD-PAH patient outcomes.
• Outline steps involved in the differential diagnosis of CTD-PAH
• Formulate comprehensive management plans for patients with CTD-PAH
• Discuss the impact that various screening protocols have on CTD-PAH patient outcomes.
• Outline steps involved in the differential diagnosis of CTD-PAH
• Formulate comprehensive management plans for patients with CTD-PAH

Supported by an educational grant from Actelion Pharmaceuticals US, Inc., a Janssen Pharmaceutical Company of Johnson & Johnson.
John Ryan, MD, MB, BCh, BAO, MRCPI, FACC, FAHA
Director, Pulmonary Hypertension Comprehensive Care Center
Medical Director, Cardiovascular Medical Unit
University of Utah Hospital
Faculty Director of Cardiology Education
University of Utah School of Medicine
Salt Lake City, UT
Director, Pulmonary Hypertension Comprehensive Care Center
Medical Director, Cardiovascular Medical Unit
University of Utah Hospital
Faculty Director of Cardiology Education
University of Utah School of Medicine
Salt Lake City, UT
The Academy for Continued Healthcare Learning (ACHL) requires that the faculty participating in an accredited continuing education activity disclose all affiliations or other financial relationships (1) with the manufacturers of any commercial product(s) and/or provider(s) of commercial services discussed in an educational presentation and (2) with any commercial supporters of the activity. All conflicts of interest have been mitigated prior to this activity.
The following financial relationships have been provided:
John Ryan, MD, MB, BCh, BAO, MRCPI, FACC, FAHA
Advisory Board: Bayer and Janssen PH
Consultant: Vertex
Speakers Bureau: Bayer, Janssen PH, United Therapeutics
Discussion of Off-Label, Investigational, or Experimental Drug/Device Use: sotatercept and GB002 (seralutinib)
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This activity will take approximately 75 minutes to complete. To receive credit, participants are required to complete the pretest, view all four online chapters and complete the posttest and evaluation. To receive credit, 75% must be achieved on the posttest. A certificate will be immediately available. There is no fee to participate in the activity or for the generation of the certificate.
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