PAH, CTEPH Diagnosis and Treatment Updates for Primary Care

In this Practical Updates in Primary Care 2023 Virtual Series session, two leading experts review the latest diagnosis strategies and guideline-recommended treatment options for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in the primary care setting.

Martha Kingman, FNP-C, DNP, who is a nurse practitioner at the University of Texas Southwestern Medical Center, and Bradley A. Maron, MD, who is a physician at the University of Maryland School of Medicine begin their session with an overview of characteristics of both PAH and CTEPH, including the WHO classification groups, functional classifications, and associated subcategories.

The 2022 European Society of Cardiology and European Respiratory Society guidelines for the treatment of PAH note that the general practitioner is the first step for most patients with unexplained exertional dyspnea. However, Martha Kingman notes that for any patient who is suspected to have pulmonary hypertension or CTEPH, general practitioners should refer the patient to a pulmonary hypertension clinic.

Dr Maron examines the three pathways that are the target of current therapeutic agents for PAH—the endothelin pathway, the NO-sGC-CGMP pathway, and the prostacyclin pathway—and discusses appropriate use for each class of medications (Figure 1).

Figure 1.

He further discusses diagnosis strategies to guide appropriate treatment selection in clinical practice, such as the use of a vasoreactivity test in individuals with PAH who are treatment naïve to determine whether they are candidates for calcium channel blockers.

Regarding CTEPH (Figure 2), Dr Maron noted that it’s generally considered a surgical disease. The treatment algorithm is built on the foundation of anticoagulation.

Figure 2. 

“The treatment algorithm for CTEPH always begins with commitment to lifelong anticoagulation,” Dr Maron said.

To conclude the session, Dr Maron and Martha Kingman emphasized the role of the primary care provider and the need for collaborative care with pulmonary hypertension care centers.

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For more information about PUPC 2023 Virtual Series and to register for upcoming sessions, visit https://practicalupdates.consultant360.com.

Reference:

Kingman M, Maron BA. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in primary care: updated diagnostic strategies and treatment options. Talk presented at: Practical Updates in Primary Care 2023 Virtual Series; July 19-21, 2023; Virtual.