PAH Care Teams, Unmet Needs, and the Four Treatment Pathways

This episode, "PAH Care Teams, Unmet Needs, and the Four Treatment Pathways," features the panel examining who belongs on the care team and what the treatment toolbox now contains.

Dr. McLaughlin asks how a multidisciplinary team supports patients with PAH. Dr. Kim explains that pulmonary hypertension does not sit within one subspecialty, so cardiologists and pulmonologists must work together to sort out the cause of shortness of breath. PAH is rare and seldom the first diagnosis considered. Right heart catheterization is required, rheumatologists refer many patients with connective tissue disease, and primary care providers remain essential because patients are living longer and still need general health maintenance.

Dr. McLaughlin adds that no PAH center is complete without nurses, nurse practitioners, and research coordinators, and she says she could not do her job if her nurses left tomorrow. Dr. Channick likens the field to six degrees of Kevin Bacon, since almost any specialty connects to pulmonary hypertension, including hepatology, infectious disease, substance use, and amphetamine-associated disease. Transplant colleagues are sometimes needed as well.

Dr. McLaughlin then asks Dr. Channick about remaining gaps. He says progress breeds impatience, because clinicians can now see what is possible and no longer accept fatalism. The approach is aggressive, optimistic, and proactive, although some patients still do not respond well enough. Dr. McLaughlin recalls that early treatment meant anticoagulants and a box of tissues, whereas today more than a dozen FDA-approved therapies exist.

Dr. Vaidya outlines the four pathways. The nitric oxide pathway includes PDE5 inhibitors and the soluble guanylate cyclase stimulator, the endothelin pathway offers three receptor antagonists, and the prostacyclin pathway spans oral, inhaled, subcutaneous, and intravenous agents. The newest is activin signaling inhibition. The first three are vasodilators with additional antiproliferative effects, and all four work in complementary combinations.

In "Initial PAH Therapy: Upfront Dual Oral Combination and Guideline Risk Stratification," Dr. Channick and Dr. Kim review how guidelines and landmark trials support upfront dual therapy.