Nick H. Kim, MD

PAH rarely belongs to a single specialty. The panel describes the team-based model behind effective care and reviews how four complementary treatment pathways now give clinicians a broad toolbox.

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.

"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.

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Intermediate- and high-risk status signals a struggling right ventricle and a prognosis the panel considers unacceptable. They argue for treating risk assessment as a routine vital sign while weighing what patients say they want from therapy.

PAH Risk Assessment: Why Reaching Low Risk Matters for Patients

Welcome back to another HCPLive Peer Exchange series. In "PAH Risk Assessment: Why Reaching Low Risk Matters for Patients," moderator Vallerie McLaughlin, MD is joined by Richard Channick, MD, Anjali Vaidya, MD, FACC, FASE, FACP, and Nick H. Kim, MD to open the series on pulmonary arterial hypertension.

Dr. McLaughlin outlines a program that spans guideline-based risk stratification, combination therapy, route of administration, escalation and switching, and newer therapies, then asks Dr. Kim what the treatment burden looks like for patients who have not reached low-risk status. Dr. Kim notes that many patients are diagnosed late and remain symptomatic even on treatment.

He explains that risk assessment combines clinical and laboratory data to gauge the health of the right ventricle, so an intermediate or high-risk label means the right ventricle is still struggling. In his view that status is not acceptable, and the goal is to bring every patient to low risk. He compares risk assessment to a new vital sign, closest to a hemoglobin A1c in this disease, and cites registry data showing hospitalization and mortality above 20% per year in high-risk patients.

Dr. Vaidya adds that patients rarely ask about their risk category and instead say they want to feel better, spend time with family, attend events, and stay out of the hospital. She argues that quality of life and functional capacity matter as much as formal calculations, although validated questionnaires are hard to complete at every visit.

Dr. Channick cautions that risk calculators work best at the population level, because each patient has different variables and needs an individualized assessment. His central message is that patients must be reassessed by whatever method the clinician prefers, and he calls patients who never get reassessed the bigger problem. As treatment options expand, frequent and regular follow-up becomes even more important.

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

 turns to the multidisciplinary team and Dr. Vaidya's overview of the four treatment pathways.

Nick H. Kim, MD

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