Escalating PAH Therapy: Setting Expectations and Choosing the Next Step

In "Escalating PAH Therapy: Setting Expectations and Choosing the Next Step," the discussion moves to the conversation about adding or changing therapy.

Dr. McLaughlin asks Dr. Channick how he combines the risk score, echo, and hemodynamics when reassessing a patient after initial treatment. He generally leans toward aggressiveness, even in patients who are doing well, because anyone other than low risk can worsen even with a normal echo and the goal is to prevent that. The harder conversation comes when a patient feels fine, is working, and asks why another medication is needed, so he frames the goal as sitting across from the patient in five and ten years. When the patient is not doing well, the discussion is easier because the question becomes which drug to add or switch.

Dr. Vaidya agrees that success with add-on therapy begins at the initial diagnostic encounter, and she recommends spending time up front explaining the journey and the goals ahead. Medications work, so patients feel better on combination therapy, and at three or four months it is difficult to celebrate that improvement while also saying they need more if that expectation was never set.

She reminds herself that risk tools predict survival at one year, whereas many patients are young and clinicians aim for five, ten, or twenty years. When patients improve but fall short of goals, treatment becomes individualized. One option is switching the PDE5 inhibitor to riociguat, supported by the REPLACE trial, which showed improved clinical outcomes in patients who had not met treatment goals. Riociguat could also be part of initial combination therapy with an endothelin receptor antagonist. Most clinicians, including Dr. Vaidya, now favor escalating by adding a third pathway and sometimes a fourth, depending on how far the patient is from goals and what the patient can manage next.

The next episode in this series, "The Fourth PAH Pathway: Activin Signaling Inhibitors and Parenteral Therapy," features the panel on the fourth pathway and the continuing need for parenteral therapy.