Individualizing PAH Therapy in an Evolving Treatment Landscape - Episode 5
Treating Established vs Newly Diagnosed PAH: Confirming the Diagnosis First
Confirming that a referred patient truly has PAH comes before any treatment change. The panel reviews diagnostic pitfalls, management of patients arriving on monotherapy, and the education needs of newly diagnosed patients.
In "Treating Established vs Newly Diagnosed PAH: Confirming the Diagnosis First," the panel considers how management changes when a patient arrives with a prior diagnosis.
Dr. McLaughlin asks Dr. Kim how his approach differs between patients with an established diagnosis and those new to him. He notes that true de novo patients are increasingly rare, because many are referred already on therapy. When a referred patient is not doing well, he returns to the drawing board and confirms the diagnosis, since PAH is rare and the patient may have Group 2, 3, or 4 disease. A de novo patient is more like a deer in the headlights, overwhelmed by the diagnosis, and needs extensive education and close follow-up to address unanswered questions. Nurses play an important role here, spending time with patients in clinic and by phone afterward.
Dr. McLaughlin then asks what the typical referred patient on treatment looks like and how he adjusts medications. Dr. Kim says community patients most often arrive on PDE5 inhibitor monotherapy. If they are not high risk, he adds the second pathway, perhaps through the fixed-dose combination for convenience. Higher-risk patients may need repeat catheterization and echo and preparation for parenteral therapy, as part of shared decision-making, and a newer add-on therapy is also an option.
Asked how he keeps community physicians involved, Dr. Kim says feeding information back to the referral base is critical and the electronic record makes it easier. A catheterization report without tracings may prompt a repeat study, and a new patient should have a baseline echo. Dr. Vaidya adds that stiff, enlarged pulmonary arteries make an under-occluded wedge common, which can produce a false Group 2 label, so integrating the echo helps catch it early. Dr. Channick recalls a referral study in which more than 40% of diagnoses changed after evaluation at a center. The panel agrees that medications work only when applied to the right diagnosis.
In "Assessing Treatment Response in PAH: Longitudinal Follow-Up and the Right Ventricle," Dr. Channick and Dr. Vaidya explain how they judge response after treatment begins.
