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Individualizing PAH Therapy in an Evolving Treatment Landscape - Episode 6

Assessing Treatment Response in PAH: Longitudinal Follow-Up and the Right Ventricle

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Longitudinal follow-up, symptoms, exercise capacity, biomarkers, and echocardiography all contribute to judging response. The panel explains why right ventricular assessment adds precision to serial noninvasive risk scores.

"Assessing Treatment Response in PAH: Longitudinal Follow-Up and the Right Ventricle" explores how clinicians judge whether a patient is responding to therapy.

Dr. McLaughlin reminds the panel that diagnosis and initial treatment are only the first step and asks Dr. Channick how to assess response. He says longitudinal experience is essential, because a clinician who has followed a patient for years reads the same visit differently than one meeting the patient for the first time. Even a simple question such as how the patient feels needs context, and he jokes that a man may say he feels great while his spouse shakes her head behind him. Beyond symptoms, he looks at exercise capacity, six-minute walk distance, and biomarkers. Cardiology colleagues have persuaded him to reassess the right ventricle, so he now orders more echocardiograms than repeat right heart catheterizations and reads them beyond the four-chamber view.

Dr. McLaughlin points out that right ventricular findings are absent from the risk scores. Dr. Vaidya agrees and distinguishes the comprehensive baseline assessment, such as the European approach or REVEAL 2.0, from the noninvasive serial tools used at follow-up, namely REVEAL Lite 2 and COMPERA 4-strata. These tools rely on functional class, six-minute walk, and BNP or NT-proBNP, which are relatively nonspecific to the disease.

Data show that adding right heart parameters from echo, such as right ventricular function or tricuspid regurgitation, helps stratify intermediate-risk patients more precisely. When patients ask how they are doing, Dr. Vaidya gives a simple answer: if the right heart is performing normally, they are doing well. Some patients calculate as high risk because they walk less far or report worse functional class, yet their right heart parameters are adequate. She urges clinicians to value the validated calculators while recognizing when right heart performance contradicts them.

Our next episode, "PAH Risk Scores vs Right Heart Function: Interpreting Echo Beyond Pressure," presents two contrasting patients that show why scores alone can mislead.

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