The Changing Landscape of Pulmonary Hypertension Diagnosis and Management

At the 2021 Fall Managed Care Forum, Ronald Oudiz, MD, director of the Liu Center for Pulmonary Hypertension at The Lundquist Institute, spoke about recent innovations in the pulmonary arterial hypertension (PAH) treatment landscape.

He began with the definition of PAH and algorithm for its diagnosis, beginning with an echocardiographic probability of pulmonary hypertension and, for patients with intermediate- or high-risk disease or an abnormal V/Q scan, referral to a pulmonary hypertension expert or treatment center. “Cardiac catheterization is the gold standard for diagnosis, but you need all of the clinical data to make the right diagnosis – always,” Dr. Oudiz advised. “Diagnosing pulmonary hypertension and categorizing it into its correct classification has prognostic and treatment implications. Believe me.”

Management at a specialized pulmonary hypertension center involves a multidisciplinary approach and consideration of clinical trial enrollment when appropriate treatment is unavailable.

Dr. Oudiz then described recent innovations in PAH management, which have led to the Food and Drug Administration approval of more than a dozen PAH drugs, that have reshaped the treatment algorithm. Notably, the introduction of prostacyclin analogues/prostanoids, including epoprostenol and treprostinil, can now be considered the “gold standard” for PAH treatment.

He specifically mentioned questions about optimal dosing of these agents, noting that “the ‘correct’ dose of intravenous/subcutaneous prostanoids is not the same for every patient. It may take some time to arrive at the optimal prostanoid dose.” Dr. Oudiz added that inhaled and oral therapies are available, but in the treatment algorithm, “[patients] have not failed PAH treatment until [they] have failed prostanoids.” He also reviewed non-medical treatments such as lung transplantation, for which there is only a narrow window of opportunity, and pointed to novel therapies on the horizon, ranging from sotatercept to an intravascular ultrasound pulmonary artery denervation.

In conclusion, Dr. Oudiz advised careful, regular, frequent assessment for all patients with PAH, is required in all cases and that, due to the complexities of prostanoid dosing, it “is best left to specialty PAH referral centers.”