Abstract
Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) require a multifaceted, guideline-directed management approach. This includes active patient participation in partnership with their healthcare team to minimize disease impact and improve survival. Inhaled treprostinil, a prostacyclin analog, has been approved for both PAH and PH-ILD in two formulations: Tyvaso (treprostinil nebulizer) and the more recent Tyvaso DPI (treprostinil dry powder inhaler) [United Therapeutics]. Both formulations deliver therapy directly to the lung vasculature, minimizing the risk of ventilation-perfusion mismatch, reducing systemic exposure, and decreasing the incidence of adverse events commonly associated with parenteral and oral prostacyclin formulations. While practical recommendations for the treprostinil nebulizer have been previously published, Tyvaso DPI provides a well-tolerated, convenient administration option for patients requiring prostacyclin therapy. This review provides an overview of inhaled prostacyclin therapy with a focus on practical considerations for managing PAH and PH-ILD patients treated with Tyvaso DPI. Recommendations from a panel of pulmonary hypertension advanced practice providers include patient selection, education, communication, onboarding and monitoring, transition and titration, side effect mitigation, and the availability of clinician- and patient-facing resources.