FDA Approves Tyvaso (treprostinil) for Pulmonary Arterial Hypertension and Pulmonary Hypertension Associated with Interstitial Lung Disease

Not reported in label.

Tyvaso is indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to improve exercise ability. Studies establishing effectiveness predominately included patients with NYHA Functional Class III symptoms and etiologies of idiopathic or heritable PAH (56%) or PAH associated with connective tissue diseases (33%). Tyvaso is also indicated for the treatment of pulmonary hypertension associated with interstitial lung disease (PH-ILD; WHO Group 3) to improve exercise ability. The study establishing effectiveness predominately included patients with etiologies of idiopathic interstitial pneumonia (IIP) (45%) inclusive of idiopathic pulmonary fibrosis (IPF), combined pulmonary fibrosis and emphysema (CPFE) (25%), and WHO Group 3 connective tissue disease (22%).

Use only with the Tyvaso Inhalation System. Administer undiluted, as supplied. A single breath of Tyvaso delivers approximately 6 mcg of treprostinil. Administer in 4 separate treatment sessions each day approximately 4 hours apart, during waking hours. Initial dosage: 3 breaths (18 mcg) per treatment session. If 3 breaths are not tolerated, reduce to 1 or 2 breaths. Dosage should be increased by an additional 3 breaths per treatment session at approximately 1- to 2-week intervals, if tolerated. Titrate to target maintenance doses of 9 to 12 breaths per treatment session, 4 times daily. If adverse effects preclude titration to target dose, continue at the highest tolerated dose. If a scheduled treatment session is missed or interrupted, resume as soon as possible at the usual dose. Do not mix Tyvaso with other medications in the Tyvaso Inhalation System. Avoid skin or eye contact with Tyvaso solution. Do not orally ingest the Tyvaso solution.

For PAH, TRIUMPH I was a 12-week, randomized, double-blind, placebo-controlled, multicenter study of patients with PAH (WHO Group 1), nearly all with NYHA Class III (98%) symptoms who were receiving either bosentan or sildenafil for at least 3 months prior to study initiation. The controlled clinical experience was limited to 12 weeks in duration. For PH-ILD, trial data not available in label.

Not reported in label.

While there are long-term data on use of treprostinil by other routes of administration, nearly all controlled clinical experience with inhaled treprostinil has been on a background of bosentan (an endothelin receptor antagonist) or sildenafil (a phosphodiesterase type 5 inhibitor). The effects diminish over the minimum recommended dosing interval of 4 hours; treatment timing can be adjusted for planned activities.