PURPOSE: The best method for transitioning patients with pulmonary arterial hypertension(PAH) from parenteral to inhaled prostacyclin therapy is unknown. We describe successful transition from parenteral prostacyclin to inhaled iloprost in three patients with PAH.
METHODS: Three adolescents (2 male) with PAH transitioned from parenteral prostacyclin to inhaled prostacyclin in clinic and intensive care unit settings.
RESULTS: A 13-year-old male with PAH following corrected congenital heart disease was given an initial dose of 2.5 mcg iloprost in a clinic setting and a second dose three hours later without problem. Two additional doses of 5 mcg were given that day, five doses the second day and six doses the third day. Subcutaneous treprostinil was decreased by 10 % of the steady state dose every 8 hours until discontinuation. A 17-year-old male with idiopathic PAH was given the first 2.5 mcg inhalation in the intensive care unit (ICU). Three hours later, he tolerated a 5 mcg dose without problem. He continued 5 mcg iloprost every three hours while awake and intravenous treprostinil was weaned by 10% increments every 6 hours. The second day, iloprost was increased to 7.5 mcg every 3 hours while awake and treprostinil was weaned until discontinuation. A 14-year-old female with PAH following corrected congenital heart disease was given the first 2.5 mcg dose of iloprost in clinic. She was admitted to the ICU and given 5 mcg iloprost every three hours while awake. Intravenous epoprostenol was decreased by 10 % of the steady state every 4 hours until discontinuation. All three patients were taking sildenafil at the time of transition. Pre-iloprost 6 minute walk distance averaged 447 yards (range 419-471) and fell to an average of 330 yards (range 241-388) over 6 months.
CONCLUSION: Three pediatric patients with PAH were successfully transitioned from parenteral prostacyclin to inhaled iloprost.
CLINICAL IMPLICATIONS: Transition from parenteral prostacyclin to inhaled prostacyclin therapy can be performed safely in pediatric patients with PAH. Larger clinical studies are needed to determine the optimal method of transition.
DISCLOSURE: Kelly Smith, Consultant fee, speaker bureau, advisory committee, etc. Co-therix.