A ≥25% fall in mean pulmonary arterial pressure on parenteral prostanoids predicted freedom from adverse clinical events with 80.7% accuracy in pediatric PAH patients.
Cohort (n=31)
What is the clinical response, time-to-effect, and dose-response relationship of parenteral prostanoids in pediatric pulmonary arterial hypertension?
In pediatric PAH, a ≥25% reduction in mPAP on parenteral prostanoids predicts positive clinical response, though this may take up to 2 years to occur, and exceptionally high doses do not appear more effective than moderate doses.
For pediatric pulmonary arterial hypertension (PAH) patients treated with parenteral prostanoids, response predictors, and the dose–effect relationship are ill defined. We determined the following: (1) which pulmonary vascular hemodynamic variable, after initiating prostanoids, best correlates with a significant clinical response; (2) the time interval after treatment when if no pulmonary hemodynamic improvement has occurred, none is ever likely to; and (3) the relationship between the prostanoid dose and its hemodynamic effects. This is a retrospective cohort study of 31 pediatric patients with Group 1 PAH treated with parenteral prostanoids. We found the following: (1) A fall in mean pulmonary arterial pressure (mPAP) of ≥25% predicted freedom from adverse clinical events with 80.7% accuracy and was also associated with improved functional class. (2) Thirty‐three percent of patients who avoided an adverse clinical event demonstrated a ≥25% reduction in mPAP after 1 year of treatment, and 65% by 2 years. (3) Lower mPAP was seldom seen with doses of epoprostenol >60 ng/kg/min (100 ng/kg/min for treprostinil). Cardiac index was positively correlated with the dose of epoprostenol but not treprostinil; cardiac index >4 l/min/m 2 was seen at modest as well as high doses. We conclude that a ≥25% fall in mPAP on prostanoids indicates a positive clinical response which, if validated in other studies, may be useful for patient management or clinical trials. Some patients take more than 2 years for this change. Exceptionally high doses were generally not more effective than lower, although we could not determine whether lower doses would have been as effective.
Tella et al. (Tue,) conducted a cohort in Pediatric pulmonary arterial hypertension (PAH) (n=31). Parenteral prostanoids (epoprostenol, treprostinil) was evaluated on Freedom from adverse clinical events. A ≥25% fall in mean pulmonary arterial pressure on parenteral prostanoids predicted freedom from adverse clinical events with 80.7% accuracy in pediatric PAH patients.