Prostanoids in pediatric pulmonary hypertension: clinical response, time-to-effect, and dose-response.

Autor: Tella JB; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA., Kulik TJ; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.; Department of Pediatrics, Harvard Medical School, Boston, MA, USA., McSweeney JE; Cardiovascular Nursing Patient Services, Boston Children's Hospital, Boston, MA, USA., Sleeper LA; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.; Department of Pediatrics, Harvard Medical School, Boston, MA, USA., Lu M; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA., Mullen MP; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.; Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
Jazyk: angličtina
Zdroj: Pulmonary circulation [Pulm Circ] 2020 Dec 07; Vol. 10 (4), pp. 2045894020944858. Date of Electronic Publication: 2020 Dec 07 (Print Publication: 2020).
DOI: 10.1177/2045894020944858
Abstrakt: 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.
(© The Author(s) 2020.)
Databáze: MEDLINE
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