Key result
Treprostinil >15.5 ng/kg·min is linked to a ~13% reduction in adverse outcomes.
Why the study?
To compare the hemodynamic effects of treprostinil and postoperative outcomes in pediatric single-ventricle patients with pulmonary hypertension following Glenn or Fontan palliation, and identify key prognostic factors.
Does intravenous Treprostinil improve hemodynamics and postoperative outcomes differently in pediatric patients following Glenn versus Fontan procedures?
Does intravenous Treprostinil improve hemodynamics and postoperative outcomes differently in pediatric patients following Glenn versus Fontan procedures?
Intravenous Treprostinil effectively reduces pulmonary artery pressure in pediatric single-ventricle patients post-palliation, with greater hemodynamic efficacy in Glenn patients, and doses >15.5 ng/kg/min may protect against adverse postoperative outcomes.
Higher-dose treprostinil was associated with fewer adverse outcomes; leaves open optimal dosing and net benefit in higher-risk Glenn patients.
To compare the hemodynamic effects of Treprostinil and postoperative outcomes in pediatric single-ventricle patients with pulmonary hypertension (PH, mean pulmonary artery pressure (mPAP) > 15 mmHg) following Glenn or Fontan palliation, and identify key prognostic factors associated with treatment response and clinical outcomes. Retrospective analysis of 79 pediatric patients (28 Glenn in Group 1 and 51 Fontan in Group 2) from 2015 to 2022, excluding those requiring postoperative extracorporeal membrane oxygenation. All received intravenous Treprostinil. Changes in mPAP, vasoactive-inotropic score, and postoperative recovery parameters were compared. Binary logistic regression identified prognostic factors, and receiver operating characteristic curve analysis evaluated Treprostinil dose predictive value. Regimen distribution did not differ between groups (14 vs. 14 in Group 1 and 35 vs. 16 in Group 2 on early/later standardized regimens, P = 0.103). Group 1 had more additional cardiac procedures (75% vs. 49%, P = 0.025), with similar Treprostinil maintenance doses (17.5 [10,25] vs. 15 [14,20] ng/(kg·min), P = 0.421). At 24 h, mPAP decreased significantly more in Group 1 (17 ± 3 to 13 ± 3 mmHg vs. 17 ± 3 to 15 ± 2 mmHg, P < 0.001). Vasoactive-inotropic score decreased in Group 1 ( P < 0.001) but not in Group 2 ( P = 0.063). Group 1 had higher resuscitation (29% vs. 6%, P = 0.014) and mortality (11% vs. 0, P = 0.041), but lower chest tube drainage, shorter placement duration, and hospital stay. Glenn surgery was an independent risk factor for resuscitation/death (odds ratio = 6.518, P = 0.012); Treprostinil (odds ratio = 0.871, P = 0.004) and maintenance dose > 15.5 ng/(kg·min) (area under the curve = 0.717) protected against adverse outcomes (thromboembolic events, renal replacement therapy, tracheal reintubation, mortality, resuscitation). Treprostinil effectively reduces mPAP in both groups, with significantly greater hemodynamic efficacy in Glenn patients, who face higher resuscitation and mortality risks due to greater anatomical complexity. Fontan patients have lower mortality but prolonged morbidity from pleural effusions. The Treprostinil maintenance dose > 15.5 ng/(kg·min) is a protective factor for postoperative adverse outcomes.
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Wang et al. (2026) studied this question. Treprostinil >15.5 ng/kg·min reduced adverse outcomes (OR 0.871), with greater mPAP reduction in Glenn patients who had higher resuscitation (29% vs 6%) and mortality (11% vs 0%).
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