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Introduction:The use of treprostinil in pediatric patients has expanded to include select patients with heart failure (HF) and/or single ventricle (SV) physiology. The safety and efficacy of this therapy, particularly in those with elevated left atrial (LA) pressures, is understudied.Hypothesis:We hypothesized that among children awaiting heart transplantation (HT), treprostinil would (1) be well tolerated, (2) improve hemodynamics and (3) reduce HF symptoms.Methods:Retrospective review of patients listed for HT between 1/1/2012-12/31/2019 who received treprostinil therapy during the listing period. Changes in hemodynamic and functional indices between the baseline catheterization (prior to drug initiation) and prior to HT, and patient outcomes were analyzed descriptively.Results:Among 16 patients, 7 (44%) were female, and 11 (69%) had SV physiology. The median age at drug initiation was 4 (IQR: 1, 12) years. The median duration of therapy was 308 (IQR: 168, 544)days. Fourteen (88%) survived to HT; 1 died while waiting, and 1 remains on the waitlist. With drug initiation, the median pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) fell (38 vs. 27 mm Hg & 4.8 vs. 2.8 WU, respectively), and the cardiac index increased (3.5 vs. 4.3 L/min/m2). Median LA or pulmonary capillary wedge (mPCW) pressure fell modestly in both SV and two-ventricle patients (11 vs. 9 mmHg and 22 vs. 16 mmHg, respectively) (table 1). HF symptoms improved in 6/10 (60%), including 2/6 (33%) non-ventricular assist device patients.Conclusions:Treprostinil may be used safely in patients awaiting HT, including those with SV disease. Treprostinil lowers mPAP and PVR without increasing mLA/mPCW pressure. HF symptoms improve in some patients.

Treatment of pediatric pulmonary hypertension (PH) with IV prostanoids has greatly improved outcomes but requires a central line, posing inherent infection risk. This study examines the types of infections, infection rates, and importantly the effect of line management strategies on reinfection in children receiving IV prostanoids for PH. This study is a retrospective review of all pediatric PH patients receiving intravenous epoprostenol (EPO) or treprostinil (TRE) at one academic tertiary care center between 2000 and 2014. No patients declined participation in the study or were otherwise excluded. Infectious complications were characterized by organism(s), infection rates, time to next infection, and line management decisions (salvage vs. replace). Of the 40 patients followed, 13 sustained 38 infections involving 49 pathogens, with a predominance of gram-positive (GP) organisms (n?=?35). The pooled infection rate was 1.06 per 1000 prostanoid days with no difference between EPO and TRE. No significant difference in reinfection rate was observed when comparing line salvage to replacement, regardless of organism type. Both overall and organism-type comparisons suggest longer time between line infections following line salvage compared with line replacement (732 vs. 410 days overall; 793 vs. 363 days for GP; 611 vs. 581 days for gram-negative [GN]; P?>?0.05 for all comparisons). Central line replacement following blood stream infections in pediatric PH patients does not improve subsequent infection rates or time to next infection, and may lead to unnecessary risks associated with line replacement, including potential loss of vascular access. A revised approach to central line infections in pediatric PH is proposed.