CEC follow up in patients with SC treprostinil therapy.

<p>(^†Deceased).</p

Effect of SC treprostinil therapy on functional and hemodynamic status.

†<p>Deceased.</p

CECs are increased in irreversible and idiopathic pediatric PAH patients.

<p>CEC counts were significantly increased in irreversible and idiopathic PAH (iPAH). Effects of the group and their interaction on CEC variability were tested using ANOVA (***p = 0.0005 and **p = 0.01 for irreversible and idiopathic patients versus controls (reversible PAH), respectively.</p

CEC modification during worsening in patients treated with SC treprostinil.

<p>A- Time course of CEC counts in a patient with stable iPAH under treatment. B- Time course of CEC counts in a patient with PAH-CHD with a small VSD (subtype 1C Dana-Point classification of CHD-PAH). C- Time course of CEC counts in a patient with iPAH.</p

CEC counts in treated PAH.

<p>A- CEC counts were significantly reduced in treated PAH with oral mono- or bi-therapy in the absence of clinical worsening. Effects of group and their interaction on CEC variability were tested using ANOVA. Mono and combined oral therapy induced a significant decrease in CEC count as compared to patients in the absence of treatment (respectively ***p = 0.0007 and **p = 0.003). No difference was noticed between mono and combined therapy groups (p = 0.96 between mono and combined therapy). <b>B/C/D</b>- Time course of CEC count during PAH worsening in three patients treated with monotherapy (bosentan). Worsening was observed concomitantly to CEC level increase. Adding sildenafil to bosentan allowed a decrease in CEC levels to normal range values.</p

Characteristics of the patients treated with SC-treprostinil.

<p><i>TGA</i>, transposition of the great arteries; <i>PDA</i>, patent ductus arteriosus; <i>VSD</i>, ventricular septal defect; <i>ASD</i>, atrial septal defect.</p>†<p>Deceased.</p

Characteristics of the patients.

<p>Data are expressed as medians and their range. Baseline characteristics were compared between the groups by using Wilcoxon’s rank sum test for none normally distributed variables (age) and Student’s unpaired test otherwise. Symbols: * p<5%.</p