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Introduction : Prior to the availability of oral/inhaled (inh) PAH drugs, i.e. 2001–2007, IV epoprostenol (EPO) was the only approved drug for pts un-responsive to calcium channel blockade (CCB). We report our experience transitioning selected pediatric IPAH pts from EPO (initiated prior to 2001) to oral/inh PAH drugs. Methods : We retrospectively reviewed all pediatric IPAH pts treated at Columbia Univ (1991–2008) who transitioned off EPO to oral/inh drugs. Pre-specified criteria for transition included: functional class (FC) I/II, mPAP<35mmHg, normal CO and age > 6 yrs at transition. Hemodynamic and exercise data were obtained at baseline (prior to EPO initiation), at peak EPO dose, and at least 2 mos after EPO discontinuation (d/c) on stable oral/inh drug doses. Data: m ± SD. Results : 13 IPAH pts (6 F) transitioned off EPO between 4/03–11/07 (max dose 100 ± 42ng/kg/min) to oral/inh drugs at 11.6 ± 3.6 yrs of age. Mean duration on EPO: 6.6 ± 3.4yrs (range 1.2–12yrs); f/u post EPO d/c: 10.4 ± 11.6 mos (range 2–42.8 mos). Treatment with EPO improved hemodynamics vs. baseline (p<0.05) with no significant changes in hemodynamics or exercise capacity off EPO. In addition, further improvement was reported in FC off EPO (p<0.002; n=13). Conclusions : In selected IPAH children who began EPO prior to the availability of oral/inh PAH drugs, transition to oral/inh PAH therapy appears safe with efficacy maintained. Whether early initiation of EPO improves long-term outcome vs. deferral of EPO until oral/inh therapy fails remains unknown; further studies with longer-term follow-up are warranted.

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