MiscellaneousOutcome of Pediatric Patients With Pulmonary Arterial Hypertension in the Era of New Medical Therapies
Section snippets
Methods
From 1993 to 2008, 52 consecutive children with PAH (iPAH, n = 29; PAH-CHD, n = 23) were seen within the Dutch network for diagnosis and treatment of pediatric PAH. Patients with persistent pulmonary hypertension of the newborn were excluded. This network includes all 8 Dutch pediatric cardiology centers, of which 1 serves as an expert center to which patients with PAH are referred for diagnostic workup, initiation of therapy, and serial follow-up.11 Patient data are entered in a database
Results
Baseline characteristics of all patients at presentation at referral center are presented in Table 1. There were significantly more patients with PAH-CHD in cohort 2 than cohort 3. In cohort 2, median time from diagnosis to second-generation drug availability was 4.0 years (range 0.8 to 13.3). This period was significantly longer in patients with PAH-CHD than in those with iPAH (median 5.2 years, range 1.6 to 13.3, and median 2.7 years, range 0.8 to 4.9, respectively, p = 0.03). Cohort 2 had
Discussion
In this study, we observed that children with PAH for whom second-generation drugs were available had improved survival compared to their predicted survival. However, this improved survival appeared to be mainly driven by patients for whom second-generation drugs became available during their disease course (cohort 2), and not by patients for whom second-generation drugs were available already at diagnosis (cohort 3). Start of second-generation drugs resulted in clinical and laboratory
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