Clinical InvestigationsPediatricsLong-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: Does the beneficial effect persist?
Section snippets
Patients
Between November 2002 and February 2007, a cohort of 30 patients (20 adults, 10 children) with PAH associated with congenital or surgically created systemic-to-pulmonary shunt and treated with bosentan (Tracleer, Actelion Pharmaceuticals, Allschwill, Switzerland) had standardized follow-up at 2 Dutch tertiary medical referral centers for pulmonary hypertension. Eight patients included in the series initially received bosentan as part of a clinical trial.11 All patients or patients'
Patients
Baseline characteristics are summarized in Table 1. Ventricular septal defect (VSD) was the most common heart defect (53%) and was frequently associated with additional shunt defects, such as atrial septal defect (ASD) and persistent ductus arteriosus. An isolated ASD was seen in 19% of all patients, all of whom were adults. Twenty-six (87%) patients had classic Eisenmenger syndrome, 6 of these with a shunt before and 20 after the level of the tricuspid valve. The remaining 4 patients had
Discussion
This study, to our knowledge, is the first to report long-term response to bosentan through 2.7 years of follow-up in both adults and children with PAH associated with systemic-to-pulmonary shunt. Our results demonstrate short-term improvement in functional class and exercise capacity in both patient groups, which is in congruence with previous reports.11, 20 During longer follow-up, improvement in 6MWD, the major outcome parameter in PAH studies, persisted up to 1 year but declined thereafter.
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Cited by (0)
A. Vonk-Noordegraaf, B.J.M. Mulder, and R.M.F. Berger have received lecture fees and/or grant support from Actelion Pharmaceuticals Ltd, Allschwill, Switzerland. R.L.E. van Loon, E.S. Hoendermis, M.G.J. Duffels, and H.L. Hillege have no conflicts of interest to disclose.