The beauty of brevityPreliminary Experience With Bosentan as Initial Therapy in Childhood Idiopathic Pulmonary Arterial Hypertension
Section snippets
Methods
The data from all children diagnosed with IPAH at The Royal Children’s Hospital, Melbourne, Australia, between January 1980 and February 2005, was retrospectively analyzed. Our institution provides the sole pediatric cardiology service for the state of Victoria, with a population of 4.5 million. For the purpose of this study, patients were divided into two groups—those receiving bosentan as primary therapy for IPAH (bosentan treatment group, n = 7) and those receiving conventional medical
Results
Table 1 shows the baseline demographics, initial cardiac catheter findings and outcomes in the two groups. Children in both the bosentan group and the historic control group were similar in terms of age, duration of symptoms and WHO classification. There were more females in the bosentan group and a higher proportion of familial cases compared with the historic control group (p = 0.027 and p = 0.014, respectively). The proportion of subjects with acute reactivity was similar in each group.
At
Discussion
We have shown that bosentan, when used as initial therapy in a small group of pediatric subjects with IPAH, is associated with improved survival compared with historic controls. These findings could not be explained by inclusion of more mildly affected cases or earlier diagnosis in the bosentan-treated subjects.
Endothelin-1 is a potent vasoconstrictor and smooth muscle cell mitogen, and plays an important role in the pathogenesis and evolution of pulmonary hypertension.19, 20 Plasma
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Supported by a research scholarship (to C.M.S.) from The National Health and Medical Research Council, Australia (Grant 334394), and the Murdoch Children’s Research Institute, Melbourne, Australia.