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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Cited by (24)
Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings
2010, American Journal of CardiologyCitation Excerpt :Patients received additional PAH-specific treatments as new therapies continued to become available during the observation period of this study. Combination treatment has become more frequent in clinical practice17,18–22 and is often considered an attractive therapeutic option to address the postulated multiple pathobiologic mechanisms involved in PAH.23 In the present study, addition of PAH-specific therapies for the reason of further improvement desired may have contributed to the observed improvement in clinical status in many patients and overall favorable survival.
Short- and Long-Term Effects of Inhaled Iloprost Therapy in Children With Pulmonary Arterial Hypertension
2008, Journal of the American College of CardiologySurvival of Patients With Idiopathic Pulmonary Arterial Hypertension After Listing for Transplantation: Impact of Iloprost and Bosentan Treatment
2007, Journal of Heart and Lung TransplantationCitation Excerpt :However, the epoprostenol survival studies were performed mainly on patients in NYHA Class III at the time of inclusion in the study.5 Survival after IPAH diagnosis with and without prostanoid (and/or ERA) treatment has been intensively analyzed.1–7 Nevertheless, survival data on Tx lists for patients with pulmonary hypertension are scarce, especially for IPAH.8,9
Quality of Life in Pulmonary Arterial Hypertension: Improvement and Maintenance With Bosentan
2007, Journal of Heart and Lung TransplantationCitation Excerpt :QoL was monitored in this clinical setting, avoiding possible distortion by invasive protocol-driven investigations. In addition, VITAL provided an opportunity to gather further bosentan safety and efficacy data, supporting previous reports of bosentan treatment outcomes in PAH patients.18–22,29,30 The absence of a placebo group may be considered a limitation of the VITAL study, with concern that some of the improvements seen during the study were attributable to the “placebo” effect, rather than drug efficacy.
The role of calcium channel blockers, steroids, anticoagulation, antiplatelet drugs, and endothelin receptor antagonists
2010, Pediatric Critical Care Medicine
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.