Congenital heart diseaseAmbrisentan for Pulmonary Arterial Hypertension Due to Congenital Heart Disease
Section snippets
Methods
A retrospective chart review was performed on consecutive patients with ES followed at the Columbia University Pulmonary Hypertension Center who were initiated on ambrisentan between January 2007 and August 2008. Data collected included demographics, type of CHD, and past or present treatment with oral and intravenous advanced therapies for PAH. Comparisons were performed of rest and exercise SaO2, 6-minute walking distance, blood hemoglobin level, World Health Organization (WHO) functional
Results
Seventeen patients with ES were initiated on ambrisentan from January 2007 to August 2008. Demographic data, type of CHD, and associated conditions are listed in Table 1. The average age at the initiation of treatment was 32.2 ± 11.9 years. Fifteen patients were previously treated with bosentan or the selective endothelin-A receptor antagonist sitaxsentan before ambrisentan (Table 1). In all, 14 patients were treated with sitaxsentan for an average of 2.5 ± 1.4 years, and 10 patients were
Discussion
During the past 20 years, there has been significant progress in the treatment and outcomes of patients with idiopathic and associated PAH. The effects are less well understood in patients with classic ES. Although many of the larger placebo-controlled randomized studies involving targeted agents for PAH have been performed on a heterogenous population of patients, only a small percentage have ES.5, 13, 14, 15, 16 One target of PAH treatment that seems to be of particular importance for
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