Congenital heart disease
Ambrisentan for Pulmonary Arterial Hypertension Due to Congenital Heart Disease

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Eisenmenger syndrome (ES) is a known complication of congenital heart disease associated with unrepaired systemic to pulmonary shunts. Evidence for use of targeted pulmonary arterial hypertension therapy in ES is limited. The early experience using ambrisentan was evaluated in a cohort of consecutive patients with ES who initiated ambrisentan at Columbia University's Pulmonary Hypertension Center from January 1, 2007, to August 1, 2008. Effects of ambrisentan on rest and exercise systemic arterial oxygen saturation (SaO2), exercise capacity, functional status, hemoglobin levels, and hemodynamics were evaluated and compared using paired Student's t tests. Seventeen patients were evaluated at short-term (mean 163 ± 57 days) and longer term (mean 2.5 ± 0.5 years) follow-up. At short-term follow-up, there was an improvement in exercise capacity (6-minute walking distance 389 ± 74 vs 417 ± 77 m, p = 0.03, n = 11) and maintenance of rest SaO2 (89 ± 7% vs 89% ± 6%, p = 0.75, n = 15), exercise SaO2 (75 ± 15% vs 77% ± 15%, p = 0.33, n = 11), functional class (improvement in 2 patients, no change in 13), and hemoglobin (16.5 ± 2.8 vs 15.8 ± 1.8 g/dl, p = 0.11, n = 14). At longer term follow-up compared to baseline and short-term follow-up, there was stability of exercise capacity, SaO2, functional class, and hemoglobin. In conclusion, in this single-center cohort of patients with ES, ambrisentan was safe and was associated with increasing exercise capacity at short-term follow-up, with patients maintaining SaO2, functional class, and hemoglobin, and with no significant evidence of clinical deterioration at longer term follow-up. Additional studies are required to further assess the efficacy of ambrisentan in patients with ES.

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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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