Key result
Targeted PAH therapies improve 6-minute walk distance by ~53 m and may extend Eisenmenger syndrome survival.
Why the study?
Clinical management of patients with Eisenmenger syndrome has dramatically changed in the new therapeutic era of pulmonary arterial hypertension, requiring updated treatment approaches.
Targeted therapies for pulmonary arterial hypertension improve functional capacity and quality of life in patients with Eisenmenger syndrome.
Supports targeted PAH therapies for functional gains in Eisenmenger syndrome; leaves open survival confirmation in prospective RCTs.
Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart defects. ES greatly affects functional capacity, quality of life, and survival of patients. Clinical management of patients with ES has been dramatically changed and developed in a new therapeutic era of PAH, approximately a century after the first ES case described by Dr. Eisenmenger. Targeted drugs for PAH improve functional capacity and quality of life of patients with ES. In addition, these drugs may also provide a benefit for patients' survival. To achieve further development in medical treatment of patients with ES, they will need to be individualized according to functional classes, underlying cardiac defects, and underlying systemic diseases.
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Hiroyuki Fukushima (2015) conducted a review in Eisenmenger syndrome. Advanced targeted PAH therapy (e.g., bosentan, sildenafil, epoprostenol) vs. Placebo or standard care was evaluated. Advanced targeted therapies for pulmonary arterial hypertension, such as bosentan, improve functional capacity (e.g., a 53.1 m treatment effect in 6-minute walk distance) and may provide a survival benefit for patients with Eisenmenger syndrome.
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