Few meta-analyses evaluated the efficacy and safety of pulmonary vasodilators in patients with Eisenmenger syndrome. Recently, some studies have reported conflicting results regarding improvements in exercise capacity. This study evaluated the efficacy and safety of pulmonary vasodilators in patients with Eisenmenger syndrome. Relevant studies were identified by searching major databases. Pooled outcomes were used to assess the efficacy and safety of pulmonary vasodilators. In total, five studies with 508 patients were included. Meta-analysis indicated that the pulmonary vasodilators reduced the mortality (odd risk (OR) = 0.35; 95% CI, 0.13 to 0.95; P = 0.04), slashed the mean pulmonary artery pressure (mean difference (MD) = -4.35 mmHg; 95% CI, -7.19 to -1.50; P = 0.003), decreased pulmonary vascular resistance index (MD = -480.08 dyn · s · cm-5 · m2; 95% CI, -753.51 to -206.64; P = 0.0006), increased the 6-min walk distance (MD = 28.38 m; 95% CI, 2.99 to 53.77; P = 0.03), and elevated the systemic oxygen saturation at rest (MD = 1.00%; 95% CI, 0.12 to 1.88; P = 0.03). Four studies reported side effects, but only two studies reported serious adverse effects which were mostly rare and curable. The present meta-analysis indicated that pulmonary vasodilators decrease mortality and improve hemodynamics and exercise capacity in patients with Eisenmenger syndrome. Overall, pulmonary vasodilators are well tolerated.
Keywords: Eisenmenger syndrome; efficacy; meta-analysis; pulmonary vasodilator; safety.
© The Author(s) 2021.