LAUSR

We aimed to assess the efficacy and safety of pulmonary vasodilators in patients after the Fontan procedure. The PubMed, EMBASE, and Cochrane Library databases were searched through November 2019 for studies comparing pulmonary vasodilators and controls in Fontan patients. The variables assessed included change in pulmonary resistance, heart function, exercise capacity, quality of life, mortality, and adverse events after drug administration. A random/fixed effects model was used to assess the mean difference (MD)/risk ratio (RR) with 95% confidence intervals (CIs). Subgroup analysis was stratified by drug category. In total, 449 Fontan patients from 13 studies were included. Pooled estimates of the change in pulmonary arterial pressure (MD, − 1.07; 95% CI, − 2.75 to 0.60; p  = 0.21), New York Heart Association class (MD, − 0.57; 95% CI, − 1.41 to 0.28; p  = 0.19), peak oxygen consumption (MD, 1.46; 95% CI, 0.01 to 2.90; p  = 0.05), Short Form 36 (MD, 1.39; 95% CI, − 0.62 to 3.39; p  = 0.17), mortality (RR, 0.37; 95% CI, 0.08 to 1.65; p  = 0.19), and any adverse event (RR, 0.94; 95% CI, 0.71 to 1.24; p  = 0.64) were not significantly different between the drug and control groups. Likewise, most results of the subgroup analysis revealed no significant between-group differences. Pulmonary vasodilator therapy appears to be safe, but not beneficial, in the categories of pulmonary resistance, heart function, or quality of life in patients who have undergone a Fontan procedure. No significant evidence was found to confirm that most pulmonary vasodilators could improve exercise capacity in Fontan patients.