Background: Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate. Methods and Results: We identifed 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certifed PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy signifcantly increased systemic blood fow (Qs) from the baseline (from 3.3 ± 0.6 L/minute to 4.2 , 1.0 L/minute, P < 0.05) with a significant improvement in the World Health Organization Functional Class (WHO-FC; from 2.8 ± 0.4 to 1.6 ± 0.5, P < 0.05). The hemodynamic data before and after ASD closure without targeted therapy showed further elevation of pulmonary vascular resistance shortly after ASD closure (678 dynes/cm5 to 926 dynes/cm5) in 1 case, as well as after a long time since ASD closure (491.0 ± 53.7 dynes/cm5 to 1045.0 ± 217.8 dynes/cm5) in 2 cases. This worsening was reversed after the targeted therapy, accompanied by an increase in Qs and an improvement in WHO-FC in all cases. Conclusions: Targeted therapy should be added to ASD closure in adult patients with ASD-PAH.
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