Key result
ASD closure plus PAH therapy is linked to ~27% greater systemic blood flow and improved WHO-FC.
Why the study?
Therapeutic strategies for pulmonary arterial hypertension associated with atrial septal defect remain a matter of debate.
Does targeted therapy improve hemodynamics and functional class in adult patients with ASD-PAH after ASD closure?
Observational (n=5)
No
Does targeted therapy improve hemodynamics and functional class in adult patients with ASD-PAH after ASD closure?
Absolute Event Rate: 4.2% vs 3.3%
p-value: p=<0.05
Targeted PAH therapy combined with ASD closure improves hemodynamics and functional class in adult patients with ASD-PAH, reversing the worsening of pulmonary vascular resistance seen with closure alone.
May support combined ASD closure and PAH therapy in adult ASD-PAH; hypothesis-generating and requires prospective validation.
BACKGROUND: Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate. METHODS AND RESULTS: We identified 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certified PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy significantly increased systemic blood flow (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 dyne · s/cm(5) to 926 dyne · s/cm(5)) in 1 case, as well as after a long time since ASD closure (491.0 ± 53.7 dyne · s/cm(5) to 1045.0 ± 217.8 dyne · s/cm(5)) 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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Fujino et al. (2015) conducted an observational in Atrial septal defect and associated pulmonary arterial hypertension (ASD-PAH) (n=5). Targeted therapy with certified PAH drugs combined with ASD closure vs. Baseline (pre-treatment) was evaluated on Systemic blood flow (Qs) (p=<0.05). The combination of ASD closure and targeted PAH therapy significantly increased systemic blood flow from 3.3 to 4.2 L/minute and improved WHO Functional Class in adult patients with ASD-PAH.
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