Targeted therapy is required for management of pulmonary arterial hypertension after defect closure in adult patients with atrial septal defect and associated pulmonary arterial hypertension

International Heart Journal
Takeo FujinoIssei Komuro

Abstract

Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate. 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. Targeted therapy should be added to ASD closure in adult patients with ASD-PAH.

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Citations

Nov 17, 2015·Circulation Journal : Official Journal of the Japanese Circulation Society·Yasufumi KijimaHiroshi Ito
Jun 30, 2015·International Heart Journal·Wei-Chieh LeeHsiu-Yu Fang
Jul 8, 2016·International Heart Journal·Ataru IgarashiHiroshi Watanabe
Sep 22, 2020·Cardiology in Review·Gnalini SathananthanJasmine Grewal

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