Abstract

Introduction: An effective therapeutic strategy in patients with atrial septal defect (ASD) and significant pulmonary arterial hypertension (PAH) remains controversial. Hypothesis: PAH-specific medications contribute to improvement of PAH. Subsequent transcatheter ASD closure may lead to further reduction of PAP. Methods: Among 646 patients with ASD, 22 who were complicated by definite PAH had successful transcatheter ASD closure. Among them, eight patients received PAH-specific medications prior to the transcatheter procedure (PHM group) and 14 patients did not (non-PHM group). Results: The mean age at the procedure in the PHM group was 37±15 years. Initially, the PHM group had significantly higher mean pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) compared with the non-PHM group (62±21 vs. 35±8 mmHg, p<0.01; 9.6±3.8 vs. 4.1±1.1 Wood units, p<0.01). PAP and PVR in the PHM group significantly decreased to 41±10 mmHg (p<0.01) and to 4.0±0.8 Wood units (p<0.01), respectively, after treatment with PAH-specific medications. In the PHM group, after ASD closure, further reduction of estimated systolic PAP was observed (72±19 to 41±11 mmHg, p<0.01). Conclusions:Treat and repair is a valid therapeutic strategy for patients with ASD and significant PAH.

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