Successful outcomes for atrial septal defect associated with pulmonary arterial hypertension using a "treat-repair-treat" strategy

Object: Due to substantial progress of medications to treat pulmonary arterial hypertension (PAH), clinicians are now reconsidering interventional procedures for congenital heart disease (CHD) that were previously considered to be contraindicated or high-risk. This study provides a retrospective evaluation of our institutional approach to treat PAH, proceed to CHD defect closure, and then continue PAH treatment, termed "treat-repair-treat". Method: We retrospectively reviewed 14 patients with atrial septal defects (ASD) (mean age of 27.9 +/- 7.4 years) with significant PAH who underwent defect closure in our hospital between 2010 and 2018. All patients received targeted PAH medications before defect closure. Results: Pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP) decreased after targeted therapy (PVR: 5.7 +/- 1.8 VS 8.7 +/- 2.9 Wood Units, P = 0.003; mPAP: 52.2 +/- 7.2 VS 57.1 +/- 7.4 mmHg, p = 0.2). Eventually, all patients underwent successful defect closure without adverse events. The average followup duration was 21.1 months. Twelve patients had post-procedure visits with improved symptoms. Five patients underwent post-procedure right heart catheterization (RHC) which confirmed normal pulmonary artery pressure (mPAP <= 25 mmHg). Two patients discontinued targeted medical therapy against medical advice and both had worsened exercise capacity at their most recent follow up. Repeat RHC confirmed that these 2 patients had persistent, severe PAH. Conclusion: Our single center results support an effective "treat-repair-repair" strategy for patients with ASD and PAH. Continued targeted PAH treatment and close follow-up remain important after ASD closure. Larger, multicenter studies are needed to confirm our findings.