Long-term outcome after percutaneous shunt closure of selected patients with atrial septal defect and severe pulmonary hypertension

Abstract

Background Pulmonary arterial hypertension (PAH) is a dreaded complication of ostium secundum atrial septal defects (ASD). Shunt closure indication remains controversial in patients with severe PAH. Purpose To investigate late outcome in selected patients with severe PAH who underwent percutaneous ASD closure. Methods We retrospectively included 14 patients (median age 47.5 years old) with mean pulmonary artery pressure (PAPm) > 40 mmHg and/or pulmonary vascular resistance indexed (PVRi) > 8 UW.m2, with persistent left to right shunting, who underwent percutaneous ASD closure. Results Three patients (21.4%) had ASD and coexisting etiology of PAH (chronic thromboembolic pulmonary hypertension, n = 1; familial form of PAH, n = 1; BMPR2 mutation, n = 1). All patients had dyspnea with 6 (42.8%) in WHO functional class (FC) 3–4. Four patients (28.6%) received PAH drugs (monotherapy, n = 3; triple therapy including intravenous prostacyclin, n = 1). Median [interquartile] PAPm and PVRi were 44 mmHg [41–47] and 9.7 UW.m2 [8.0–10.3]. Median delay between symptoms onset and shunt closure was 12 months [6–18]. Median Qp/Qs was 2.0 [1.6–2.2]. During early follow-up, WHO FC 2 was unchanged in 3 patients (21%), while the remaining 11 patients (79%) had clinical improvement. No patient died. After a median follow-up of 93 months [43–133], all patients were in WHO FC 1 or 2. PAPm at last catheterization (n = 10) had decreased in all cases. Median PAPm was 28 mmHg [26–32]. PAPm was below 25 mmHg in 3 cases. Median PVRi was 8.0 UW.m2 [6.4–9.0]. The 4 patients without catheterization were in WHO-FC 1 and with normal pulmonary pressure on echography. Five patients receiving PAH therapy (monotherapy, n = 3; double therapy, n = 2). Three patients died (one from lung infection, 6 years after closure and two from cancer, 1 and 2 years after closure). Conclusion Improved WHO FC and decreased pulmonary pressure are observed in carefully selected patients with severe PAH after ASD closure.