Atrial Septal Defect–Associated Pulmonary Hypertension: Outcomes of Closure With a Fenestrated Device

Unlike other intracardiac shunts, there is no known linear relationship between ostium secundum atrial septal defects (ASD) and development of pulmonary hypertension (PH). PH is observed in 8% to 10% of all ASD patients. Atrial septal defect–associated pulmonary hypertension (ASDAPH) is usually independent of the degree, duration of shunting, and defect size. Complete closure of ASD in these patients can be detrimental due to the potential risk for increase in pulmonary vascular resistance (PVR). Fenestrated closure allows for controlled residual shunt providing adequate cardiac output with a mechanism for decompression in the event of critical increase in PVR. After approval from institutional review boards and agencies protecting human subjects, 42 patients from 29 international centers underwent compassionate use of the Occlutech Fenestrated Atrial Septal Defect (FASD) device. Physician implanters reported outcomes via electronic survey. Follow-up data were available for 25 patients (72% female, n=18) from 18 centers. Symptomatic improvement was observed in a majority of the patients with reduction in New York Heart Association class III symptoms from 68% at baseline to 8% at long-term follow-up. Mean oxygen saturation improved from 93% at baseline to 97% at long-term follow-up (P=0.0066). Reduction in right atrial pressure and mean pulmonary arterial pressure were also noted. During follow-up, one patient had spontaneous occlusion of the fenestration requiring emergency stenting. No other major complications were observed. FASD implantation improves outcomes in patients with ASDAPH; however, further studies are required in a large cohort of patients to determine timing of intervention, optimal fenestration size, and long-term prognosis.