Reversibility of Pulmonary Hypertension Following Surgical Atrial Septal Defect Closure in Children with Down Syndrome

BACKGROUND: Many Down syndrome (DS) patients have an atrial septal defect (ASD) and associated pulmonary hypertension (PH) from early childhood. ASD closure in DS patients with PH is often controversial due to concerns regarding exacerbation of PH. The aim of this study was to investigate the clinical outcome following surgical ASD closure in children with DS. METHODS: We retrospectively reviewed the medical records of DS patients who underwent surgical ASD patch closure from January 2000 to December 2016. RESULTS: A total of 15 patients underwent surgery for ASD. Prior to ASD patch closure, nine patients were diagnosed with PH, three of whom took medications for PH. The mean age of patients at ASD patch closure was 17.3 months, and the mean diameter of the ASD was 10.2 mm. Three patients who took medications for severe PH underwent ASD patch closure at ages 7, 12, and 25 months. Two patients continued medication for an additional 13 and 21 months, and one patient remained on medication 52 months after ASD closure. PH did not recur following discontinuation of selective pulmonary vasodilators in two patients. Although a moderate degree of PH remained in one patient due to a chronic lung problem, it was improved compared to before ASD closure. No PH was observed in the remaining 12 patients following ASD closure. CONCLUSIONS: A large ASD can be closed even in DS patients with severe PH during early childhood with the support of multiple selective pulmonary vasodilators.