Complete Atrioventricular Septal Defect Repair in Patients With Down Syndrome Presenting Beyond Six Months- A Single Center Experience.

Abstract

BackgroundComplete atrioventricular septal defect (cAVSD) is the most common cardiac lesions associated with Down syndrome (DS). In DS, cAVSD repair is ideally performed before six months of age to prevent irreversible pulmonary artery hypertension (PAH). However, in developing countries, delayed repair is often unavoidable. We retrospectively analyzed the outcomes of cAVSD repair in DS patients presenting beyond six months of age.MethodBetween 2015 and 2019, 60 DS patients with cAVSD who underwent biventricular repair were reviewed. During the same time period 42 DS patients under 6 months of age underwent cAVSD repair. Their laboratory parameters and echocardiographic data were analyzed. Clinical findings as well as echocardiographic results at last follow-up were recorded.ResultMedian age of the cohort was eight months. There were 7/60 (11.7%) early mortalities due to right ventricular dysfunction secondary to persistent PAH (n = 4) and sepsis (n = 3). We found that right atrioventricular valve regurgitation (AVVR) grade (OR = 5.7, P = .017), postoperative serum bilirubin (OR = 4.03, P = .04), postoperative creatinine (OR = 7.06, P = .008), and right AVVR Gmax (OR = 6.08, P = .009) were independent risk factors for mortality. Mean follow-up was 1658 ± 771 days with a survival rate of 85% at 1, 5, and 10 years. Late reoperation (5.6% vs 3.7%) as well as prevalence of more than mild regurgitation was higher for the right AV valve as compared with the left AV valve (11.5% vs 1.9%). We did not find any factors associated with reoperation. All patients were clinically asymptomatic and did not have residual severe PAH.ConclusionComplete Atrioventricular septal defect repair in late-presenting patients with DS is feasible and yields good long-term outcomes. However, close follow-up is necessary to monitor for the late development of right AVVR.