Key result
Late biventricular CAVSD repair in Down syndrome carries ~12% early mortality.
Why the study?
In Down syndrome patients, complete atrioventricular septal defect repair is ideally performed before six months to prevent irreversible pulmonary artery hypertension, but delayed repair is common in developing countries and outcomes are unclear.
Does biventricular repair of complete atrioventricular septal defect yield good long-term outcomes in Down syndrome patients presenting beyond six months of age?
Cohort (n=60)
No
Does biventricular repair of complete atrioventricular septal defect yield good long-term outcomes in Down syndrome patients presenting beyond six months of age?
Biventricular repair of complete AVSD in Down syndrome patients presenting after 6 months of age is feasible with an 85% long-term survival rate, though right AV valve regurgitation requires close monitoring.
Right AV valve regurgitation warrants monitoring after late repair; leaves open optimal timing for cAVSD in Down syndrome.
Background Complete 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. Method Between 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. Result Median 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. Conclusion Complete 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.
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Patel et al. (2025) conducted a cohort in Complete atrioventricular septal defect (cAVSD) in Down syndrome (n=60). Biventricular repair for complete atrioventricular septal defect was evaluated on Early mortality. Biventricular repair for complete atrioventricular septal defect in Down syndrome patients presenting beyond six months of age resulted in 11.7% early mortality and an 85% survival rate at 10 years.
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