Kartik Patel, Chandrasekaran Ananthanarayanan, Trushar Gajjar, Amit Mishra, Bhavik Champaneri, Jigar Surti, Himani Pandya
U. N. Mehta Institute of Cardiology and Research Centre.
India
World Journal of Pediatric and Congenital Surgery
World J Pediatr Congenit Surg 2025;
DOI: 10.1177/21501351251322162
Abstract
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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