Contemporary outcomes of complete atrioventricular septal defect repair: Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database

Abstract

Objective Contemporary outcomes data for complete atrioventricular septal defect (CAVSD) repair are limited. We sought to describe early outcomes of CAVSD repair across a large multicenter cohort, and explore potential associations with patient characteristics, including age, weight, and genetic syndromes. Methods Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having repair of CAVSD (2008–2011) were included. Preoperative, operative, and outcomes data were described. Univariate associations between patient factors and outcomes were described. Results Of 2399 patients (101 centers), 78.4% had Down syndrome. Median age at surgery was 4.6 months (interquartile range, 3.5–6.1 months), with 11.8% (n = 284) aged ≤2.5 months. Median weight at surgery was 5.0 kg (interquartile range, 4.3–5.8 kg) with 6.3% (n = 151) <3.5 kg. Pulmonary artery band removal at CAVSD repair was performed in 122 patients (4.6%). Major complications occurred in 9.8%, including permanent pacemaker implantation in 2.7%. Median postoperative length of stay (PLOS) was 8 days (interquartile range, 5–14 days). Overall hospital mortality was 3.0%. Weight<3.5 kg and age ≤ 2.5 months were associated with higher mortality, longer PLOS, and increased frequency of major complications. Patients with Down syndrome had lower rates of mortality and morbidities than other patients; PLOS was similar. Conclusions In a contemporary multicenter cohort, most patients with CAVSD have repair early in the first year of life. Prior pulmonary artery band is rare. Hospital mortality is generally low, although patients at extremes of low weight and younger age have worse outcomes. Mortality and major complication rates are lower in patients with Down syndrome.

Prevalence of Noncardiac and Genetic Abnormalities in Neonates Undergoing Cardiac Operations: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

“…In our population, neonates undergoing AVC surgery have a 33% prevalence of trisomy 21, which is less than the 78.4% prevalence noted by St. Louis and colleagues for all patients with AVSD [29]. However, our study focuses specifically on neonates; among those with AVSD having neonatal repair, trisomy 21 seems to be half as prevalent versus prior studies that include all age groups, likely due to differences in study populations in time to presentation for surgery.…”

Advances in the Understanding of the Genetic Determinants of Congenital Heart Disease and Their Impact on Clinical Outcomes

“…Early surgical studies reported worse surgical outcomes in patients with Down syndrome undergoing repair for complete atrioventricular septal defect compared with patients without genetic syndromes . More recently, several studies have demonstrated equal or decreased risk of in‐hospital mortality for patients with Down syndrome undergoing repair of CHD (including complete atrioventricular septal defect) compared with patients with normal karyotypes except among patients with single ventricle physiology . Several studies that included long‐term outcomes for complete atrioventricular septal defect repair have demonstrated a decreased rate of reoperation for left atrioventricular valve repair and for subaortic stenosis in patients with Down syndrome, which is likely related to valve and left ventricular outflow tract morphology differences …”

Cardiopatia Congénita em Crianças com Síndrome de Down: O que Mudou nas Últimas Três Décadas?

“…6 As crianças com SD e cardiopatia congé-nita passaram a ser operadas cada vez mais cedo, com resultados cirúrgicos e mortalidade sobreponíveis aos das crianças sem SD. 7,8 …”