Surgical Management of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, Trisomy 21

Study Questions:

What are the contemporary short-term results and factors associated with outcomes following repair of a complete atrioventricular septal defect (CAVSD)?

Methods:

This was a multicenter observational study of 120 patients from seven Pediatric Heart Network centers from June 2004 to 2006. Demographic, procedural, and outcome data were collected, and echocardiographic (ECHO) images were reviewed by a core laboratory. Follow-up ECHO assessment was performed at 1 and 6 months. Comparative ECHO studies were available for 83/120 patients.

Results:

Median age at repair was 3.7 months, and 80% had trisomy 21. Concurrent surgical procedures were performed in 48%, with ligation of a patent ductus being most common. Median ventilator days, intensive care unit stay, and hospital stay were 2, 4, and 8 days, respectively, and were independent of trisomy 21. Presence of an associated anomaly or younger age (<2.5 months of age) was associated with a longer hospital stay. The overall mortality was 4% (n = 5) with 2.5% (n = 3) in-hospital mortality all occurring in infants <2.5 months of age (p = 0.06). Postdischarge deaths were secondary to pulmonary hypertension. Complications were common, with 41% of patients having at least one. Residual shunts were common at 1 month and uncommon at 6 months. Moderate or greater left-sided atrioventricular valve regurgitation (LAVVR) was present in 24% at 1 month and 22% at 6 months. The strongest predictor of moderate or greater LAVVR at 1 month was a greater number of in-hospital complications. The greatest predictor at 6 months was presence of moderate or greater LAVVR at 1 month. The double patch technique (72%) was most commonly used, followed by single path (18%) and Australian technique (10%) (p = 0.002), with no difference in outcomes. The cleft was closed in 93% and was independent of repair type.

Conclusions:

This multicenter study reports similar short-term outcomes for CAVSD repair as has been reported in single-center studies. The presence of trisomy 21 did not adversely affect outcome. No significant differences in terms of residual shunts or degree of LAVVR were demonstrated between repair types. Repair at <2.5 months of age and need for concurrent surgical procedures were associated with prolonged hospital stay, but were not associated with residual defects.

Perspective:

Repair of CAVSD can be performed with reasonable morbidity and mortality regardless of surgical technique or the presence of trisomy 21. Repair can be performed in younger patients with equivalent outcomes, but a longer and more complex hospital course can be anticipated. Longer-term follow-up is necessary to determine the fate of the LAVVR and any potential association with repair technique.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Pulmonary Hypertension and Venous Thromboembolism, Valvular Heart Disease, Congenital Heart Disease, CHD & Pediatrics and Imaging, CHD & Pediatrics and Quality Improvement, Pulmonary Hypertension, Echocardiography/Ultrasound, Mitral Regurgitation

Keywords: Heart Diseases, Down Syndrome, Follow-Up Studies, Mitral Valve Insufficiency, Australia, Trisomy, Hypertension, Pulmonary, Echocardiography


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