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Original Articles: Pediatric Cardiac

Partial and Transitional Atrioventricular Septal Defect Outcomes

^a University of Utah, Salt Lake City, Utah
^b Medical University of South Carolina, Charleston, South Carolina
^c New England Research Institutes, Watertown, Massachusetts
^d The Hospital for Sick Children, Toronto, Ontario, Canada
^e Duke University Medical Center, Durham, North Carolina
^f Children's Hospital Boston, Boston, Massachusetts
^g Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Accepted for publication October 21, 2009.

^_* Address correspondence to Dr Minich, 100 N Mario Capecchi Dr, Salt Lake City, UT 84113 (Email: luann.minich{at}imail.org).

Background: Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.

Methods: We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.

Results: Median age at surgery was 1.8 years; median weight z-score was –0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt.

Conclusions: Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.