A 20-year review of ostium primum defect repair in children.

Between July, 1963, and July, 1983, a total of 69 patients (35 boys and 34 girls) underwent ostium primum defect repair. There were four perioperative deaths and four patients were subsequently lost to follow-up, leaving 61 children followed for 6 months to 20 years (mean 5 years). Results of surgery were assessed by cardiac catheterization in 17 of 61 patients, while the remaining patients were evaluated noninvasively. Postoperative mitral insufficiency was found to be absent in 19 patients, mild to trivial in 35, moderate in four, and severe in two. Four patients were found to have large residual atrial septal defects. Significant late postoperative arrhythmias were found in 14 of 61 patients. The types of arrhythmias included isolated complete atrioventricular block in 5 of 14, complete atrioventricular block with sinus node dysfunction in 2 of 14, and isolated sinus node dysfunction in 7 of 14. Pacemakers have been implanted in 8 of 14 of these patients. Based on this 20-year review of a large number of children: (1) ostium primum defect repair is associated with a low mortality rate, (2) residual mitral insufficiency although common is usually mild to trivial and nonprogressive, and (3) significant arrhythmias are a frequent complication and often require pacemaker implantation.