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The ideal timing and optimal management of surgical repair for isolated complete atrioventricular septal defect (CAVSD) still remains controversial. To determine outcomes after the surgical repair of CAVSD, we reviewed 100 consecutive patients who underwent complete repair at our institute between January 1992 and August 2003. Among these 100 patients, 52 were female and 73 had Down's syndrome. Twelve had received preceding pulmonary artery banding. A two-patch repair was employed in all cases. The patients' median age and weight were 4.5 (1.2-48) months and 4.7 (2.5-12.5) kg, respectively. The mean stays in the intensive care unit and in the hospital were 5.3+/-3.8 and 25.4+/-18.1 days, respectively. The median duration of mechanical ventilation was 11.6 h. There were two in-hospital deaths, in patients 5.2 and 5.9 months of age. Both had underlying Down's syndrome, and significant pulmonary vascular obstructive disease (PVOD) was detected on postmortem specimen. The operative outcome of CAVSD was generally satisfactory. PVOD can progress rapidly from four to five months, especially with Down's syndrome. Therefore, in order to avoid progression to irreversible PVOD, surgical intervention within four months of birth may be appropriate in such patients.

Ideal timing of surgical repair of isolated complete atrioventricular septal defect