Long-Term Follow-Up of 133 Atrioventricular Septal Defects

Abstract

From 1962 to 1986 we operated on 133 patients with atrioventricular (AV) septal defect. Median follow-up time was 8 years. Of these 133, 90 had separate AV orifices and 43 had a common AV orifice. Primary complete repair was done in 129, pulmonary artery banding was done in three, of whom two underwent subsequent repair, and one patient with a concomitant severe pulmonary stenosis received a shunt. Total (operative and late) mortality was 23 patients [17%, 70% confidence limits (CL): 14%–22%]. Left AV valve replacement was done in eight patients (6%; CL: 4%–9%), never at the primary correction; of these, four patients died (50%; CL: 27%–73%). Logistic regression analysis showed (a) common AV orifice; (b) inability to repair the left AV valve adequately; and (c) major associated anomalies to be incremental risk factors for death. Late follow-up was obtained in all patients. Doppler echocardiographic investigation — more than 1 year after correction — of the left AV valve was obtained in 78 of the 110 survivors. The Doppler severity of regurgitation was semiquantified and the left atrium (LA)/aorta (Ao) ratio was measured. No or little regurgitation was found in 66 patients (85%; CL: 79%–89%), moderate regurgitation in ten patients (13%; CL: 9%–18%), while severe regurgitation was found in only two patients (3%; CL: 1%–6%). There was no difference in regurgitation or LA/Ao ratio between patients with common or separate AV orifices. Our conclusion is that most survivors show some regurgitation, but LA/Ao ratio is usually in the upper range of normal. Most patients are able to lead a normal life without physical limitations. Residual severe left AV valve regurgitation remains a risk factor that should be avoided at all costs. This is even more compelling because of the high mortality associated with late left AV valve replacement.