Management

Definitive treatment is surgical, with closure of the atrial and ventricular septal defects with a patch and reconstruction of the common atrioventricular valve to form competent mitral and tricuspid valve equivalents.

There is a high mortality with this procedure, especially if the pulmonary vascular resistance is high. If the procedure is completed successfully then there is a significant risk of residual haemodynamic defect.

The mortality is falling in some centres, especially when surgery is performed early. The main difficulty is the choice given to the parents; the child has a condition, usually relatively symptomless when diagnosed but which is invariably fatal in the third decade, compared to surgery with a major risk of mortality.