Tetralogy of Fallot: selective staged management.

One hundred twenty-four patients with tetralogy of Fallot have undergone either primary total repair (61), shunt and later repair (30), or an initial shunt (33). The mean ratio of pulmonary anulus to descending thoracic aorta increased from 0.80 +/- 0.25 before the shunt to 1.22 +/- 0.26 before the repair (p less than 0.0001). The mean ratio in the primary repair group was 1.23 +/- 0.25. A transannular patch was necessary in only six of 91 patients (6.6%). Postrepair right ventricular/left ventricular pressure ratio averaged 0.50 +/- 0.11 in the shunt plus repair group and 0.43 +/- 0.12 in the primary repair group. Only four patients had a right ventricular/left ventricular pressure ratio less than 0.65. A significant inverse linear relationship existed between this ratio and the pulmonary anulus size measured at operation and normalized for the patient's height (p less than 0.01). Postoperative complications occurred in 21% of patients after a shunt and 20% of patients after open heart repair. The early mortality was 0.8% (1/124). An initial shunt in patients with a small pulmonary anulus can result in an increased anulus size and better hemodynamic result with frequent avoidance of a transannular patch. Staged repair may result in improved overall mortality rates.