[Surgical treatment of the tetralogy of Fallot with a single pulmonary artery].

Eleven patients with Tetralogy of Fallot and congenital or acquired absence of one pulmonary artery (PA) have been operated upon at the Department of Cardiac Surgery of Ospedali Riuniti-Bergamo-Italy. Eight children had agenesis of the left PA, one of the right PA and two had complete occlusion of the right PA after a palliative operation. Four patients had only a palliative procedure (one Blalock-Taussig, two Waterston, three PTFE shunts) with one operative death. Two patients underwent a two-staged procedure, that is an initial shunt (one Blalock and one PTFE shunt) followed by subsequent total correction. Five patients underwent primary total correction. No operative death occurred among the patients who had total correction. Right ventricular outflow reconstruction has been accomplished with an infundibular patch in one patient, an external valved conduit in two patients, and a transanular patch plus orthotopic bioprosthesis in 4 patients. One patient with a valved conduit died one year postoperatively in right heart failure caused by pulmonary hypertension. The pulmonary vascular disease was probably due to pulmonary microembolization following the severe hemolysis that lasted for twenty days in the early postoperative period. The nine surgical survivors are in good general condition with maximum follow-up of 7 years. The surgical approach either in case of palliation or in case of corrective surgery is finally discussed. In our experience TOF with unilateral absence of a PA is a congenital anomaly that can be corrected with results comparable to those of uncomplicated TOF.