One-stage repair of Fallot's tetralogy is a satisfactory operative procedure with an acceptable mortality, provided the operation is undertaken by a team who are skilled in managing this heart lesion (Kirklin et al., 1959;Barnard and Schrire, 1961;Malm et al., 1963). The clinical results in the survivors are rewarding because the change in status is remarkable. Several recent reports on the haemodynamics have emphasized the clinical well-being of the patients. The present study set out to confirm this clinical impression, to assess the long-term improvement in the anatomy of the outflow tract, and to study the response of the heart and pulmonary circulation to exercise.
PATIENTS AND METHODSEleven patients with the most severe pre-operative disability who had undergone a one-stage repair operation of Fallot's tetralogy were selected from a group of 34 long-term survivors. They were cyanosed before operation with clubbing of the fingers. Three were having recurrent episodes of extreme cyanosis. They were small (below the 25th percentile of height and weight for age). The right ventricle was dominant with a short systolic ejection murmur in the pulmonary area and a loud, apparently single second sound in the aortic area. Electrocardiography, chest radiograph, cardiac catheterization, and selective angiocardiography confirmed the presence of severe cyanotic Fallot's tetralogy, with marked obstruction to the outflow tract of the right ventricle and a large right-to-left shunt through the ventricular septal defect.Pulmonary valvotomy and infundibular resection was performed in all the patients: in 10 an outflow tract prosthesis was inserted, and in 7 it was carried across the pulmonary valve ring into the pulmonary artery. Two patients had such severe outflow tract obstruction that there was only a probe patent communication between the body of the right ventricle and the pulmonary artery. The ventricular septal defect was closed by Received April 26, 1965. * Present address: The Cardiac Clinic, Groote Schuur Hospital Observatory, Cape, South Africa. 448 direct suture in 6 and with a teflon patch in 5. Six patients in whom the prosthesis was extended into the pulmonary artery had severe residual pulmonary incompetence.The patients were all well, living a normal life, and with a near normal exercise tolerance. They were able to run up four flights of stairs, a vertical height of 60 ft. (18-3 m.), without becoming short of breath. The finger clubbing disappeared.In the presence of pulmonary incompetence, the right ventricle was hyperdynamic with a long systolic ejection murmur heard best in the pulmonary area, a single second sound of aortic valve closure, and a diamondshaped early diastolic murmur which was delayed 0 04 sec. after aortic valve closure. Complete right bundle-branch block was present in all the patients (Fig. 1). The pulmonary vascularity had become normal but the heart size had increased since operation. A convex left middle segment was present in patients with an outflow tract prosthesis (Fig. 2). Pul...