Work capacity and central hemodynamics thirteen to twenty-six years after repair of tetralogy of Fallot.

Exercise tests and cardiac catheterization were performed in 53 patients, 13 to 26 years after intracardiac repair of tetralogy of Fallot. At the time of repair, the median age was 7 years, and 60% of patients with cyanosis had had a previous palliative procedure. The right ventriculotomy was closed without a patch in 21 patients (40%), a patch restricted to the right ventricle was inserted in 18 patients (34%), and in 14 (26%) the patch extended across the pulmonary anulus. At follow-up, 94% of the patients were free of symptoms. Symptom-limited work capacity was 87% of the predicted value (95% confidence limits, 82% to 94%). Work capacity was inversely related to age at follow-up, to right ventricular systolic pressure at rest, and to presence of moderate or severe pulmonary valve regurgitation. Cardiac output in relation to oxygen uptake was reduced in 74% of patients during exercise. In 12 patients (23%), systolic pressure at rest in the right ventricle was 50 mm Hg or higher. Systolic pressure during exercise in the right ventricle was lower in patients without a patch than in those with a patch and was abnormally high in all groups compared with healthy subjects. The ratio of right to left ventricular pressure was significantly lower than measurements taken immediately after repair. An intracardiac left-to-right shunt was present in 6 patients (11%). Three patients required invasive treatment as a result of our follow-up. We conclude that work capacity was moderately reduced 13 to 26 years after repair of tetralogy of Fallot and was adversely influenced by right ventricular hypertension and pulmonary valve regurgitation. Intermittent lifelong surveillance is advocated, because patients without symptoms may have hemodynamic abnormalities that necessitate intervention.