PULMONARY-FUNCTION 13 TO 26 YEARS AFTER REPAIR OF TETRALOGY OF FALLOT

Lung function was evaluated in 68 patients 13 to 26 (median 19) years after repair of tetralogy of Fallot. Age at repair was 7 years (9 mont hs to 42 years) and 51% had a palliative shunt. An outflow patch was i nserted in 56%. Median vital capacity was 84% of predicted, forced exp iratory volume in 1 second 83%, maximal voluntary ventilation at 40 br eaths/min 70%, and diffusing capacity for carbon monoxide 77% of predi cted. Scintigraphy demonstrated abnormal pulmonary perfusion in 86%. A verage right lung perfusion was 57% (predicted 52%). Regional hypoperf usion could in some patients be explained by previous palliative shunt , pulmonary artery obstruction, or presence of aortopulmonary collater als. Median symptom-limited work capacity was 82% (95% confidence limi ts 78% to 90%) of predicted. Twenty-eight physically active patients h ad high values for symptom-limited work capacity, vital capacity, forc ed expiratory volume in 1 second, and maximal voluntary ventilation at 40 breaths/min compared with those of inactive patients. Lung functio n variables were related to physical exercise and previous palliative shunt. Moderate or severe pulmonary valve incompetence had negative bu t not significant influence on lung function. There was no significant influence of acyanosis before repair, use of transannular patch, dura tion of follow-up, or smoking. We found moderately reduced work capaci ty and lung function late after repair of tetralogy of Fallot that did not cause symptoms. Lung function variables were high in young active male patients and low in patients with previous palliative shunt. A b etter lung function in active patients indicates that physical activit y should be encouraged after repair of tetralogy of Fallot.