Ventilatory response in patients with corrected tetraology of Fallot with major aortopulmonary collateral arteries

AIM: To investigate the lung function and cardiopulmonary exercise (CPX) performance in patients with corrected tetralogy of Fallot with major aortopulmonary collateral arteries. METHODS: Routine lung function and CPX tests were performed in 23 patients with tetralogy of Fallot with major aortopulmonary collateral arteries (group A), 30 patients with tetralogy of Fallot without major aortopulmonary collateral arteries (group B), and 37 patients with ventricular septal defect or atrial septal defect (group C). The study was performed 1 year after corrective heart surgery. RESULTS: No significant differences were observed in FEV1, FEV1%pred, MVV, and MVV%pred among group A, group B and group C. FVC and FVC%pred decreased significantly in group A compared with those in group B and group C (P<0.01). In group A, FEV1/FVC% was the highest (P<0.01). CPX showed that WR max, MV at peak exercise, RQ, VO2 max/kg, AT and VO2/HRmax in group A were the lowest (P<0.01), whereas VE/VCO2 at peak exercise was the highest (P<0.01). CONCLUSION: There is marked restrictive ventilatory impairment in patients with tetralogy of Fallot with major aortopulmonary collateral arteries. Early repair of major aortic pulmonary collateral arteries may prevent progression of the impaired ventilatory response to exercise in these patients.