Ventilatory response to exercise in patients with major aortopulmonary collateral arteries after definitive surgery

Patients with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) may be at risk for both ventilatory impairment and abnormal pulmonary circulation after definitive surgery. We measured the ventilatory response to exercise in 16 patients with MAPCAs after definitive surgery (group A) and compared the results with those in 16 patients with tetralogy of Fallot with pulmonary atresia and without MAPCAs after definitive operation (group B), with 24 patients with tetralogy of Fallot after one-stage repair without previous palliation (group C), and with 48 healthy subjects (group D). Pulmonary function and treadmill exercise tests were performed. Arterial blood gases were also analyzed and the dead space to tidal volume ratio calculated. In group A, the vital capacity, diffusion capacity, and peak oxygen uptake were lowest (p <0.001), whereas the ventilatory equivalent for carbon dioxide was highest and its value at peak exercise correlated with age at time of surgery (r = 0.73, p <0.002). The arterial oxygen tension decreased progressively in group A and its value at peak exercise inversely correlated with the mean pulmonary artery pressure in all patients (r = -0.75, p <0.001). The arterial carbon dioxide tension decreased significantly at peak exercise in controls but showed no change in group A. The dead space to tidal volume ratio decreased during exercise in patients without MAPCAs and in controls but increased in group A, and the dead space to tidal volume ratio at peak exercise was inversely correlated with vital capacity in all patients (r = -0.77, p <0.001). Diffusion capacity independently predicted arterial carbon dioxide tension and dead space ventilation during exercise. Marked restrictive ventilatory impairment with low diffusion capacity along with a pulmonary obstructive change contributed to the abnormal pulmonary gas exchange during exercise in group A. Earlier repair of MAPCAs may prevent the progression of the impaired ventilatory response to exercise in these patients.