Contributions of residual hypoxemia to exercise hyperventilation in Fontan patients

It is unsettled whether increased exercise ventilation in Fontan subjects is due to increased pulmonary dead space or augmented ventilatory drive. Twenty-six Fontan patients underwent symptom-limited treadmill cardiopulmonary exercise testing. Two groups of age- and sex- matched subjects served as controls: the biventricularly repaired (Bi, n = 18), and the “true” control (C, n = 29) groups. Peak oxygen uptake (V̇O(2peak)) was not different among groups (41.0 +/– 8.4 ml/min/kg, 43.5 +/– 6.6 ml/min/kg, and 45.9 +/– 11.6 ml/min/kg for Fontan, Bi, and C groups, respectively, p = 0.16). Fontan subjects, however, showed steeper alveolar ventilation/carbon-dioxide (V̇A/V̇CO(2)) regression slope (35.5 +/– 5.3, 28.7 +/– 3.8, and 29.5 +/– 3.0 l/ml, for Fontan, Bi, and C groups, respectively, p<0.0001), and lower end-expiratory carbon-dioxide fraction (FetCO(2VAT)) at ventilatory threshold (VAT) (4.4 +/– 0.5%, 5.5 +/– 0.5%, and 5.5 +/– 0.4%, for Fontan, Bi, and C groups, respectively, p<0.001). The dead-space ventilation fraction at VAT was similar among groups (0.33 +/– 0.06, 0.33 +/– 0.04, 0.35 +/– 0.05 for Fontan, Bi, and C groups, respectively, p = 0.54). In Fontan subjects, arterial oxygen saturation at rest (SaO(2rest)) was correlated with V̇A/V̇CO(2) regression slope (r = –0.41, p = 0.04) and with FetCO(2VAT) (p = –0.53, p<0.01). We conclude that Fontan patients show exercise hyperventilation due to augmented central and/or peripheral ventilatory drive, which is further augmented by residual hypoxemia.