Late cardiopulmonary and musculoskeletal exercise performance after repair for total anomalous pulmonary venous connection during infancy

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We evaluated cardiopulmonary function at rest and during exercise in children after surgical repair for total anomalous pulmonary venous connection. Long-term assessment of cardiopulmonary function during exercise in children after repair for total anomalous pulmonary venous connection during infancy is limited. Resting lung function and cardiopulmonary function during maximal ramp cycle ergometry were evaluated in 27 patients (age = 11 +/- 4 years, 20 were male). Peak oxygen consumption, ventilatory anaerobic threshold, and physical working capacity were compared with normal reference values. Neurologic assessment included neuromuscular function, inattentiveness, and hyperactivity. Patient- and procedure-related variables were assessed for association with peak oxygen consumption, ventilatory anaerobic threshold, and physical working capacity. Compared with healthy children, peak oxygen consumption (88% +/- 16% of predicted) and ventilatory anaerobic threshold (91% +/- 21% of predicted) were mildly reduced. Chronotropic impairment was observed in 7 patients (32%). Patients with impaired resting lung mechanics were more likely to have impairment in peak oxygen consumption (P < .05). Breathing reserve was normal. Specific anatomy and all operative factors did not have a significant impact on overall exercise performance. Composite score for fine and gross motor function was associated with lower ventilatory anaerobic threshold (P < .05). Exercise performance is mildly impaired at long-term follow-up after total anomalous pulmonary venous connection repair during infancy. Residual pulmonary abnormalities are common and associated with lower exercise performance. Neurologic abnormalities are evident in a subgroup, but the impact on late exercise performance is inconclusive.