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J Thorac Cardiovasc Surg 2007;133:1533-1539
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

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

^a Division of Cardiology, The Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa
^b Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa
^c Division of Biostatistics and Epidemiology, The Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa
^d Division of Neurology, The Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa
^e Division of Psychology, The Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pa
^f Division of Cardiothoracic Surgery, Emory University, Atlanta, Ga
^g Department of Kinesiology, Temple University, Philadelphia, Pa.

Received for publication October 18, 2006; accepted for publication December 13, 2006.

^_* Address for reprints: Michael G. McBride, PhD, Division of Cardiology, Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104. (Email: mcbride{at}email.chop.edu).

Objectives: We evaluated cardiopulmonary function at rest and during exercise in children after surgical repair for total anomalous pulmonary venous connection.

Background: Long-term assessment of cardiopulmonary function during exercise in children after repair for total anomalous pulmonary venous connection during infancy is limited.

Methods: 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.

Results: 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).

Conclusions: 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.