EXERCISE PERFORMANCE FOLLOWING REPAIR OF HYPOPLASTIC LEFT-HEART SYNDROME - A COMPARISON WITH OTHER TYPES OF FONTAN PATIENTS

Reports of exercise performance after Fontan surgery for hypoplastic l eft heart syndrome (HLHS) are lacking. We compared the exercise perfor mance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n = 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n = 6) or a systemic left ventricle (group 3, n = 8). The subjects underwent assessment of rest ing pulmonary mechanics followed by maximal exercise testing with a bi cycle or treadmill protocol. EGG, oxygen consumption, and carbon dioxi de production were measured continuously, There was not a significant difference seen between HLHS and the comparison groups for the followi ng parameters: maximum heart rate, maximum oxygen consumption, respira tory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise, Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgic al approach.