Pulmonary Restriction in Patients With a Fontan Circulation: DETERMINANTS, DETERIORATION OVER TIME, AND CLINICAL IMPLICATIONS

Pulmonary restriction is prevalent in patients with a Fontan circulation. Multiple thoracotomies and a longer time since Fontan completion predict the development of pulmonary restriction. The FVC, not TLC, is associated with lower exercise tolerance. This emphasizes the importance of muscle-dependent pulmonary ventilation. Finally, FVC z scores are decreased in adolescence and deteriorate faster than in the general population.