A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

Aim The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. Methods This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assessment (SF‐36 questionnaire) were performed between 2003 and 2015. Results Overall, mean forced expiratory volume in one second (FEV1) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV1 was 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV1 was associated with a reduced peakVO2 of 67.0 ± 17.6%pred. (r = 0.43, P