Alessia Callegari1,2,4, Rhoia Neidenbach2, Ornella Milanesi1, Biagio Castaldi1, Martin Christmann4, Masamichi Ono3, Jan Müller2, Peter Ewert2, Alfred Hager2
Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694
Abstract 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 qual‐
ity 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 connec‐
tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐
ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced expiratory volume… More >
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