@Article{chd.12694,
AUTHOR = {Alessia Callegari, Rhoia Neidenbach, Ornella Milanesi, Biagio Castaldi, Martin Christmann, Masamichi Ono, Jan Müller, Peter Ewert, Alfred Hager},
TITLE = {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},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {2},
PAGES = {147--155},
URL = {http://www.techscience.com/schd/v14n2/38761},
ISSN = {3071-1738},
ABSTRACT = {<b>Aim:</b> 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.<br/>
<b>Methods:</b> 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.<br/>
<b>Results:</b> Overall, mean forced expiratory volume in one second (FEV<sub>1</sub>) was
74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV<sub>1</sub> was <80%pred., and all
of these patients had FEV<sub>1</sub>/forced vital capacity (FVC) > 80%, suggestive of a restric‐
tive ventilatory pattern. Reduced FEV<sub>1</sub> was associated with a reduced peakVO<sub>2</sub> of
67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible con‐
founding factors (sex, BMI, age, years after palliation, number of interventions, sco‐
liosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV<sub>1</sub> was 
associated to quality of life in terms of physical component summary (r = 0.30, 
P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and
general health (r = 0.16, P = .024). Lower FEV<sub>1</sub> was associated with diaphragmatic
paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002),
and lower BMI (P = .01). No correlation was found to ventricular morphology, type of
surgeries, or other perioperative/long‐term complications.<br/>
<b>Conclusions:</b> This study shows that the common restrictive ventilatory pattern in
Fontan patients is associated with lower exercise capacity and quality of life. Risk
factors are diaphragmatic paralysis, scoliosis, a high total number of interventions
and low BMI.},
DOI = {10.1111/chd.12694}
}