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Erschienen in: Pediatric Cardiology 5/2017

12.05.2017 | Original Article

Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients

verfasst von: Aída L. R. Turquetto, Luiz F. Canêo, Daniela R. Agostinho, Patrícia A. Oliveira, Maria Isabel C. S. Lopes, Patrícia F. Trevizan, Frederico L. A. Fernandes, Maria A. Binotto, Gabriela Liberato, Glaucia M. P. Tavares, Rodolfo A. Neirotti, Marcelo B. Jatene

Erschienen in: Pediatric Cardiology | Ausgabe 5/2017

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Abstract

Central factors negatively affect the functional capacity of Fontan patients (FP), but “non-cardiac” factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8–17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO2 expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO2 Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02–97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO2 [FVC (r = 0.86, p < 0.001); FEV1 (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [−79(±28) vs −109(±44) cmH2O (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO2 with the FVC, FEV1, TLC, and DLCO measured by complete pulmonary test.
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Metadaten
Titel
Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients
verfasst von
Aída L. R. Turquetto
Luiz F. Canêo
Daniela R. Agostinho
Patrícia A. Oliveira
Maria Isabel C. S. Lopes
Patrícia F. Trevizan
Frederico L. A. Fernandes
Maria A. Binotto
Gabriela Liberato
Glaucia M. P. Tavares
Rodolfo A. Neirotti
Marcelo B. Jatene
Publikationsdatum
12.05.2017
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 5/2017
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-017-1606-9

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