Older age at Fontan completion is associated with reduced lung volumes and increased lung reactance

Laohachai, Karina; Badal, Tanya; Thamrin, Cindy; Robinson, Paul D.; Kennedy, Brendan; Rice, Kathryn; Selvadurai, Hiran; Weintraub, Robert G.; Cordina, Rachael; d’Udekem, Yves; Ayer, Julian

doi:10.1016/j.ijcard.2022.06.037

Cited by 5 publications

(5 citation statements)

References 34 publications

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“…Physical activity might, therefore, have a more beneficial impact on lung function in Fontan patients than previously assumed. Previous studies have also reported associations between VO2 peak and FVC as well as FEV1 [ 6 , 13 ], which we did not find, aligning with the more recent study by Laohachai et al [ 10 ]. Laohachai et al, however, found an association between higher age at Fontan completion and abnormal lung volumes, which we did not find in this study.…”

Section: Discussionsupporting

confidence: 92%

“…The impaired lung volume could, in part, elucidate the observed decline in diffusion capacity among Fontan patients, as a reduced lung volume translates to a diminished surface area available for effective diffusion. This is in line with novel findings in one of the few studies on DLCO in Fontan patients, in which Laohachai et al concluded that the reduced DLCO in their patients was largely caused by a low VA [ 10 ].…”

Section: Discussionsupporting

confidence: 88%

“…Studies investigating lung function in Fontan patients are limited, but it seems evident that impaired lung function is common [ 10 , 11 , 13 , 14 , 28 ]. In our study, we observed a restrictive lung pattern and a severely reduced DLCO and DLNO in both PFT-I and PFT-II, consistent with previous reports [ 29 ].…”

Section: Discussionmentioning

confidence: 99%

“…An impaired DLCO is found in various studies on Fontan patients, along with different theories on its causes [ 10 , 11 , 13 , 28 ]. As mentioned by Laohachai et al in their 2022 study, the assumption of a reduced VA as a cause of the reduced DLCO is opposed to findings in the first Danish study on our patient cohort by Idorn et al from 2014, where the Vc seemed to be the primary cause of the reduced DLCO [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

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Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development?

Ravndal,

Idorn,

Nielsen

et al. 2024

Pediatr Cardiol

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Few studies have investigated how the Fontan circulation affects lung function, and no studies have investigated the development of lung function over longer time in these patients. We aimed to describe the development of lung function in Fontan patients over a 10-year period. Pulmonary function tests (PFT), including spirometry and diffusion capacity for Carbon Monoxide (DLCO) and Nitric Oxide (DLNO), were conducted in a Danish Fontan cohort in 2011 (PFT-I). In 2021, re-investigations were performed (PFT-II). We investigated changes in percent predicted (%pred) lung function from PFT-I to PFT-II. Patients were categorized into a pediatric group (age under 18 at PFT-I) and an adult group (age 18 or older at PFT-I). Out of the 81 patients completing PFT-I, 48 completed PFT-II. In the pediatric group (32 patients), there were significant declines in %pred forced expiratory volume in 1s (99.7 (92.4, 104.4)–89.3 (84.9, 97.2), p < 0,001), forced vital capacity (98.3 (87.8, 106.1)–96.7 (86.7, 100.6), p = 0.008), and alveolar volume (95.5 (89.5, 101.6)–89.5 (79.7, 93.2), p < 0.001). The corresponding measurements remained stable in the adult group. However, the median %pred DLNO significantly declined in the adult group (58.4 (53.3, 63.5)–53.7 (44.1, 57.3), p = 0.005). Over a 10-year period, several lung function parameters declined significantly in the younger Fontan patients, suggesting possible impairments in lung development during growth. The decline in %pred DLNO in the adult patient group indicates deterioration of the membrane component of diffusion capacity, implying that the Fontan circulation might negatively affect the alveolar membrane over time.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development?

Ravndal,

Idorn,

Nielsen

et al. 2024

Pediatr Cardiol

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“…Lung stiffness increases, affecting alveolar volume and capillary membrane function. These parameters have a negative correlation with Fontan completion age, implying that earlier Fontan completion may benefit lung function ( 25 ). We believe that early Fontan completion might preserve systemic ventricular function, prevent (or at least mitigate) the harmful consequences of the Fontan circulation, and provide better functional ability.…”

Section: Discussionmentioning

confidence: 99%

Pleural and mediastinal effusions after the extracardiac total cavopulmonary connection: Risk factors and impact on outcome

Heinisch

Metz

Staehler

et al. 2022

Front. Cardiovasc. Med.

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BackgroundThis study investigated the volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection, as well as preoperative risk factors and their impact on outcome.Materials and methodsA total of 210 patients who underwent extracardiac total cavopulmonary connection at our center between 2012 and 2020 were included in this study. Postoperative daily amount of pleural and mediastinal drainage were collected and factors influencing duration and amount of effusions were analyzed. The impact of effusions on adverse events was analyzed.ResultsMedian age at extracardiac total cavopulmonary connection was 2.2 (interquartile range, 1.8–2.7) years with median weight of 11.6 (10.7–13.0) kg. Overall duration of drainage after extracardiac total cavopulmonary connection was 9 (6–17) days. The total volume of mediastinal, right pleural, and left pleural drainage was 18.8 (11.9–36.7), 64.4 (27.4–125.9), and 13.6 (0.0–53.5) mL/kg, respectively. Hypoplastic left heart syndrome (p = 0.004) and end-diastolic pressure (p = 0.044) were associated with high volume of drainages, and hypoplastic left heart syndrome (p = 0.007), presence of aortopulmonary collaterals (p = 0.002), and high end-diastolic pressure (p = 0.023) were associated with long duration of drainages. Dextrocardia was associated with higher volume (p < 0.001) and longer duration (p = 0.006) of left pleural drainage. Duration of drainage was associated with adverse events following extracardiac total cavopulmonary connection (p = 0.015).ConclusionVolume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection were related with hypoplastic left heart syndrome, aortopulmonary collaterals, and end-diastolic pressure. The duration of drainage for effusions was a risk factor for adverse events after total cavopulmonary connection.

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Deriving fontan-specific normative exercise data from well-functioning adolescents

Laohachai,

Webb,

Cordina

et al. 2024

International Journal of Cardiology Congenital Heart Disease

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Older age at Fontan completion is associated with reduced lung volumes and increased lung reactance

Cited by 5 publications

References 34 publications

Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development?

Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development?

Pleural and mediastinal effusions after the extracardiac total cavopulmonary connection: Risk factors and impact on outcome

Deriving fontan-specific normative exercise data from well-functioning adolescents

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