TY - JOUR

T1 - Lung Function in Fontan Patients Over a Ten-Year Period

T2 - Is the Fontan Circulation Impairing Lung Development?

AU - Ravndal, Maren

AU - Idorn, Lars

AU - Nielsen, Kim Gjerum

AU - Hjortdal, Vibeke

N1 - Publisher Copyright: © 2024, The Author(s).

PY - 2024

Y1 - 2024

N2 - 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.

AB - 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.

KW - Cohort study

KW - Congenital heart disease

KW - Fontan circulation

KW - Lung development

KW - Lung function

KW - Pulmonary function test

U2 - 10.1007/s00246-023-03389-2

DO - 10.1007/s00246-023-03389-2

M3 - Journal article

C2 - 38273050

AN - SCOPUS:85183038697

VL - 45

SP - 500

EP - 512

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

ER -