J Pediatr. 2019 Nov 5. pii: S0022-3476(19)31219-3. doi: 10.1016/j.jpeds.2019.09.053. [Epub ahead of print]
Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors.
To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH).
This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model.
During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045).
In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.
Copyright © 2019 Elsevier Inc. All rights reserved.
congenital diaphragmatic hernia; generalized estimating equations; generalized linear model; perfusion; ventilationPMID: 31704054 DOI: 10.1016/j.jpeds.2019.09.053