Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors

J Pediatr. 2020 Apr;219:160-166.e2. doi: 10.1016/j.jpeds.2019.09.053. Epub 2019 Nov 5.


Objective: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH).

Study design: 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.

Results: 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).

Conclusions: 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.

Keywords: congenital diaphragmatic hernia; generalized estimating equations; generalized linear model; perfusion; ventilation.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Hernias, Diaphragmatic, Congenital / physiopathology*
  • Humans
  • Infant
  • Infant, Newborn
  • Longitudinal Studies
  • Lung / physiopathology*
  • Male
  • Retrospective Studies
  • Ventilation-Perfusion Ratio*
  • Young Adult