Factors Associated with Early-Childhood Ipsilateral Perfusion Abnormalities among Patients with Congenital Diaphragmatic Hernia

Abstract

Objectives: To evaluate long-term, postdischarge, ipsilateral lung perfusion patterns and to identify associated factors among patients with congenital diaphragmatic hernia (CDH).

Study design: A long-term follow-up database was created to assimilate data across 4 centers, covering patients with left-sided CDH managed as outpatients between 2010 and 2021. Outcomes from discharge to age 5, focusing on ipsilateral lung perfusion at 2 and 5 years, were analyzed using both univariable and multivariable generalized linear modeling.

Results: Among the 258 patients with left-sided CDH, 213 (83%) and 100 (39%) had 2- and 5-year follow-up data, respectively. Of these, 173 patients (68%) had low-risk (A/B) defects. At discharge, 58 (22.5%) needed supplemental oxygen and 56 (21.7%) had pulmonary hypertension on echocardiogram. Perfusion data were available for 121 patients (47%) at 2 years and 54 (21%) at 5 years. Compared with the normal left lung perfusion index of 45%-50% at 2 years of age, patients with CDH had abnormal ipsilateral perfusion deficits, with a median of 37.0% for the cohort, and a median ipsilateral perfusion of 39.0% for low-risk defects and 31.0% for high-risk defects, an absolute difference of 8.0 (P < .001). In the longitudinal multivariable analysis, only ipsilateral perfusion at 2 years (P < .001) and CDH defect stage (P = .025) were associated significantly with ipsilateral perfusion at 5 years.

Conclusions: All patients with CDH had abnormal ipsilateral perfusion at 2 and 5 years, with defect size as the only factor associated with perfusion. Pulmonary hypertension was not associated with perfusion. Further clinical correlation is needed. These findings may help when counseling families on long-term outcomes.

Keywords: CDH Study Group; CDH repair; center volume; congenital diaphragmatic hernia; diaphragm repair; outcomes; registry.