Early echocardiographic hemodynamic phenotypes and clinical outcomes in congenital diaphragmatic hernia
In brief
Early echo's left-heart phenotype had 65% ECMO or death in infants with CDH
In this 66-infant cohort, ECMO or death occurred in 65% of infants with left-ventricle-involved pulmonary hypertension, versus 27% with right-ventricle-predominant pulmonary hypertension and none with no or mild pulmonary hypertension. Greater phenotype severity remained associated with poor outcomes after accounting for prenatal lung size, but the left- versus right-ventricle comparison was no longer significant after adjustment, so added prognostic value needs validation.
- Journal
- European journal of pediatrics (Q1)
- Published
- 28 October 2026
- Study design
- Retrospective cohort
- Evidence level
- Level 3, Low (CEBM 3b)
- Authors
- Kyusang Yoo, Soo Hyun Kim, Juhee Park, Tae-Gyeong Kim, Jung Il Kwak, Jeong Min Lee, et al.
- PMID
- 42955372
- DOI
- 10.1007/s00431-026-07559-1
Why clinicians should know about it
- Picked for Pediatric Surgery (paper of the day, 30 October 2026): Neonatal congenital diaphragmatic hernia prognostic phenotypes
Abstract
UNLABELLED: Early postnatal hemodynamic adaptation may contribute to outcome heterogeneity in congenital diaphragmatic hernia (CDH). We evaluated whether echocardiography-defined hemodynamic phenotypes within 24 h of birth were associated with cardiorespiratory severity and extracorporeal membrane oxygenation (ECMO) or death. This retrospective cohort included infants with CDH admitted to a tertiary neonatal intensive care unit from 2021 to 2025 who underwent echocardiography within 24 h. Phenotypes were classified as no/mild pulmonary hypertension (PH), right ventricular (RV)-predominant PH, or left ventricular (LV)-involved PH based on ventricular function and ductal/atrial shunt direction. Associations with prenatal severity, respiratory indices, and ECMO or death were assessed. Among 66 infants, 21 had no/mild PH, 22 RV-predominant PH, and 23 LV-involved PH. ECMO or death occurred in 0%, 27.3%, and 65.2%, respectively (P < 0.001). Compared with RV-predominant PH, LV-involved PH was associated with greater respiratory severity and more frequent LV dysfunction; observed-to-expected lung-to-head ratio (O/E LHR) did not differ significantly between these two groups. Ordinal phenotype severity remained associated with ECMO or death after adjustment for O/E LHR (odds ratio (OR), 5.98; 95% confidence interval (CI), 1.76-20.31; P = 0.004). However, the comparison between LV-involved and RV-predominant PH was no longer statistically significant after adjustment for O/E LHR (OR, 3.42; 95% CI, 0.87-15.10). CONCLUSION: Early hemodynamic phenotype was associated with cardiorespiratory severity and ECMO or death in CDH. LV-involved PH identified a high-risk phenotype and may complement prenatal anatomic risk stratification, although its independent prognostic value beyond prenatal lung size requires validation. WHAT IS KNOWN: • Prenatal lung size and liver herniation are established markers of disease severity and outcome in congenital diaphragmatic hernia (CDH). • Pulmonary hypertension and ventricular dysfunction contribute substantially to early cardiorespiratory instability and adverse outcomes in infants with CDH. WHAT IS NEW: • Early echocardiographic phenotypes were associated with cardiorespiratory severity and ECMO or death. • LV-involved PH identified a high-risk phenotype, although its prognostic value beyond prenatal severity remains uncertain.
Abstract as published, via PubMed.
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