Pulmonary hypertension in congenital diaphragmatic hernia patients: Prognostic markers and long-term outcomes

Prenatal observed/expected lung–to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients.

Methods

A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5 years of age.

Results

Of 153 newborns, 123 survived (80.4%), 58 (37.9%) had prenatal O/E LHR, and 42 (27.5%) had postnatal ECHO results. High mortality risk neonates (O/E LHR ≤ 45%) correlated with higher right ventricular systolic pressure (RVsp) at birth. Generally PH resolved by age 5 years. LPS results did not change over time (p > 0.05) regardless of initial PH severity, suggesting that PH resolution did not correlate with increased ipsilateral lung perfusion to offload the right ventricle.

Conclusion

Prenatal prognostic markers correlated with initial PH severity for CDH newborns, but PH resolved over time despite fixed perfusion bias to the lungs. These results suggest favorable PH outcomes for CDH patients who survive beyond infancy.

Type of Study

Retrospective Cohort Study.

Level of Evidence

3b