Early, Postnatal Pulmonary Hypertension Severity Predicts Inpatient Outcomes in Congenital Diaphragmatic Hernia.

INTRODUCTION

Pulmonary hypertension (PH) is the major pathophysiologic consequence of congenital diaphragmatic hernia (CDH). We aimed to evaluate the association between early CDH-associated PH (CDH-PH) and inpatient outcomes.

METHODS

The CDH Study Group registry was queried for infants born 2015-2019 with echocardiograms before 48h of life. PH was categorized using echocardiographic findings: none, mild (right ventricular systolic pressure <2/3 systemic), moderate (between 2/3 systemic and systemic), or severe (supra-systemic). Univariate and multivariate analyses were performed. Adjusted Poisson regression was used to assess the primary composite outcome of mortality or oxygen support at 30 days.

RESULTS

Of 1,472 patients, 86.5% had CDH-PH: 13.9% mild (n = 193), 44.4% moderate (n = 631), and 33.2% severe (n = 468). On adjusted analysis, the primary outcome of mortality or oxygen support at 30 days occurred more frequently in infants with moderate (incidence rate ratio [IRR] 1.8, 95% confidence interval [CI], 1.2-2.6) and severe CDH-PH (IRR 2.0, 95% CI, 1.3-2.9). Extracorporeal life support (ECLS) utilization was associated only with severe CDH-PH after adjustment (IRR 1.8, 95% CI, 1.0-3.3).

DISCUSSION/

CONCLUSION

Early, postnatal CDH-PH is independently associated with increased risk for mortality or oxygen support at 30 days and utilization of ECLS. Early echocardiogram is a valuable prognostic tool for early, inpatient outcomes in neonates with CDH.