Prognostic utility of noninvasive estimates of pulmonary vascular compliance in neonates with congenital diaphragmatic hernia.

Aim: To evaluate echocardiographic indices of pulmonary vascular resistance and right ventricular (RV) function in predicting death or ECMO in congenital diaphragmatic hernia (CDH).

Methods: In this single center study, early (<48 h) echocardiograms of neonates with CDH (n = 47) were reviewed by a single reader for the ratio of tricuspid regurgitant velocity to velocity time integral at the pulmonary valve (TRV/VTIpv), TRV2/VTIpv, RV fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE). Receiver operating characteristic curves were generated for each parameter to obtain optimal cutoff values.

Results: Infants who died or received ECMO (n = 30) had significantly higher TRV/VTIpv and TRV2/VTIpv, and lower VTIpv, RV FAC and TAPSE, compared to the 17 survivors without ECMO. For TRV2/VTIpv and TRV/VTI at cutoffs of 1.3 and 0.38 respectively, the sensitivities were 92.3 (95% CI 75-99.1%) and 96 (95% CI 80.4-99.9%), specificities were 78.6 (49.2-95.4%) and 71.4 (42-91.6%), positive predictive values were 89 (75-95.6%) and 86.2 (73.2-93.5%) and negative predictive values were 84.6 (58.5-95.5%) and 90.9 (58.7-98.6%).

Conclusion: Early echocardiographic evidence of pulmonary hypertension and RV dysfunction predicts outcome in CDH. Further studies utilizing these indices to guide therapeutic decision-making are warranted.

Level Of Evidence: Level 1 for diagnostic study.