Objective: Mortality associated with congenital diaphragmatic hernia (CDH) remains high, despite improvements in management. The aim of this study was to identify echocardiographic predictors of outcome in newborns with an isolated CDH.

Methods: We reviewed medical charts and echocardiograms of 40 newborns who presented with CDH in the first 24 hours of life, from 1992 to 1996. We compared survivors and nonsurvivors' cardiac valves and great arteries diameters, left-ventricular volume and mass, Apgar scores, and modified McGoon index (the combined diameter of hilar pulmonary arteries, indexed to the diameter of the descending aorta).

Results: Fourteen patients died 1-33 days after birth (median 3 days), including 8 from progressive hypoxemia without operation; 26 have survived up to 5 years (median 2 y) after successful operations. Nonsurvivors had significantly smaller diameters of right (3.8±0.7 mm vs. 4.9±0.4 mm in survivors; p<.05) and left (3.2±0.6 mm vs. 4.0±0.7 mm; p<.05) hilar pulmonary arteries, more frequent right-sided CDH (36% vs. 4%; p<.007), and lower Apgar scores at 1(3.4±1.7 vs. 5.2±2.2; p<.05) and 5 minutes (5.6±1.8 vs. 7.2±1.7; p<.05). The most significant prognostic factor was the modified McGoon index (1.19±0.15 in nonsurvivors vs. 1.65±0.20 in survivors; p>.0001). A lower McGoon index was significantly associated with lower Apgar scores at 1 (r=0.50; p<.0003) and at 5 minutes (r=0.41; p<.02). In multiple logistic regression only the modified McGoon index was independently related to mortality. A modified McGoon index ≤1.3 predicted mortality with a sensitivity of 85% and specificity of 100%.Although all mean z scores of cardiac valves diameters were smaller than a normal population, it did not differentiate the nonsurvivor vs. survivor groups (mitral valve:-2.8±1.2 vs. -2.1±1.5, p=0.11; tricuspid valve: -1.6±2.1 vs. -1.0±1.9, p=0.38; aortic valve: -1.8±1.1 vs.-2.4±1.0, p=0.15; pulmonary valve: -1.6±1.3 vs. -1.2±1.1, p=0.34)

Conclusion: Echocardiographic measurements of the hilar pulmonary arteries, representing the adequacy of the pulmonary vascular bed, is a strong prognostic factor for newborns with congenital CDH.