Abnormal shape and size of the cardiac ventricles is associated with a higher risk of neonatal death in fetuses with isolated left congenital diaphragmatic hernia.

Objective To evaluate the association between fetal cardiac deformation analysis (CDA) and cardiac function with severe adverse perinatal outcomes in fetuses with isolated left congenital diaphragmatic hernia (CDH). Methods CDA in each ventricle (contractility, size, and shape), evaluated by speckle tracking and novel FetalHQ software, and markers of cardiac function (E/A ratios, pulmonary and aortic peak systolic velocities, and sigmoid annular valves diameters), were evaluated in fetuses with isolated left CDH. Two evaluations were performed: at referral (CDA and function) and within three weeks of delivery (CDA). Severe adverse neonatal outcomes were considered neonatal death (ND) or survival with CDH associated pulmonary hypertension (CDH-PH). Differences and associations between CDA, cardiac function, and severe adverse outcomes were estimated. Results Fifty fetuses were included and seventeen (34%) had severe adverse neonatal outcomes (11 ND and 6 survivors with CDH-PH). At first evaluation, the prevalence of a small left ventricle was 34% (17/50) with a higher prevalence among neonates presenting severe adverse outcomes (58.8% [10/17] vs. 21.2% [7/33]; p=0.01; OR 5.03 [1.4-19.1; p=0.01]) and among those presenting with neonatal mortality (8/11 [72.7%] vs. 9/39 [23.0%]; p=0.03]; OR 8.9 1.9-40.7 p=0.005). No differences in cardiac function or strain were noted between fetuses with or without severe adverse outcomes. Within 3 weeks of delivery the prevalence of small left ventricle was higher (19/34; 55.8%) with a more globular shape (reduced transverse/longitudinal ratio TLR). A globular right ventricle was significantly associated with neonatal death or survival with CDH-PH (OR, 14.2 (1.5-138.3); p=0.02). Conclusion Fetuses with isolated CDH at risk of perinatal death or survival with CDH-PH had a higher prevalence of a small left ventricle and abnormal shape of the right ventricle.