Abstract

To provide an update on the outcomes of isolated prenatally diagnosed right-sided CDH (RCDH), either managed expectantly or with fetal endoscopic tracheal occlusion (FETO). Multicenter retrospective analysis of prospectively collected data from patients with isolated RCDH managed according to a standardized protocol. Prenatal variables were lung size assessed with ultrasound (observed-to- expected lung-to-head ratio, o/eLHR) and with MRI (observed-to-expected total lung volume o/eTLV), occurrence of polyhydramnios, pleural or pericardial effusion, preterm rupture of membrane (pPROM) and gestational age (GA) at delivery. Postnatal outcome measures included survival until discharge and morbidity parameters (patch repair, need of treatment for pulmonary hypertension, length of admission at NICU, oxygen dependency at discharge). A total of 163 infants with RCDH live born after 30 weeks were included. Sixty-four cases were managed expectantly. In this group, the overall survival rate was 44%. At univariate analysis, the o/eLHR was the only significant predictor of survival, even though with a low predictive value (table 1). None of the expectantly managed fetuses with an o/eLHR below 30% survived at discharge. Survival rate was 25% in fetuses with o/eLHR between 30 and 45%, leading to a pooled survival rate of 17% in the severe group. This was significantly lower than in the cases with o/eLHR above 45% (62%, p<0.001). Ninety-nine cases, all with o/eLHR ≤45%, underwent FETO at a mean GA of 28±1.4 weeks. Survival at discharge in this group was significantly higher as compared to the severe cases managed expectantly (38%, p=0.04). On univariate analysis in the FETO group, no significant predictor of survival (Table 2) could be identified. Morbidity analysis is ongoing. Antenatal measurement of o/eLHR predicts survival. In this study, an o/eLHR below 45% corresponds to an estimated survival rate under 20%. In this group, FETO is associated with a significant increase in survival.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

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