EP09.29: <i>In utero</i> hydrops resolution improves cardiac function in a fetus affected by right congenital diaphragmatic hernia: a case report

A pregnant woman at 29 weeks with a fetal right congenital diaphragmatic hernia (RCDH), complicated by non-immune hydrops (NIH) was sent to our unit. The fetus presented with voluminous right hydrothorax, mediastinal shift to the left and congestive cardiac failure. The combined cardiac output (CCO) (according Mielke&Benda, 2001) resulted 460.7ml/min (Z-score -1.2 according to Rocha, 2019). Magnetic resonance confirmed the ultrasound suspects. A 35-mm thoracoamniotic shunt (TaS) was placed in the right hemithorax without complications. One week after, resolution of edema, ascites, and pleural effusion was noted. CCO significantly increased, with a Z-score of -0.2, alongside improvements in right and left CO (Z-scores: -0.3 and -0.8, respectively). After two weeks, despite proper TaS placement, cardiac function deteriorated (CCO -1.4 Z score) with ascites, suggesting a TaS occlusion. At 33 weeks, an urgent Caesarean section was performed for labour in breech presentation. Despite intensive care, the newborn died the day after due to pulmonary hypertension and respiratory insufficiency. Autopsy revealed a thin serous right hemidiaphragm, suggestive of diaphragmatic eventration with muscular agenesis. The impact of the TaS on fetal circulation and the mechanisms of NIH in RCDH are still unclear. In our case, CO improvement is strictly related to the reduction of the NIH, revealing a correlation between lymphatic effusions and CCO. It could be useful to observe fetal heart function's modification in case of extracardiac anomalies. Due to the high mortality rate of RCDH, TaS cannot be considered a treatment. Further studies will provide info about the CCO trend to better understand fetal circulation physiopathology. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.