EP10.17: Right heart failure in a 36‐week fetus with umbilical vein aneurism: a key to pathophysiology

Abstract

We report a case of a 26-year-old G1 P0 with an enormous rhabdomyoma arising from the septum and compressing both ventricles. The first trimester NT was normal. A biochemical screening was suggested and refused by the couple. The diagnosis was done at 22 weeks with a hyperechoic mass located at the septum. Morphology scan did not reveal any other associated abnormalities. The mass increased in size during ultrasound follow-up to reach 3.4*2.8 cm. Despite that the mass reached a size big enough to obliterate the ventricular cavities and to limit severely the cardiac output, there was no hydrops. Umbilical and cerebral Doppler were within normal. Biometry was at the 10th percentile. At 36 weeks the patient was admitted for labour and delivered by Caesarean section in our university hospital. The baby had respiratory distress and underwent intubation and ventilation. Hemodynamic stabilisation was not possible, and as the couple had already agreed to this possibility the baby was taken for an immediate salvage surgery. He, unfortunately, deceased briefly following surgery because of cardiac failure. Pathology of the mass confirmed a rhabdomyoma. Parents' consent was obtained for the scientific report of this case.In conclusion, cardiac rhabdomyoma can have different neonatal outcomes. Size seemed to be an important prognostic factor in this reported case.