P06.12: Monozygotic twin pregnancy discordant for partial trisomy 13

Case: A 34-year-old female, 0 gravida 0 para, was diagnosed with TTTS stage III atypical at 18 weeks’ gestation. SFLP was performed for the treatment of TTTS stage IV at 20 weeks’ gestation. At 21 weeks gestation, the recipient demised and the donor manifested pulmonary valve regurgitation (PR) and tricuspid valve regurgitation (TR). The patient was referred to our department at 22 weeks gestation. Fetal echocardiography revealed PR, TR and continuous reverse flow of the pulmonary artery (PA) from PDA to right ventricle (RV) with worsening cardiac function. Reverse flow of PA made RV enlarged, RV wall motion worse, reverse flow of IVC and DV increased with advancing gestational age, and then the donor was in the condition of right heart failure. At 26 weeks gestation, the donor manifested hydropic sign: systemic edema, cardiac effusion, ascites and cardiomegaly. She was then managed with serial laboratory studies and regular ultrasound scan. Cesarean delivery was performed at 36 weeks resulting in a live donor baby of 2564 g and a dead recipient of 110 g. The donor was put on intubation and ventilator therapy and PGE1 div. In the stable pulmonary condition, right ventricular wall was thick moderately and TR was estimated about 3 m/s despite of poor wall motion, so PGE1 div was stopped to evaluate the remaining RV function. PA flow was improved forward according only to the heart failure therapy, and systemic condition was recovered almost normal at 50 days of age. Written informed consent was obtained from the mother and father to report this case.