Careful cardiovascular screening and follow-up of women with Turner syndrome before and during pregnancy is necessary to prevent maternal mortality.

Objective: To report the fatal outcome of a woman with Turner syndrome (TS) undergoing assisted reproductive technology (ART).

Design: Case report.

Setting: Reproductive medicine center.

Patient(s): A 33-year-old woman with TS.

Intervention(s): Screening before oocyte donation and treatment of aortic dissection occurring at term pregnancy.

Main Outcome Measure(s): Evaluation of cardiovascular risk.

Result(s): After a normal cardiac screening, a woman with TS got pregnant as a result of oocyte donation. At 16 weeks of gestation, a bicuspid aortic valve was detected and associated with moderate aortic root dilation. Aortic dissection was diagnosed at 38 weeks of gestation, which required emergent cesarean delivery and aortic root replacement. Despite surgical treatment, early maternal death was recorded.

Conclusion(s): Careful cardiac screening and close follow-up before and during pregnancy are necessary in patients with TS.