Maternofetal Cardiovascular Complications in Turner Syndrome After Oocyte Donation: Insufficient Prepregnancy Screening and Pregnancy Follow-up Are Associated With Poor Outcome

Abstract

The life expectancy of girls with Turner syndrome (TS) is reduced, primarily due to the well-known cardiovascular complications, including cardiovascular malformations, early-onset hypertension, coronary ischemia, and aortic dissection and rupture. The use of recombinant hGH treatment and successful oocyte donation (OD) in ovarian insufficiency has improved the quality of life of girls with TS. With the increasing use of OD during in vitro fertilization in recent years among TS women, pregnancy is now possible, but these patients appear to be at substantial risk for adverse maternofetal outcome. Limited data from case reports and other small studies on TS women suggest that there is an increased frequency of maternal deaths due to the rupture of the aortic root as well as a high frequency of pregnancy-related hypertension. Pregnancies in TS women should be considered as high risk. Specific recommendations made by French Medical Societies for TS pregnancies include pregravid cardiac evaluation, aggressive treatment when indicated, and careful follow-up. This multicenter retrospective study investigated the maternofetal outcome of TS pregnancies achieved with OD. All pregnancies were ≥20 weeks of gestation. Data were obtained from reproductive technology centers affiliated with the French Study Group for Oocyte Donation and included 93 pregnant TS patients with ongoing pregnancies who had received OD between 1991 and 2009. Less than 38% of the 93 patients had received pregravid cardiac screening evaluation (including echocardiography [33.3%, n = 31] and thoracic magnetic resonance imaging [4.3%, n = 4]. Among the 82 patients with complete outcome data in this study, 31 (37.8%) developed pregnancy-associated hypertensive disorders (PAHD), including 54.8% (17/31) with PE and 4 with severe preeclampsia or eclampsia. The rate of prematurity was high (38.3%) and was primarily correlated with PAHD (P = 0.01). PAHD were related to a high rate of intrauterine growth retardation and severe intrauterine growth retardation (27.5 and 13.7%, respectively). One case of eclampsia resulted in fetal demise at 23 weeks of gestation. There was an increased risk of maternal death due to aortic dissection or rupture after delivery by cesarean section in 2 patients (2.2%, 4/93). An absolutely normal maternofetal outcome occurred in only 40.2% of the TS pregnancies. These findings show that pregnancies obtained by OD in TS are at risk for maternal death due to aortic dissection and rupture, and require management according to specific recommendations for pregravid cardiac evaluation, aggressive treatment for hypertension and other cardiovascular problems, and careful follow-up.