Reply of the authors: Cardiovascular risks of pregnancy in women with Turner syndrome & Susceptibility to aortic dissection in Turner Syndrome pregnancy: inadequacy of the ASRM Practice Committee Report

Abstract

We thank Dr. Silberbach, Dr. Bondy, and collaborators for their thoughtful letters and comments. We do agree that American Society for Reproductive Medicine (ASRM) recommendations (1The Practice Committee of the American Society for Reproductive MedicineIncreased maternal cardiovascular mortality associated with pregnancy in women with Turner syndrome.Fertil Steril. 2005; 83: 1074Abstract Full Text Full Text PDF PubMed Scopus (53) Google Scholar) should be modified for better control of cardiovascular risk in women with Turner syndrome (TS) attempting pregnancy. Thanks to the study of Matura et al. (2Matura L.A. Ho V.B. Rosing D.R. Bondy C.A. Aortic dilatation and dissection in Turner syndrome.Circulation. 2007; 116: 1663-1670Crossref PubMed Scopus (275) Google Scholar), we know that the aortic diameter should be normalized to body surface area particularly in women with TS because of their small size. We agree that women with TS should be accepted into an oocyte donation (OD) program only if their aortic size index (ASI) is <2 cm/m2. Moreover, we do agree with Dr. Silberbach that even an isolated bicuspid aortic valve (BAV) is a powerful predictor for aortic complications. An ASI >2 cm/m2, a BAV, and other congenital heart disease should be contraindications for OD because of an unacceptable risk of aortic dissection during or after pregnancy. Hypertension, when moderated and controlled, should be also discussed before attempting a pregnancy because of the enhanced risk of toxemia and preeclampsia and their deleterious consequences on thoracic aorta. Other abnormalities like renal and metabolic defects should also be taken into account for acceptance of pregnancy. We think that the cardiovascular screening (CVS) of women with TS before OD should be done in a center with good expertise in cardiovascular imaging. Magnetic resonance imaging (MRI) and transthoracic echocardiography (TTE) both have their advantages and limitations in the assessment of thoracic aorta. The diagnosis of a bicuspid valve is easier with TTE than with MRI especially in bicuspid valves with raphe, which represent 50% of cases (3Roberts W.C. The congenitally aortic bicuspid aortic valve. A study of 85 autopsy cases.Am J Cardiol. 1970; 26: 72-83Abstract Full Text PDF PubMed Scopus (698) Google Scholar), but MRI is indicated when echogenicity is not good enough and has the advantage of analyzing the entire aorta. If both techniques can be used for CVS before OD, TTE will be preferred for careful follow-up during pregnancy. Furthermore, we think that the same referent center should make the preconception screening and should ensure the cardiovascular follow-up during pregnancy when possible. Turner women, without any cardiac abnormalities such as BAV, aortic coarctation, aortic dilation, or hypertension, remain at a significantly higher risk of aortic dissection during pregnancy because of their “latent” aortopathy (4Lopez L. Arheart K.L. Colan S.D. Stein N.S. Lopez-Mitnik G. Lin A.E. et al.Turner syndrome is an independent risk factor for aortic dilation in the young.Pediatrics. 2008; 121: e1622-e1627Crossref PubMed Scopus (90) Google Scholar). Furthermore, the physiologic increase in aortic diameter during pregnancy can hasten the modifications in the aortic wall and the dilatation of the aorta and lead to premature death in the years after pregnancy. Patients must be informed of this risk. After the dramatic death we reported, all women with TS requesting OD in our center were carefully screened again. We were surprised to observe the gap between current cardiovascular evaluations made during childhood and/or adulthood and the malformations we discovered, particularly undetected BAV. We also noticed a lack of follow-up after childhood for most patients. There is a real need for a better follow-up of Turner women particularly at the beginning of adulthood and before any pregnancy attempt as recently recommended (5Bondy C.A. The Turner Syndrome Consensus Study GroupCare of girls and women with Turner syndrome: a guideline of the Turner syndrome study group.J Clin Endocrinol Metab. 2007; 92: 10-25Crossref PubMed Scopus (723) Google Scholar). Cardiovascular risks of pregnancy in women with Turner syndromeFertility and SterilityVol. 91Issue 5PreviewLetter to the Editor: Full-Text PDF Susceptibility to aortic dissection in Turner Syndrome pregnancy: inadequacy of the ASRM Practice Committee ReportFertility and SterilityVol. 91Issue 5PreviewLetter to the Editor: Full-Text PDF