The review by Hadnott and Bondy, in the previous issue of Expert Review of Obstetrics & GynecologyCitation[1], surveys the important but sparse case literature that establishes the incremental risk for aortic dissection contributed by pregnancy in Turner syndrome (TS) individuals. The authors point out that while the risk of aortic dissection in TS in general is low (˜1%), the risk during TS pregnancy is three- to five-times that. Indeed, the risk may be as high as sixfold if the reported aortic dissections occurring 1–3 years after TS pregnancy are taken into account Citation[2,3]. Furthermore, TS women suffer from other maladies of pregnancy including hypertensive disorders, such as HELLP syndrome Citation[4], hypothyroidism Citation[5] and increased spontaneous abortion rates that add to the risks of pregnancy. Hadnott and Bondy should be commended for their diligence in putting together this important manuscript. However, in their ‘Expert Commentary,’ too little is said about the very important topics of adoption or surrogacy as alternatives to assisted reproductive therapy (ART). Three major points need to be made:
• The consequences of aortic dissection are severe and often catastrophic Citation[6];
• Although individuals with congenital heart disease are more likely to dissect, because there is a TS aortopathy, all TS women are at risk Citation[7];
• TS women are at increased risk for other pregnancy complications Citation[8].
The decision algorithm to recommend ART is fundamentally different from what is typically employed by caregivers when trying to decide whether a treatment should be offered or withheld. Usually we determine whether the procedure’s benefit outweighs both the risk of performing the procedure and the risk of withholding it (i.e., becoming more ill if left untreated). For example, the decision to offer the dangerous Norwood operation for hypoplastic left heart syndrome is supported by the fact that withholding the procedure would be uniformly fatal. In the case of offering ART to women with TS, the decision must be based solely on an estimate of the risk of the procedure to the patient, which we know is considerable. Importantly, withholding ART is not constrained by the risk of any medically significant adverse outcome. There is no medical downside to not becoming pregnant.
No doubt the work by Hadnott and Bondy will become a key reference for physicians who counsel TS women about pregnancy, and will also serve as a major medical–legal citation when pregnant TS women unfortunately die after erroneous recommendations regarding its safety. Certainly any woman has the right to choose their reproductive destiny. However, it is incumbent on medical caregivers to carefully weigh all risks and offer the safest effective alternative to their patients. In my view, if the goal of a TS woman is motherhood, then adoption or surrogacy are the best options.
Disclaimer
This work is the opinion of the author and does not represent the views of Expert Reviews Ltd or its employees.
Financial & competing interests disclosure
The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
No writing assistance was utilized in the production of this manuscript.
References
- Hadnott TN, Bondy AC. Risks of pregnancy for women with Turner syndrome. Expert Rev. Obstet. Gynecol.6(2), 217–227 (2011).
- Gravholt CH, Landin-Wilhelmsen K, Stochholm K et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol. Young16(5), 430–436 (2006).
- Bondy C, Rosing D, Reindollar R. Cardiovascular risks of pregnancy in women with Turner syndrome. Fertil. Steril.91(5), e31–e32; author reply e34 (2009).
- Bodri D, Vernaeve V, Figueras F, Vidal R, Guillen JJ, Coll O. Oocyte donation in patients with Turner’s syndrome: a successful technique but with an accompanying high risk of hypertensive disorders during pregnancy. Hum. Reprod.21(3), 829–832 (2006).
- Saenger P. Turner’s syndrome. N. Engl. J. Med.335(23), 1749–1754 (1996).
- Carlson M, Silberbach M. Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature. J. Med. Genet.44(12), 745–749 (2007).
- Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS. Vasculopathy in Turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction. J. Clin. Endocrinol. Metab.90(9), 5161–5166 (2005).
- Chevalier N, Letur H, Lelannou D et al. Materno–fetal cardiovascular complications in Turner syndrome after oocyte donation: insufficient prepregnancy screening and pregnancy follow-up are associated with poor outcome. J. Clin. Endocrinol. Metab.96(2), E260–E267 (2011).