<div>Re: Pregnancies in women with
Turner Syndrome: A retrospective multicentre UK study</div><div>Niels H. Andersen<sup>1</sup>, Claus H
Gravholt<sup>2-3</sup></div><ol><li>Department of Cardiology, Aalborg University Hospital, Hobrovej 18-22,
DK-9000 Aalborg</li><li>Department of Molecular Medicine, Aarhus University Hospital, Palle
Juul-Jensens Boulevard, DK-8200 Aarhus.</li><li>Department of Endocrinology and Internal Medicine, Aarhus University
Hospital, Palle Juul-Jensens Boulevard, DK-8200 Aarhus</li></ol><div>Corresponding author:</div><div>Niels Holmark Andersen</div><div>Department of Cardiology, Aalborg University Hospital, Hobrovej 18-22,
DK-9000 Aalborg</div><div>n.holmark@rn.dk</div><div>Sir,</div><div>We read with interest the article by Cauldwell et al., which concluded
that pregnancy in women with Turner syndrome is associated with major
maternal cardiovascular risks and cardiovascular assessment and
counselling prior to assisted or spontaneous pregnancy should be managed
by a specialist team.</div><div>These conclusions are based on data from 127 pregnancies in 81 women
with a Turner syndrome.</div><div>Cauldwell et al. describe data
from 16 different centres with one to 42 pregnancies per centre over an
observation period of 20 years. Details on pregnancies per centre are
not presented but there must have been many centres where a Turners
syndrome pregnancy was a rarity not even happening once a year. When
analysing the numbers, it seems that the different centres beyond the
centre with 42 cases will have seen an average of 6 pregnant women with
Turner syndrome over 20 years. This must have had significant impact on
the experience and expertise of the obstetricians in the different
centres. Could this be the reason why the caesarean section rates where
markedly higher than other wise reported?</div><div>In the present article the rate was 61-67 % compared to data from
Sweden where it was 38 % and 47 % in France. <sup>1-2</sup></div><div>Cauldwell et al. also state that pregnancies in Turner syndrome are
associated with major cardiovascular risks based upon three events. One
woman suffered a type A dissection at 18 weeks gestation despite a
normal aortic size but a bicuspid aortic valve. No other details about
hypertension, aortic morphology or growth rates are presented. The two
other events were women with severe aortic disease that should have been
counselled and offered prophylactic aortic surgery before pregnancy. So,
two out of three cases were not events but results of inadequate
clinical care. In the Cauldwell cohort only 57.4% had seen a
cardiologist within 24 months before getting pregnant, which is far from
what is necessary in the caretaking of women with Turner syndrome and a
childbearing potential.</div><div>Data from Scandinavia, France, and from the Alliance for Adult Research
in Congenital Cardiology tells another story. <sup>2-4</sup> In
these cohorts the number of pregnancy related dissections was very low
due to pre-pregnancy counselling, centralised follow-up, and timely
prophylactic aortic surgery before considerations about normal pregnancy
or egg donation. This simply generates better results.</div><div>A safe pregnancy and childbirth are very important for women with Turner
syndrome. What we can learn from this article is what not to do.
Pregnancy and delivery in women with Turner syndrome must be centralised
and a timely cardiovascular assessment of women with Turner syndrome and
a pregnancy wish should be obligatory. Otherwise, we will end with
results as described in this article.</div><div>In other words, the conclusion of the article ought to have been lack of
centralisation and appropriate cardiovascular pre-pregnancy assessment
leads to increased morbidity and cardiovascular risk during pregnancy
childbirth. If such pre-pregnancy assessment is in place and caretaking
is centralised, cardiovascular risk during pregnancy is very low in
Turner syndrome. <sup>1-4</sup></div><div>References</div><div>1. Hagman A, Källén K, Barrenäs M-L, Landin-Wilhelmsen K, Hanson C,
Bryman I, et al. Obstetric outcomes in women with Turner karyotype. J
Clin Endocrinol Metab. 2011; 96: 3475–82.</div><div>2. Bernard V, Donadille B, Zenaty D, Courtillot C, Salenave S, Brac de
la Perrière A, et al. Spontaneous fertility and pregnancy outcomes
amongst 480 women with Turner syndrome. Hum Reprod 2016; 31:782–8.</div><div>3. Hagman A, Loft A, Wennerholm U-B, Pinborg A, Bergh C, Aittomäki K, et
al. Obstetric and neonatal outcome after oocyte donation in 106 women
with Turner syndrome: a Nordic cohort study. Hum Reprod 2013; 28:
1598–609.</div><div>4. Grewal J, Valente AM, Egbe AC, Wu FM, Krieger EV, Sybert VP et al.
Cardiovascular outcomes of pregnancy in Turner syndrome. Heart 2021;107
:61–6.</div>