Aortic dissection in a pregnant patient with Turner syndrome
Authors:
V. Ťápalová 1; I. Vlašínová 1; P. Janků 1; P. Ventruba 1; P. Kysela 2; T. Zatočil 3; P. Štourač 4
Authors‘ workplace:
Gynekologicko-porodnická klinika, LF MU a FN Brno
1; Chirurgická klinika, LF MU a FN Brno
2; Interní kardiologická klinika, LF MU a FN Brno
3; Anesteziologicko-resuscitační klinika, LF MU a FN Brno
4
Published in:
Prakt Gyn 2011; 15(2): 98-100
Category:
Case Report
Overview
Summary:
Turner syndrome is one of the most frequent genetic disorders, affecting 1/2,500 live female births. The principal features of Turner syndrome include short stature and ovarian dysgenesis with oestrogen deficit. Congenital defects of cardiovascular system are common, making women with TS a high risk group with respect to pregnancy.
Case report:
a 31 years old woman with TS was hospitalized at our clinic in December 2009 for imminent preterm birth at 32nd week of pregnancy. Coarctation of the aorta was performed at 4 years of her age. The present gravidity after a 2nd cycle of in-vitro fertilisation with donated oocytes - gemini biamniales bichoriales. Due to placental insufficiency, she was indicated for the C-section on the 8th day of her hospitalization. During the surgery, retroperitoneal bleeding occurred. The called-in surgeon specified a spontaneous rupture of abdominal aorta as the source of bleeding. The rapture was sutured and, subsequently, plastic surgery of the aorta was performed. Supracervical hysterectomy and adnexectomy were executed bilaterally due to devitalized uterus. Haemorrhagic shock as well as a disseminated intravascular coagulation and cardiac arrest (CPR 45 min) developed in the course of the surgery. Total blood loss was 19 l. Despite immediate surgical and resuscitation treatment, the patient died during a revision of abdominal cavity the following day. Because of frequent cardiovascular abnormalities, gravidity is not recommended in women with TS, unless they have undergone a thorough evaluation by a cardiologist, including MRI of the arteries and larger veins. Singleton gravidity is to be prioritized in IVF patients.
Key words:
Turner syndrome – in-vitro fertilisation – coarctation of the aorta
Sources
1. Turner H. A syndrom of infatilism, congenital webbed neck and cubitus valgus. Endocrinology 1938; 23: 566–574.
2. Zapletalová J, Lebl J, Šnajderová M. Turnerův syndrom. Praha: Galén 2003: 35–37.
3. Ho VB, Bakalov VK, Cooley M et al. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation 2004; 110(12): 1694–1700.
4. Tarani L, Lampariello S, Raguso G et al. Pregnancy in patients with Turner’s syndrome: six new cases and review of literature. Gynecol Endocrinol 1998; 12(2): 83–87.
5. Berková M, Berka Z, Krčová Z. Turnerův syndrom vyžaduje multidisciplinární přístup. Vnitř lék 2009; 55(5): 501–505.
6. Gravholt CH. Turner syndrome in adulthood. Horm Res 2005; 64 (Suppl 2): 86–93.
7. Gøtzsche CO, Krag-Olsen B, Nielsen J et al. Prevalence of cardiovascular malformations and association with karyotypes of Turner’s syndrome. Arch Dis Child 1994; 71(5): 433–436.
Labels
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicineArticle was published in
Practical Gynecology
2011 Issue 2
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