Congenital connective tissue dysplasia and obstetric risks
Authors:   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Makatsariya A.D. , Radetskaya L.S. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Bitsadze V.O. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Khizroeva J.Kh. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Di Renzo G.C. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Tretyakova M.V. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Makatsariya N.A. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Shkoda A.S. ,   L.A.Vorokhobov City Clinical Hospital No 67, Moscow, Russian Federation, Degtyareva N.D. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Kudryavtseva E.S. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Lazarchuk A.V. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Drozhzhina M.P. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Vorobev A.V. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Kuneshko N.F. ,   Odintsovo Maternity Hospital, Odintsovo, Moscow, Russian Federation, Grigoryeva K.N. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Gashimova N.R. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Egorova E.S. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Gris J.-Ch. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation, Elalamy I. ,   I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation
Published: January 2025

Congenital connective tissue diseases are numerous disorders that are associated with connective tissue pathology, involving different organs and systems: musculoskeletal, cardiovascular, ocular, etc.
Objective. To study the features of pregnancy course in patients with mesenchymal connective tissue dysplasias.
Patients and methods. The course of pregnancy and labor in 56 women aged 18 to 36 years with congenital connective tissue dysplasias (CTDs) was analyzed: 23 patients with Marfan syndrome, 22 with Ehlers–Danlos syndrome, and 11 with hereditary hemorrhagic telangiectasia.
Results. All patients with congenital CTD had hemorrhagic manifestations during pregnancy. They also had a significantly increased incidence of both obstetric and hemorrhagic complications during pregnancy, labor, and postpartum period. The high incidence of fetal growth restriction in pregnant women with congenital CTD may be due to abnormalities in the structure of connective tissue of the uteroplacental vascular complex. In general, patients with congenital CTD are at higher risk of perinatal morbidity.
Conclusion. The decision on the possibility of pregnancy for each woman with congenital CTD is made individually. Given the high risk of life-threatening complications during vaginal delivery, cesarean section is recommended for most of these patients.
Key words: congenital connective tissue diseases, mesenchymal dysplasias, Marfan syndrome, Ehlers–Danlos syndrome, Osler–Weber–Rendu syndrome, hereditary hemorrhagic telangiectasia, gestational process, pregnancy, aortic dissection in pregnancy, hemorrhage