PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA)
Aim. To study the pregnancy course and delivery outcome in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome (EDS), Hereditary hemorrhagic telangiectasia (HHT).Material and Methods. 56 pregnant women with mesenchymal dysplasias (23 with Marfan syndrome, 22 with Ehlers-Dan...
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IRBIS LLC
2016-06-01
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| Series: | Акушерство, гинекология и репродукция |
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| Online Access: | https://www.gynecology.su/jour/article/view/39 |
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| author | L. S. Radetskaya A. D. Makatsariya |
| author_facet | L. S. Radetskaya A. D. Makatsariya |
| author_sort | L. S. Radetskaya |
| collection | DOAJ |
| description | Aim. To study the pregnancy course and delivery outcome in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome (EDS), Hereditary hemorrhagic telangiectasia (HHT).Material and Methods. 56 pregnant women with mesenchymal dysplasias (23 with Marfan syndrome, 22 with Ehlers-Danlos syndrome, 11 with hereditary hemorrhagic telangictasia (HHT) were examined in specialized cardiological maternity house by using physical examination, routine laboratory tests, hemostasiological tests, ECG, echocardiography, MRI, radiology, pulse oximetry.Results. All patients had hemorrhagic complications various localizations; 18 women with Marfan syndrome developed increasing of mitral regurgitation, 10 – aortic insufficiency, 13 – mitral valve insufficiency. 1 patient with Marfan syndrome was died due to aortic dissection in 45 day after delivery. Pulmonary arteriovenous malformations were visualized in 10 patients with HHT; the symptoms of hypoxemia developed in 6 HHT patients and were result of right-to-left. One patient with HHT developed the symptoms of compression of vena cava inferior due to aneurism of truncus brachiocephalicus. 40 patients had abnormal hemostasiological tests (platelet dysfunction – in 38 patients; DIC syndrome – in 31). Cesarean section was performed in 53 patients. Vaginal delivery (3 patients with EDS) was complicated by massive postpartum hemorrhage and deep vaginal tears.Conclusions. Patients with mesenchymal dysplasias have a great risk of different complications, during pregnancy and delivery and require the multidisciplinary care and repeated hemostasiological testing. Cesarean section is the preferred method of delivery in such patients. |
| format | Article |
| id | doaj-art-6bd4bd4252844ffba0b04e294237ccd3 |
| institution | DOAJ |
| issn | 2313-7347 2500-3194 |
| language | Russian |
| publishDate | 2016-06-01 |
| publisher | IRBIS LLC |
| record_format | Article |
| series | Акушерство, гинекология и репродукция |
| spelling | doaj-art-6bd4bd4252844ffba0b04e294237ccd32025-08-20T02:53:58ZrusIRBIS LLCАкушерство, гинекология и репродукция2313-73472500-31942016-06-0110110011010.17749/2313-7347.2015.10.1.100-11038PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA)L. S. Radetskaya0A. D. Makatsariya1First Moscow State Medical Sechenov University of the Ministry of Health Russian FederationFirst Moscow State Medical Sechenov University of the Ministry of Health Russian FederationAim. To study the pregnancy course and delivery outcome in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome (EDS), Hereditary hemorrhagic telangiectasia (HHT).Material and Methods. 56 pregnant women with mesenchymal dysplasias (23 with Marfan syndrome, 22 with Ehlers-Danlos syndrome, 11 with hereditary hemorrhagic telangictasia (HHT) were examined in specialized cardiological maternity house by using physical examination, routine laboratory tests, hemostasiological tests, ECG, echocardiography, MRI, radiology, pulse oximetry.Results. All patients had hemorrhagic complications various localizations; 18 women with Marfan syndrome developed increasing of mitral regurgitation, 10 – aortic insufficiency, 13 – mitral valve insufficiency. 1 patient with Marfan syndrome was died due to aortic dissection in 45 day after delivery. Pulmonary arteriovenous malformations were visualized in 10 patients with HHT; the symptoms of hypoxemia developed in 6 HHT patients and were result of right-to-left. One patient with HHT developed the symptoms of compression of vena cava inferior due to aneurism of truncus brachiocephalicus. 40 patients had abnormal hemostasiological tests (platelet dysfunction – in 38 patients; DIC syndrome – in 31). Cesarean section was performed in 53 patients. Vaginal delivery (3 patients with EDS) was complicated by massive postpartum hemorrhage and deep vaginal tears.Conclusions. Patients with mesenchymal dysplasias have a great risk of different complications, during pregnancy and delivery and require the multidisciplinary care and repeated hemostasiological testing. Cesarean section is the preferred method of delivery in such patients.https://www.gynecology.su/jour/article/view/39inherited connective tissue disordersmesenhymal dysplasiasmarfan syndromeehlers-danlos syndromeosler-weber- rendu diseasehereditary hemorrhagic telangiectasiapregnancyaortic dissectionarteriovenous malformations |
| spellingShingle | L. S. Radetskaya A. D. Makatsariya PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) Акушерство, гинекология и репродукция inherited connective tissue disorders mesenhymal dysplasias marfan syndrome ehlers-danlos syndrome osler-weber- rendu disease hereditary hemorrhagic telangiectasia pregnancy aortic dissection arteriovenous malformations |
| title | PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) |
| title_full | PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) |
| title_fullStr | PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) |
| title_full_unstemmed | PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) |
| title_short | PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA) |
| title_sort | pregnancy and delivery in patients with mesenhymal dysplasias marfan syndrome ehlers danlos syndrome hereditary hemorrhagic telangiectasia |
| topic | inherited connective tissue disorders mesenhymal dysplasias marfan syndrome ehlers-danlos syndrome osler-weber- rendu disease hereditary hemorrhagic telangiectasia pregnancy aortic dissection arteriovenous malformations |
| url | https://www.gynecology.su/jour/article/view/39 |
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