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      A patient with Marfan’s syndrome who developed an acute aortic dissection at 28 weeks of pregnancy treated with aortic root replacement

      case-report

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          Abstract

          The patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She was diagnosed with Marfan’s syndrome based on physical findings; however, the condition was not diagnosed before the onset. The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save the patient’s life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any complications.

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          A longitudinal study of the relationship between maternal cardiac output measured by impedance cardiography and uterine artery blood flow in the second half of pregnancy.

          A Vårtun, K Flo, T Wilsgaard … (2010)
          To study serial changes in maternal systemic and uterine artery haemodynamics and establish reference ranges for the second half of pregnancy. Prospective longitudinal observational study. University hospital in Norway. Low-risk pregnant women. Fifty-three low-risk pregnancies were evaluated at approximately 4-weekly intervals. Maternal systemic haemodynamics was assessed with impedance cardiography. Uterine artery blood velocity and diameter were measured using Doppler ultrasonography and uterine artery volume blood flow (Q(uta)) was calculated as the product of mean velocity and cross-sectional area of the uterine artery. The fraction of cardiac output (CO) distributed to the uterine circulation was calculated as: Q(uta)/CO x 100. CO, Q(uta), uterine vascular resistance (R(uta)) and the fraction of CO distributed to the uterine circulation. The CO increased (P = 0.0063) until 34 weeks and remained stable until term. Total Q(uta) increased from 299 to 673 ml/minute and R(uta) halved from 0.26 to 0.13 mmHg/ml/minute (P < 0.0001). The fraction of CO distributed to the uterine circulation increased from 5.6% to 11.7% (P < 0.0001). During the second half of pregnancy, Q(uta) and the fraction of maternal CO distributed to the uterine circulation increase approximately two-fold, mainly as a result of decrease in R(uta).
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            Changes in uterine blood flow during human pregnancy

            Joseph Itskovitz, Shraga Rottem, Joseph M. Brandes … (1990)
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              Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall.

              Gianluca Perrucci, Roberto Chiesa, Patrizia Nigro … (2017)
              Marfan syndrome (MFS) is a connective tissue disorder with multiple organ manifestations. The genetic cause of this syndrome is the mutation of the FBN1 gene, encoding the extracellular matrix (ECM) protein fibrillin-1. This genetic alteration leads to the degeneration of microfibril structures and ECM integrity in the tunica media of the aorta. Indeed, thoracic aortic aneurysm and dissection represent the leading cause of death in MFS patients. To date, the most effective treatment option for this pathology is the surgical substitution of the damaged aorta. To highlight novel therapeutic targets, we review the molecular mechanisms related to MFS etiology in vascular smooth muscle cells, the foremost cellular type involved in MFS pathogenesis.
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                Author and article information

                Contributors
                Riki Sumiyoshi:
                ORCID: http://orcid.org/0000-0003-1737-7587
                rikinista2120@gmail.com
                Journal
                Journal ID (nlm-ta): Gen Thorac Cardiovasc Surg
                Journal ID (iso-abbrev): Gen Thorac Cardiovasc Surg
                Title: General Thoracic and Cardiovascular Surgery
                Publisher: Springer Singapore (Singapore )
                ISSN (Print): 1863-6705
                ISSN (Electronic): 1863-6713
                Publication date (Electronic): 7 February 2021
                Publication date PMC-release: 7 February 2021
                Publication date (Print): 2021
                Volume: 69
                Issue: 6
                Pages: 1004-1007
                Affiliations
                GRID grid.416704.0, ISNI 0000 0000 8733 7415, Department of Cardiovascular Surgery, , Saitama Red Cross Hospital, ; 1-5 Shintoshin, Chuo-ku, Saitama-shi, Saitama 330-8553 Japan
                Author information
                http://orcid.org/0000-0003-1737-7587
                Article
                Publisher ID: 1596
                DOI: 10.1007/s11748-021-01596-3
                PMC ID: 8131282
                PubMed ID: 33550484
                SO-VID: 14da68d1-0d18-430e-acbf-fcc783277662
                Copyright © © The Author(s) 2021
                License:

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                Date received : 18 July 2020
                Date accepted : 14 January 2021
                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © The Japanese Association for Thoracic Surgery 2021

                ScienceOpen disciplines: Surgery
                Keywords: marfan’s syndrome,pregnancy,acute aortic dissection,aortic root surgery
                Data availability:
                ScienceOpen disciplines: Surgery
                Keywords: marfan’s syndrome, pregnancy, acute aortic dissection, aortic root surgery

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