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Marfan pregnancy

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https://www.readbyqxmd.com/read/28622940/feasibility-of-performing-non-contrast-magnetic-resonance-angiography-in-pregnant-subjects-with-familial-aortopathies
#1
William E Moody, Luke Pickup, Emma Plunkett, John Fryearson, Paul F Clift, R Katie Morris, Peter J Thompson, Sara Thorne, Lucy E Hudsmith
BACKGROUND: Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic resonance angiography (MRA) in pregnant subjects with inherited aortopathy. METHODS: Fifteen pregnant subjects (age 27±4yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D-SSFP MRA at 1...
June 2, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28616354/comparing-american-european-and-asian-practice-guidelines-for-aortic-diseases
#2
Jose Rozado, Maria Martin, Isaac Pascual, Daniel Hernandez-Vaquero, Cesar Moris
The aortic disease comprises a group of different pathologies of high prevalence, seriousness and ever changing by the medical and surgical investigations. Therefore cardiovascular scientific societies in USA, Europe and Asia have created Task Force on practice guidelines (PG) to develop, update and revise PG for aortic diseases. These documents issue recommendations on the diagnosis and management of different aortic diseases. The three societies agree on the recommendations about diagnostic tests and on the value of computed tomography and magnetic resonance as the main tools for the diagnosis and follow-up of aortic disease...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28611929/epidural-anesthesia-for-cesarean-section-in-a-pregnant-woman-with-marfan-syndrome-and-dural-ectasia
#3
Franco Pepe, Mariagrazia Stracquadanio, Francesco De Luca, Agata Privitera, Elisabetta Sanalitro, Puccio Scarpinati
Marfan syndrome (MFS) is a genetic disorder of connective tissue, characterized by variable clinical features and multisystem complications. The anesthetic management during delivery is debated. Regional anesthesia has been used with success during cesarean delivery, but in some MFS patients there is a probability of erratic and inadequate spread of intrathecal local anesthetics as a result of dural ectasia. In these cases, epidural anesthesia may be a particularly useful technique during cesarean delivery because it allows an adequate spread and action of local anesthetic with a controlled onset of anesthesia, analgesia, and sympathetic block and a low risk of perioperative complications...
2017: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28609711/birth-characteristics-of-women-with-marfan-syndrome-obstetric-and-neonatal-outcomes-of-their-pregnancies-a-nationwide-cohort-and-case-control-study
#4
Kristina Kernell, Gunilla Sydsjö, Marie Bladh, Ann Josefsson
OBJECTIVE: The aim was to investigate birth characteristics, obstetric and neonatal outcomes of the first childbirth in women with Marfan syndrome by use of Swedish national registers since pregnancy-related outcomes in women with Marfan syndrome have only been sparsely investigated. STUDY DESIGN: In this national population-based cohort study and matched case-control study of Swedish women born 1973-1993, women with Marfan syndrome (n=273) were compared to women without the condition (n=1 017 265)...
June 1, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28606654/management-of-marfan-syndrome-during-pregnancy-a-real-world-experience-from-a-joint-cardiac-obstetric-service
#5
Joanna C E-S Lim, Matthew Cauldwell, Roshni R Patel, Anselm Uebing, Ruth A Curry, Mark R Johnson, Michael A Gatzoulis, Lorna Swan
BACKGROUND: Pregnancy in Marfan Syndrome (MFS) is associated with increased maternal risk of cardiovascular events. Given the maternal and genetic risks, pre-conception counselling is essential to facilitate informed choices. Multidisciplinary antenatal care with regular imaging is mandatory and best delivered through a Joint Cardiac Obstetric Service (JCOS). The aim of this study was to compare the care delivered in a JCOS against recognised international standards (European Society of Cardiology (ESC))...
May 22, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28600893/pregnancy-marfan-syndrome-and-type-b-aortic-dissection
#6
Mark R Johnson, Jolien W Roos Hesselink
Sayama et al describe the outcome of 17 pregnancies in 15 women with a clinical or genetic diagnosis of Marfan syndrome (MS) as per the revised Ghent criteria (Dietz et al, J Med Genet. 2010;47;476-85). Five patients (5 pregnancies) had undergone aortic root replacement prior to pregnancy and 10 (12 pregnancies) had not. A type B aortic dissection AD(B) occurred in 3 of the 5 with prior aortic root replacement (ARR) and 1 of the 10, without. This case series reminds us of critically important points in the management of pregnant women with MS who have undergone ARR...
June 10, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28482718/pregnancy-and-mesenchimal-dysplasias-marfan-syndrome-ehlers-danlos-syndrome-hereditary-hemorrhagic-telangiectasia
#7
L S Radetskaya, A D Makatsariya, V O Bitsadze, J K Khizroeva
No abstract text is available yet for this article.
May 9, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28371362/pregnancy-related-acute-aortic-dissection-in-marfan-syndrome-a-review-of-the-literature
#8
REVIEW
Katherine Smith, Bernard Gros
A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified...
April 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28294527/peripartum-type-b-aortic-dissection-in-patients-with-marfan-syndrome-who-underwent-aortic-root-replacement-a-case-series-study
#9
S Sayama, N Takeda, T Iriyama, R Inuzuka, S Maemura, D Fujita, H Yamauchi, K Nawata, M Bougaki, H Hyodo, R Shitara, T Nakayama, A Komatsu, T Nagamatsu, Y Osuga, T Fujii
OBJECTIVE: To investigate pregnancy outcomes, especially the risk of pregnancy-related aortic dissection (AD), in patients with Marfan syndrome (MFS) after prophylactic aortic root replacement (ARR). DESIGN: Retrospective case series study. SETTING: Tertiary perinatal care centre at a university hospital. POPULATION: Pregnant women fulfilling the revised Ghent nosology (2010) criteria for MFS who were managed at our institute...
March 11, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/27843325/the-role-of-the-multidisciplinary-health-care-team-in-the-management-of-patients-with-marfan-syndrome
#10
REVIEW
Yskert von Kodolitsch, Meike Rybczynski, Marina Vogler, Thomas S Mir, Helke Schüler, Kerstin Kutsche, Georg Rosenberger, Christian Detter, Alexander M Bernhardt, Axel Larena-Avellaneda, Tilo Kölbel, E Sebastian Debus, Malte Schroeder, Stephan J Linke, Bettina Fuisting, Barbara Napp, Anna Lena Kammal, Klaus Püschel, Peter Bannas, Boris A Hoffmann, Nele Gessler, Eva Vahle-Hinz, Bärbel Kahl-Nieke, Götz Thomalla, Christina Weiler-Normann, Gunda Ohm, Stefan Neumann, Dieter Benninghoven, Stefan Blankenberg, Reed E Pyeritz
Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS...
2016: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/27825688/aortic-dissection-in-pregnancy-management-strategy-and-outcomes
#11
Jun-Ming Zhu, Wei-Guo Ma, Sven Peterss, Long-Fei Wang, Zhi-Yu Qiao, Bulat A Ziganshin, Jun Zheng, Yong-Min Liu, John A Elefteriades, Li-Zhong Sun
BACKGROUND: Aortic dissection in pregnancy is a rare but lethal catastrophe. Clinical experiences are limited. We report our experience in 25 patients focusing on etiology, management strategies, and outcomes. METHODS: Between June 1998 and February 2015, we treated 25 pregnant women (mean age, 31.6 ± 4.7 years) in whom aortic dissection developed at a mean of 28 ± 10 gestational weeks (GWs). Type A aortic dissection (TAAD) was present in 20 (80%) and type B (TBAD) in 5 (20%)...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27788933/risk-of-aortic-dissection-in-pregnant-patients-with-the-marfan-syndrome
#12
Rafael Kuperstein, Tal Cahan, Rakefet Yoeli-Ullman, Sagit Ben Zekry, Amihay Shinfeld, Michal J Simchen
Patients with Marfan syndrome (MS) face a high risk of aortic dissection during pregnancy. A dilated aortic root (>40 to 45 mm) is considered a relative contraindication for pregnancy. We investigated the risk for aortic dissection and pregnancy outcome in patients with MS. Women with MS who attended our cardiology high-risk pregnancy clinic from 2006 to 2015 were followed clinically and with serial echocardiograms by a multidisciplinary team. Beta blockers were offered and titrated by blood pressure and heart rate...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27695168/epidural-analgesia-complicated-by-dural-ectasia-in-the-marfan-syndrome
#13
Benjamin B Vacula, Chelsea Gray, Michael P Hofkamp, Patrick T Noonan, Russell K McAllister, Kimberly A Pilkinton, Zhiying Diao
Patients with the Marfan syndrome are considered to be high risk during pregnancy and warrant a complete multidisciplinary evaluation. One goal is to minimize hemodynamic fluctuations during labor since hypertensive episodes may result in aortic dissection or rupture. Although they may prevent these complications, neuraxial techniques may be complicated by dural ectasia. The case of a parturient with the Marfan syndrome and mild dural ectasia is presented. During attempted labor epidural placement, unintentional dural puncture occurred...
October 2016: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/27515814/aortic-complications-associated-with-pregnancy-in-marfan-syndrome-the-nhlbi-national-registry-of-genetically-triggered-thoracic-aortic-aneurysms-and-cardiovascular-conditions-gentac
#14
Mary J Roman, Norma L Pugh, Tabitha P Hendershot, Richard B Devereux, Hal Dietz, Kathryn Holmes, Kim A Eagle, Scott A LeMaire, Dianna M Milewicz, Shaine A Morris, Reed E Pyeritz, William J Ravekes, Ralph V Shohet, Michael Silberbach
BACKGROUND: The risk of aortic complications associated with pregnancy in women with Marfan syndrome (MFS) is not fully understood. METHODS AND RESULTS: MFS women participating in the large National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) were evaluated. Among 184 women with MFS in whom pregnancy information was available, 94 (51%) had a total of 227 pregnancies. Among the women with pregnancies, 10 (10...
August 11, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/26903455/genetic-testing-in-marfan-syndrome
#15
REVIEW
Anne H Child, Jose A Aragon-Martin, Karen Sage
Genetic testing is aiding rapid diagnosis of Marfan syndrome as a basis for management of eye, heart and skeletal disease. The affected patient's mutation can be used as a basis for prenatal or postnatal diagnosis of offspring. Preimplantation genetic diagnosis, the technique of choice, can ensure an unaffected pregnancy.
January 2016: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/26885307/stanford-type-a-aortic-dissection-in-a-patient-with-marfan-syndrome-during-pregnancy-a-case-report
#16
Won Ho Kim, Jisue Bae, Seung Won Choi, Jong-Hwan Lee, Chung Su Kim, Hyun Sung Cho, Sangmin M Lee
Aortic dissection during pregnancy is a devastating event for both the pregnant woman and the baby. We report a case of acute aortic dissection (Stanford type A) in a pregnant woman with Marfan syndrome at the 29(th) week of gestation. She underwent a cesarean section followed by an ascending aorta and total arch replacement with cardiopulmonary bypass, without a prior sternotomy. The hemodynamic parameters were kept stable during the cesarean section by using inotropes and vasopressors under transesophageal echocardiography monitoring...
February 2016: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/26604124/pregnancy-and-thoracic-aortic-disease-managing-the-risks
#17
REVIEW
Shaynah Wanga, Candice Silversides, Annie Dore, Vivian de Waard, Barbara Mulder
The most common aortopathies in women of childbearing age are bicuspid aortic valve, coarctation of the aorta, Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, SMAD3 aortopathy, Turner syndrome, and familial thoracic aneurysm and dissection. The hemodynamic and hormonal changes of pregnancy increase the risk of progressive dilatation or dissection of the aorta in these women. The presence of hypertension increases the risk further. Therefore, appropriate preconception counselling is advised. For women who become pregnant, serial follow-up by a specialized multidisciplinary team throughout pregnancy and postpartum period is required...
January 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/26474229/a-woman-with-marfan-syndrome-in-pregnancy-managing-high-vascular-risk-with-multidisciplinary-care
#18
Kentia Naud, Gabrielle Horne, Michiel Van den Hof
BACKGROUND: Women with connective tissue disorders are at risk for cardiovascular complications during pregnancy, but there are no guidelines for pregnant women with aortic root diameter>45 mm or with rapid aortic widening. We describe the issues of practical significance in the management of pregnancy and delivery in a woman with Marfan syndrome (MFS). CASE: A pregnant woman with MFS presented for tertiary care at 26 weeks' gestation. Rapid aortic dilatation triggered a decision to undertake delivery preterm, with a resulting good neonatal outcome...
August 2015: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/26419107/-anesthetic-management-of-a-patient-complicated-with-marfan-syndrome-and-suffering-from-stanford-type-a-aortic-dissection-during-pregnancy
#19
Nako Uozaki, Kaori Mizuno, Yoshito Shiraishi, Matsuyuki Doi, Shigehito Sato
We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery...
April 2015: Masui. the Japanese Journal of Anesthesiology
https://www.readbyqxmd.com/read/26379991/aortic-dissection-in-women-during-the-course-of-pregnancy-or-puerperium-a-report-of-11-cases-in-central-south-china
#20
Guifang Yang, Wen Peng, Qin Zhao, Jing Peng, Xudong Xiang, Xiangping Chai
We have characterized 11 women diagnosed with aortic dissection during the course pregnancy or puerperium by analysis of 1,271 patients enrolled at the Second Xiangya Hospital in central south university of China from 2010 to 2013. The age of these patients ranged from 22 to 39 years old (30.6 ± 5.6 years old). Among which, 5 cases were noted secondary to Marfan syndrome. Three patients were diagnosed at the late stage of pregnancy, 6 cases were in the postpartum stage, and 2 cases occurred in the early stage of pregnancy...
2015: International Journal of Clinical and Experimental Medicine
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