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Marfan pregnancy

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https://www.readbyqxmd.com/read/27843325/the-role-of-the-multidisciplinary-health-care-team-in-the-management-of-patients-with-marfan-syndrome
#1
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
#2
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%)...
November 5, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27788933/risk-of-aortic-dissection-in-pregnant-patients-with-the-marfan-syndrome
#3
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...
September 30, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27695168/epidural-analgesia-complicated-by-dural-ectasia-in-the-marfan-syndrome
#4
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
#5
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 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/26903455/genetic-testing-in-marfan-syndrome
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/26311451/-maternal-and-fetal-outcomes-with-aortic-dissection-in-pregnant-patients-with-marfan-syndrome
#12
Puyu Yang, Jun Zhang, Yanna Li, Hui Wang, Jun Zheng
OBJECTIVE: To evaluate the clinical characteristics of aortic dissection in pregnant patients with Marfan syndrome and the maternal and fetal outcomes in cardiovascular surgery. METHODS: Seven pregnant women with Marfan syndrome with aortic dissection were identified, who were treated in Beijing Anzhen Hospital Affiliated to Capital Medical University between January 2012 and September 2014. Patient charts were reviewed for cardiovascular surgery, occurrence of complications, clinical features and the maternal and fetal outcomes...
May 2015: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/26151074/marfan-syndrome-and-pregnancy-monitoring-and-management
#13
Mechthild Westhoff-Bleck, Denise Hilfiker-Kleiner
No abstract text is available yet for this article.
May 7, 2015: European Heart Journal
https://www.readbyqxmd.com/read/26140774/endograft-as-bridge-to-open-repair-for-ruptured-thoracic-aneurysm-in-a-pregnant-marfan-patient
#14
Randall R De Martino, Jill Johnstone, Elizabeth A Baldwin, Brian C Brost, Heidi M Connolly, Alberto Pochettino
Women with Marfan syndrome are at increased risk of aortic events during pregnancy. We present the case of a ruptured descending thoracic aortic aneurysm in a woman with Marfan syndrome who was 25 weeks pregnant. Emergent intervention was performed using an endovascular repair as a bridge to allow continuation of pregnancy, decreasing fetal morbidity, and allow subsequent later definitive open aortic repair.
July 2015: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/26026739/heart-disease-in-pregnancy
#15
REVIEW
Y Emmanuel, S A Thorne
Cardiac disease is the leading cause of maternal mortality in the developed world. The majority of women with heart disease are able to successfully undergo pregnancy. However, in women with severely impaired ventricular function, severe left heart obstruction, pulmonary hypertension and aortopathy, such as Marfan syndrome, with significant aortic dilatation, pregnancy is associated with a significant risk, and these women should be counselled against pregnancy if there is no option for treatment that reduces risk...
July 2015: Best Practice & Research. Clinical Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/26020727/critical-illness-in-pregnancy-part-ii-common-medical-conditions-complicating-pregnancy-and-puerperium
#16
REVIEW
Kalpalatha K Guntupalli, Dilip R Karnad, Venkata Bandi, Nicole Hall, Michael Belfort
The first of this two-part series on critical illness in pregnancy dealt with obstetric disorders. In Part II, medical conditions that commonly affect pregnant women or worsen during pregnancy are discussed. ARDS occurs more frequently in pregnancy. Strategies commonly used in nonpregnant patients, including permissive hypercapnia, limits for plateau pressure, and prone positioning, may not be acceptable, especially in late pregnancy. Genital tract infections unique to pregnancy include chorioamnionitis, group A streptococcal infection causing toxic shock syndrome, and polymicrobial infection with streptococci, staphylococci, and Clostridium perfringens causing necrotizing vulvitis or fasciitis...
November 2015: Chest
https://www.readbyqxmd.com/read/25788785/cesarean-section-under-epidural-anesthesia-in-a-documented-case-of-ruptured-aneurysm-of-the-sinus-of-valsalva
#17
S R Divakar, Chandrashekhar Singh, Chandra Mohan Verma, Chaitanya D Kulkarni
Ruptured aneurysm of sinus of Valsalva (RSOV) occurring in pregnancy is a rare cardiac anomaly and it may be either congenital or acquired. Congenital sinus of Valsalva aneurysms are commonly associated with other structural defects such as ventricular septal defect (50-55%), aortic regurgitation (AR) (25-35%), bicuspid aortic valve (10-15%) and Marfan's syndrome (10%). RSOV in pregnancy accentuates the hemodynamic stress on maternal cardiovascular system and pose a significant challenge from obstetric anesthesia point of view...
January 2015: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/25722251/endovascular-aortic-repair-combined-with-chimney-technique-in-the-treatment-of-stanford-type-b-aortic-dissection-involving-aortic-arch
#18
Hong Liu, Chang Shu, Xin Li, Tun Wang, Ming Li, Quan-Ming Li, Kun Fang, Shalong Wang
BACKGROUND: In this study, we sought to explore the efficacy of endovascular aortic repair combined with the chimney technique in the treatment of Stanford type B aortic dissection involving the aortic arch. METHODS: Between June 2009 and June 2012, 41 consecutive patients were treated with chimney technique at our institute for aortic arch dissection with the primary entry tear adjacent to the orifices of the supra-aortic arteries. The chimney technique was used to reconstruct the left subclavian artery (n = 5) and the left common carotid artery (n = 34)...
2015: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/25612980/-high-risk-pregnancy-in-a-woman-with-marfan-syndrome-a-bicuspid-aortic-valve-and-a-dilated-aortic-sinus
#19
Kristian Ambjørn Groth, Jacob Raben Greisen, Birgitte Bruun Nielsen, Niels Holmark Andersen
A 29-year-old woman with Marfan syndrome, a bicuspid aortic valve, and a dilated aortic sinus (5.2 cm) presented herself in clinic 14 weeks pregnant. She was advised to discontinue the pregnancy due to risk of dissection; however, she decided to continue. She was treated with labetalol (300 mg/day) to reduce blood pressure and was admitted for bed rest from week 30. Her aortic diameter was assessed by echocardiography every 2nd week and remained unchanged. She was treated with betamethason at week 26 and the child was born by a caesarean section in week 35...
January 26, 2015: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/25524653/perioperative-management-and-outcomes-of-aortic-surgery-during-pregnancy
#20
Martin T Yates, Gopal Soppa, Jeremy Smelt, Nick Fletcher, Jean-Pierre van Besouw, Basky Thilaganathan, Marjan Jahangiri
OBJECTIVE: Pathology of the aortic valve and ascending thoracic aorta is an uncommon but life-threatening complication of pregnancy. Cardiac surgery during pregnancy is known to carry a high risk of mortality to both the mother and fetus. We present our experience of performing aortic surgery during the patients' pregnancy. METHODS: All patients undergoing aortic surgery during pregnancy at St George's Hospital, from January 2004 until October 2013, were identified...
February 2015: Journal of Thoracic and Cardiovascular Surgery
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