STABILISE Technique for a Non-A Non-B Acute Aortic Dissection in Marfan Syndrome

2021 ◽  
Vol 70 ◽  
pp. 569.e5-569.e10
Author(s):  
Raphael J. Soler ◽  
Michel A. Bartoli ◽  
Jeremy Bourenne ◽  
Alberto Riberi ◽  
David Lagier ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection

scholarly journals Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Mandana Master ◽  
Gavin Day
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection ◽  
Congenital Abnormalities ◽  
Surgical Repair ◽  
Phenotypic Expression ◽  
Sudden Onset ◽  
Lower Segment Caesarean Section ◽  
Type B Dissection ◽  
Type A Dissection

We report a case of acute aortic dissection in a lady of 28 weeks of gestation with undiagnosed Marfan syndrome. The patient had been seen in our antenatal clinics. Her history documented in her pregnancy record was negative for genetic/congenital abnormalities. There was no family history documented. Subsequently, at 28 weeks of gestation, the patient presented with sudden onset chest, jaw, and back pain. Further history revealed that her father had died at the age of 27 of an aortic dissection. Echocardiography showed aortic root dissection with occlusion of aortic branches. She subsequently underwent an emergency lower segment caesarean section followed by surgical repair of type A dissection. A simultaneous type B dissection was managed conservatively. On later examination, our patient fulfilled the diagnostic criteria for phenotypic expression of Marfan syndrome. Genetic testing also confirmed that she has a mutation of the fibrillin (FBN 1) gene associated with the disease.

scholarly journals The Characteristics of Acute Aortic Dissection among Young Chinese Patients: A Comparison between Marfan Syndrome and Non-Marfan Syndrome Patients

2009 ◽  
Vol 50 (2) ◽  
pp. 239 ◽  
Author(s):  
Shih-Hung Tsai ◽  
Yen-Yue Lin ◽  
Chin-Wang Hsu ◽  
Yu-Long Chen ◽  
Min-Tser Liao ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection ◽  
Chinese Patients

Two Surgical Cases of Acute Aortic Dissection in Pregnancy with Marfan Syndrome

2007 ◽  
Vol 15 (5) ◽  
pp. e63-e65 ◽  
Author(s):  
Hidetaka Wakiyama ◽  
Michihiro Nasu ◽  
Hiroshi Fujiwara ◽  
Aki Kitamura ◽  
Yukikatsu Okada
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection ◽  
Deep Hypothermia ◽  
Aortic Root Replacement ◽  
The Other ◽  
Normal Delivery ◽  
First Case ◽  
Type A Dissection ◽  
In Pregnancy

We describe 2 surgical cases of acute aortic type A dissection during pregnancy in women with Marfan syndrome. Both of them underwent emergency aortic root replacement under deep hypothermia; one patient was in her 21st week of pregnancy and the other was treated 1 day after a normal delivery. The patients experienced fair postoperative courses, but intrauterine fetus death was confirmed in the first case.

scholarly journals Acute aortic dissection in a patient with Marfan syndrome during advanced pregnancy

2019 ◽  
Vol 35 (2) ◽  
pp. 499-502
Author(s):  
Beti Kostadinovska ◽  
Aleksandar Nikolic ◽  
Dimche Slaveski ◽  
Milan Milojevic
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection

scholarly journals Operation for Acute Aortic Dissection 13 Years after Operation for Funnel Chest in Marfan Syndrome.

2001 ◽  
Vol 30 (1) ◽  
pp. 33-35 ◽  
Author(s):  
Yuji Kanaoka ◽  
Kazuo Tanemoto ◽  
Takashi Murakami ◽  
Keiichiro Kuroki ◽  
Hitoshi Minami ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection ◽  
Funnel Chest

scholarly journals Inhibition of HIPK2 Alleviates Thoracic Aortic Disease in Mice With Progressively Severe Marfan Syndrome

2021 ◽  
Author(s):  
Cristina I. Caescu ◽  
Jens Hansen ◽  
Brittany Crockett ◽  
Wenzhen Xiao ◽  
Pauline Arnaud ◽  
...  
Keyword(s):  
Protein Kinase ◽  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Thoracic Aortic Aneurysm ◽  
Acute Aortic Dissection ◽  
Transforming Growth Factor ◽  
Transforming Growth Factor Β ◽  
Aortic Disease ◽  
Computational Analyses

Objective: Despite considerable research, the goal of finding nonsurgical remedies against thoracic aortic aneurysm and acute aortic dissection remains elusive. We sought to identify a novel aortic protein kinase that can be pharmacologically targeted to mitigate aneurysmal disease in a well-established mouse model of early-onset progressively severe Marfan syndrome (MFS). Approach and Results: Computational analyses of transcriptomic data derived from the ascending aorta of MFS mice predicted a probable association between thoracic aortic aneurysm and acute aortic dissection development and the multifunctional, stress-activated HIPK2 (homeodomain-interacting protein kinase 2). Consistent with this prediction, Hipk2 gene inactivation significantly extended the survival of MFS mice by slowing aneurysm growth and delaying transmural rupture. HIPK2 also ranked among the top predicted protein kinases in computational analyses of genes differentially expressed in the dilated aorta of 3 MFS patients, which strengthened the clinical relevance of the experimental finding. Additional in silico analyses of the human and mouse data sets identified the TGF (transforming growth factor)-β/Smad3 signaling pathway as a potential target of HIPK2 in the MFS aorta. Chronic treatment of MFS mice with an allosteric inhibitor of HIPK2-mediated stimulation of Smad3 signaling validated this prediction by mitigating thoracic aortic aneurysm and acute aortic dissection pathology and partially improving aortic material stiffness. Conclusions: HIPK2 is a previously unrecognized determinant of aneurysmal disease and an attractive new target for antithoracic aortic aneurysm and acute aortic dissection multidrug therapy.

Heart Transplantation Following Acute Aortic Dissection in an Adolescent with Marfan Syndrome

2021 ◽  
Author(s):  
Gabriella Ricciardi ◽  
Jerome Soquet ◽  
Osama Abou-Arab ◽  
Agnes Mugnier ◽  
Stephanie Delangue ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Heart Transplantation ◽  
Marfan Syndrome ◽  
Acute Aortic Dissection
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