Successful repair of an arch aneurysm with acute aortic dissection in a patient with Marfan Syndrome using a hybrid surgical approach and the STABILISE technique

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.

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

Yonsei Medical Journal ◽

10.3349/ymj.2009.50.2.239 ◽

2009 ◽

Vol 50 (2) ◽

pp. 239 ◽

Cited By ~ 12

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

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

Annals of Vascular Surgery ◽

10.1016/j.avsg.2020.08.132 ◽

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

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

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849230701500527 ◽

2007 ◽

Vol 15 (5) ◽

pp. e63-e65 ◽

Cited By ~ 15

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.

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