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

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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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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Abstract 280: Cocaine-Related Aortic Dissection: Lessons from the International Registry of Acute Aortic Dissection

Circulation Cardiovascular Quality and Outcomes ◽

10.1161/circoutcomes.6.suppl_1.a280 ◽

2013 ◽

Vol 6 (suppl_1) ◽

Author(s):

Joshua H Dean ◽

Patrick O’Gara ◽

Daniel G Montgomery ◽

Santi Trimarchi ◽

Truls Myrmel ◽

...

Keyword(s):

Aortic Dissection ◽

Tobacco Use ◽

Acute Aortic Dissection ◽

Type A ◽

Type B ◽

Cocaine Use ◽

International Registry ◽

Type B Dissection ◽

History Of ◽

Type A Dissection

Background: Acute Aortic Dissection (AAD) associated with cocaine use is a rare event for which only limited case reports and small cohort studies are available. This study compares demographics, history, presenting symptoms and in-hospital outcomes among AAD patients with a history of cocaine use (C+) to those of patients without a history of cocaine use (C-) in a large international registry. Methods: Our study analyzed 3584 patients enrolled in the International Registry of Acute Aortic Dissection from 1996 to 2012. We divided the population based on documented cocaine use and further stratified patients into Type A (33 C+/2332, 1.4%) and Type B (30 C+/1252, 2.4%) dissection cohorts. Results: C+ patients presented at a younger age and were more likely to be male and black for both Type A and B dissections. Type B dissection was more common among C+ patients than in C- patients. Cocaine-related AAD was reported more often at US sites compared to European sites (86.4% 51/63 v. 13.6% 8/63, p<0.001). Tobacco use was more prevalent in the C+ cohort. No differences were seen in history of hypertension, known atherosclerosis or time from symptom onset to presentation between cohorts. Type B C+ patients were more likely to be hypertensive at presentation. Both Type A and Type B C+ cohorts had significantly smaller ascending aortic diameters than C- patients. Acute renal failure was more common in Type A C+ patients but mortality was significantly lower in Type A C+ patients vs type C- patients for reasons than could not be identified from the data base. Conclusions: Cocaine abuse is implicated in approximately 2% of patients with acute aortic dissection. The typical patient is a relatively young male cigarette smoker, who like the majority of patients with AAD, has a history of hypertension. In hospital mortality for cocaine-related Type A dissection is lower than that seen with non-cocaine related type A dissection. The combination of tobacco use, cocaine use, and hypertension may predispose patients to AAD who may otherwise have little risk for the condition.

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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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Abstract TP144: Mechanisms And Prognosis Of Acute Ischemic Stroke Related To Acute Aortic Dissection

Stroke ◽

10.1161/str.44.suppl_1.atp144 ◽

2013 ◽

Vol 44 (suppl_1) ◽

Author(s):

Seung-Jae Lee ◽

Dong-Suk Shim ◽

Si-Ryung Han

Keyword(s):

Ischemic Stroke ◽

Aortic Dissection ◽

Early Onset ◽

Acute Aortic Dissection ◽

Type A ◽

Posterior Circulation ◽

Type B ◽

Delayed Onset ◽

Bilateral Carotid ◽

Type B Dissection

Background: Acute aortic dissection (AD) is one of the lethal cardiac diseases involving the aorta. Although pain is a typical symptom, stroke may not rarely occur with the occlusive dissection of aortic branches or hypotension under the condition of AD. We attempted to explores the clinical features, possible mechanisms and prognosis of acute ischemic stroke (AIS) related to AD Method: Medical records of 278 consecutive patients with AD (165 with type A and 113 with type B dissection) over 11.5 years were retrospectively analyzed for clinical history, CT or MRI findings and outcome. AIS were categorized into early- or delayed-onset stroke. Early-onset stroke was defined as an AIS presented at admission, and delayed-onset stroke was an AIS which were developed during the two months after the first admission. Results: 26 (9.4%) patients experienced an ischemic stroke, which included 22 with type A and 4 with type B dissection. 8 patients (2.9%) including a case of TIA had an early-onset stroke, whereas delayed-onset stroke occurred in 18 patients (6.5%) postoperatively or under medical treatment. Early-onset stroke was all referable to the anterior circulation, predominantly right-sided (87.5%). One or more main branches of the aortic arch were involved in 6 out of 8 patients (75%) with early-onset stroke. Innominate artery was most frequently involved (75.0%). In contrast, delayed-onset stroke affected similarly bilateral carotid territories, and also included lesions in bilateral carotid, posterior-circulation and anterior/posterior-circulation territories. Among the 26 patients, 8 patients (30.8%) expired within 6 months of the disease onset (3 cases from hemispheric stroke with brain herniation, 2 cases from aortic rupture, 2 cases from sepsis with multiple organ failure and a case from mesentery ischemia and renal failure). Additionally, 9 patients (34.6%) remained functionally dependent six months later. Conclusion: AD not infrequently causes AIS with grave prognosis, especially in patients with type A dissection. The presumed mechanisms were aortic branch dissection causing luminal occlusion and emboli from thrombosed vascular lumen and hypotension under the condition of AD.

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Acute aortic syndrome: aortic dissection—descending aortic dissection

ESC CardioMed ◽

10.1093/med/9780198784906.003.0617 ◽

2018 ◽

pp. 2598-2600

Author(s):

Rachel E. Clough ◽

Xun Yuan ◽

Christoph A. Nienaber

Keyword(s):

Aortic Dissection ◽

Acute Coronary Syndromes ◽

Open Surgery ◽

Aortic Rupture ◽

Definitive Treatment ◽

Team Approach ◽

Acute Aortic Syndrome ◽

Type B Dissection ◽

Coronary Syndromes ◽

Type A Dissection

The concept of an acute aortic syndrome (AAS) facilitates early identification of patients with chest pain due to an aortic cause, and expedites implementation of definitive treatment. Important differentials are acute coronary syndromes and pulmonary embolism. Patients with AAS should be evaluated using a combination of blood tests, electrocardiography, and imaging, such as computed tomography, echocardiography, and magnetic resonance imaging. Aortic dissection is the most common form of AAS. Risk factors include congenital factors such as connective tissue disease and bicuspid aortic valve, and acquired disease such as hypertension. Patients with evidence of aortic rupture or end-organ ischaemia require urgent intervention, which in cases of type A dissection is typically open surgery and in type B dissection, endovascular stent graft placement. The optimal treatment of patients with AAS is challenging and requires a multidisciplinary team approach. Further studies are required to fully characterize conditions within the AAS spectrum and to design individualized, patient-centred treatment.

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