Recurrent Aortic Dissection in Marfan Syndrome

2010 ◽  
pp. 479-483
Author(s):  
Maarten Groenink ◽  
Gary D. Webb ◽  
Barbara J.M. Mulder
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome

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 Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with Marfan syndrome

2017 ◽  
Vol 154 (4) ◽  
pp. 1175-1189.e2 ◽  
Author(s):  
Wei-Guo Ma ◽  
Wei Zhang ◽  
Jun-Ming Zhu ◽  
Bulat A. Ziganshin ◽  
Ai-Hua Zhi ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Type A ◽  
Frozen Elephant Trunk ◽  
Long Term Outcomes ◽  
Elephant Trunk ◽  
Type A Aortic Dissection

scholarly journals Stanford type B aortic dissection associated with pregnancy in patients with Marfan syndrome—A case report and review of the literature

2010 ◽  
Vol 1 (3) ◽  
pp. e180-e183 ◽  
Author(s):  
Hiroshi Nishino ◽  
Kenji Suda ◽  
Akitaka Kuramaoto ◽  
Yoshihiro Honda ◽  
Kiyoko Takemiya ◽  
...  
Keyword(s):  
Case Report ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Type B ◽  
Review Of The Literature ◽  
Type B Aortic Dissection ◽  
Stanford Type B

scholarly journals Type B Aortic Dissection and Distal Aortic Repair in Patients With Marfan Syndrome

2021 ◽  
Vol 74 (3) ◽  
pp. e300-e301
Author(s):  
Karina Newhall ◽  
Thoetphum Benyakorn ◽  
Stephanie Banning ◽  
Palcah Shibale ◽  
Matthew P. Sweet ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Type B ◽  
Type B Aortic Dissection ◽  
Aortic Repair

Common Maternal Genetic Syndromes II: Marfan Syndrome

2019 ◽  
Author(s):  
Ali Said Al-Beshri ◽  
Nathaniel H. Robin
Keyword(s):  
Genetic Testing ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Accurate Diagnosis ◽  
Genetic Syndromes ◽  
Connective Tissues ◽  
Ectopia Lentis ◽  
Surgical Interventions ◽  
Obstetric Management ◽  
Careful Physical Examination

Marfan syndrome is an autosomal dominant syndrome that affects various connective tissues including the aorta and skeletal system. It represent a major cause of aortic dissection in individuals with seemingly unremarkable past medical history, and is the most common cause of aortic dissection in pregnancy. Prompt and accurate diagnosis can be lifesaving. Careful physical examination and detailed personal and family history is vital for clinical evaluation. Genetic testing is often needed for accurate diagnosis but result interpretation might be challenging and genetic counseling is always required. Established guidelines can help navigate the challenges in obstetric management, which may include major surgical interventions during or after pregnancy. This review contains 6 figures, 4 tables, and 40 references. Keywords: Marfan syndrome, FBN1, aortic dissection, dilatation, connective tissue, ectopia lentis, pectus, systemic score, Ghent diagnostic criteria, genetic testing.

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

scholarly journals Aortic diameter predicts acute type A aortic dissection in patients with Marfan syndrome but not in patients without Marfan syndrome

2014 ◽  
Vol 147 (5) ◽  
pp. 1505-1510 ◽  
Author(s):  
Eun Kyoung Kim ◽  
Seung Hyuk Choi ◽  
Kiick Sung ◽  
Wook Sung Kim ◽  
Yeon Hyeon Choe ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Type A ◽  
Aortic Diameter ◽  
Acute Type ◽  
Type A Aortic Dissection

Aortic dissection in children and young adults: diagnosis, patients at risk, and outcomes

2003 ◽  
Vol 13 (4) ◽  
pp. 341-344 ◽  
Author(s):  
Eli Zalzstein ◽  
Robert Hamilton ◽  
Nili Zucker ◽  
Samuel Diamant ◽  
Gary Webb
Keyword(s):  
Chest Pain ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Medical Condition ◽  
Young Patients ◽  
Diagnostic Tools ◽  
Cardiac Defects ◽  
Congenital Cardiac Defects ◽  
Ischemic Bowel ◽  
Presenting Symptoms

Objective: To heighten the awareness of pediatricians and pediatric cardiologists to aortic dissection, a potentially dangerous medical condition. Methods: We reviewed the charts of 13 patients, seen in four medical centers, who suffered acute or chronic aortic dissection over the period 1970 through 2000 whilst under the age of 25 years. Results: There were seven male and six female patients, with the mean age at diagnosis being 12.1 years, with a range from one day to 25 years. Congenital cardiac defects were present in five patients, and Marfan syndrome in four. In three of the patients with congenital cardiac defects, aortic dissection developed as a complication of medical procedures. In three patients, dissection followed blunt trauma to the chest. We could not identify any risk factors in one patient. The presenting symptoms included chest pain in four patients, abdominal pain and signs of ischemic bowel in two, non-palpable femoral pulses in one, and obstruction of the superior caval vein in one. Angiography and magnetic resonance imaging were the main diagnostic tools. Overall mortality was 38%. Only six patients had successful surgical outcomes. Conclusion: Due to the rarity of aortic dissection a high index of suspicion is required to reach the diagnosis in a timely manner. It should be considered in young patients complaining of chest pain in association with Marfan syndrome, anomalies of the aortic valve and arch, and chest trauma.

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