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Axial computed tomography (CT) image through ascending (AA) and descending (DA) thoracic aorta showing the aneurysmal dilatation of the ascending aorta (top-right arrow) with intimal flap (type A dissection, top-left arrow) and pericardial effusion (middle arrow). Compare the diameter of ascending aorta with descending aorta (bottom arrow).
Source publication
Marfan syndrome is a heritable disorder of the connective tissue that affects many systems of the body. However, the most serious complication in patients with Marfan syndrome is progressive enlargement of the aortic root, which may lead to aortic dissection, rupture, or aortic regurgitation. Prevention of these life threatening complications is ve...
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Marfan syndrome (MS) is an autosomal dominant connective tissue disorder affecting mainly cardiovascular system, eyes and skeleton. However, the most serious complication in patients with MS is progressive aortic root dilatation, aortic dissection or regurgitation. We have reviewed all patients with MS in our hospital over a six year period to dete...
Acute type A aortic dissection is a surgical emergency. Unfortunately, the early outcome of surgical correction has not improved significantly over the past 20years (Chiu and Miller, 2016Chiu and Miller (2016) ¹). There is still conflict regarding operative extent and optimal conduct of the operation (Chiu and Miller, 2016Chiu and Miller (2016) ¹)....
Background: Cardiovascular complications account for a significant proportion of the shortened lifespan of Marfan syndrome (MFS) patients, with aortic dissection being the most dreadful complication. The aortic root dilates initially in MFS patients, and given its important hemodynamic role, this can lead to aortic regurgitation and poses a substan...
Objective
This study aimed to evaluate the changes in postoperative aortic regurgitation (AR) and determine the predictors of significant AR and root reoperation after ascending aortic replacement (AAR) in patients with acute type A aortic dissection.
Methods
From January 1995 to December 2017, 271 consecutive patients underwent valve/root-preserv...
Citations
Marfan syndrome (MFS) is a rare autosomal dominant connective tissue disorder that can affect many organ systems of the body. MFS is inherited as a dominant trait carried by the FBN1 gene, which encodes the connective protein fibrillin-1 [5]. The diagnosis is based on internationally defined classification criteria [9]. The majority of clinical manifestations of MFS increase with age, and diagnosis in early childhood may be difficult [6]. A follow-up monitoring in case of clinical suspicion of MFS is mandatory to initiate therapeutical interventions in time.
