Vascular and/or Interventional RadiologyE2549. Imaging of the Aorta in Marfan Syndrome
Kriger L1, Green D2, Legasto A2, Devereux R.2 1. Morristown Medical Center, New York, NY; 2. Weill Cornell Medical College, New York, NY
Address correspondence to D. Green (firstname.lastname@example.org)
Background Information: Marfan syndrome is a multisystem disease involving the aorta in most patients. This exhibit is a pictorial review of vascular manifestations of Marfan syndrome in both the pre- and postsurgical aorta. Postsurgical complications will focus on complications that are more likely to affect patients with Marfan syndrome compared other patients.
Educational Goals/Teaching Points: Medial degeneration is characterized by disruption and loss of elastic fibers and smooth muscle. Annuloaortic ectasia results in aortic valve regurgitation despite normal valve leaflets. Surgical repair of the dilated aortic root/ascending aorta is usually performed at 5 cm, smaller than for other patients due to a greater risk of dissection. After ascending aorta repair, routine surveillance is necessary to evaluate for later-onset aneurysm or dissection. Coronary button aneurysm and intercostal artery patch aneurysm are two postsurgical complications that affect Marfan syndrome more frequently than other patients.
Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: Key issues include pathophysiology of medial degeneration; presurgical aorta (aneurysm and dissection); and postsurgical aorta (new aneurysm, dissection, anastomotic dehiscence, coronary button aneurysm, and intercostal artery patch aneurysm).
Conclusion: Virtually all patients with Marfan syndrome develop aortic disease at some point in their lifetimes, and they remain at additional risk for further aortic disease after repair. Some of these complications occur at increased frequency in Marfan syndrome but are rare in other patients. This exhibit focuses on complications of the aorta characteristic of Marfan syndrome.