LAUSR

Marfan syndrome is a heritable multisystem connective tissue disease and is the most common genetic cause of aortic disease. Guidelines for surgical repair and recommendations for surveillance imaging aim to prevent aneurysm rupture, aortic dissection, and death. Options for proximal aorta repair include the modified Bentall procedure and valve-sparing aortic root repairs. Hemiarch and total arch replacement are options for aortic arch repair, and the elephant trunk procedure is a special two-staged total arch repair facilitating future descending aorta repair. Endovascular repair is not currently considered an acceptable long-term treatment option in Marfan syndrome but may be performed when open repair is not feasible or in the acute setting as a bridge to definitive surgical treatment. After an initial surgery, patients remain at risk for new aortic dissection, propagation of a pre-existing aortic dissection, and new or enlarging aortic aneurysm. Anastomotic pseudoaneurysm is a potential postsurgical complication at multiple sites including proximal and distal aortic anastomoses, coronary anastomoses, and intercostal and visceral artery patches. Patients undergoing endovascular repair are at increased risk of endoleak and aortic dissection. CT and MR angiography are the main imaging modalities for surveillance of the aorta in Marfan syndrome and are capable of demonstrating these complications, as well as expected postsurgical appearances. 4D flow MRI is an emerging technique providing hemodynamic information that may risk-stratify aneurysms better than size alone and help predict future aortic events.