Imaging of the Postsurgical Aorta in Marfan Syndrome

Abstract

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.

Successful orthotopic heart transplantation in a patient with Marfan syndrome

“…MR angiography (MRA) is performed every 6 months to monitor the morphologic change of the aorta. Although special resolution and examination time are inferior to CT, MRA does not utilize ionizing radiation, so it may be better in young patients with Marfan syndrome requiring frequent postsurgical image surveillance . We propose that the reported management strategy can prevent aortic complications in patients undergoing orthotopic heart transplantation with CTD, without subjecting them to the increased morbidity of extensive prophylactic aortic reconstruction.…”

Hemodynamic Profiles Before and After Surgery in Bicuspid Aortic Valve Disease—A Systematic Review of the Literature

“…Prophylactic aortic root replacement is often considered at the time of AVR in patients with aortopathy to mitigate the risk of future aortic dissection. The Modified Bentall procedure is the standard technique, consisting of a synthetic ascending aorta and root graft with a bioprosthetic or mechanical valve (68). A large study has demonstrated the low rate of long-term aortic complications with this technique (69).…”

“…Systolic flow vortices in the Sinus of Valsalva (SOV) occur after peak systole and persist until diastole and are thought to have an important role in valve closure and coronary perfusion ( Figure 5) (75). Valve-sparing aortic root replacement, by either the David or Yacoub technique, involves excision of the native Sinus of Valsalva (68). This loss of sinus architecture abolishes vortical flow and may have longer term consequences.…”

Features of Marfan syndrome not listed in the Ghent nosology – the dark side of the disease

“…Extra-aortic aneurysms and dissections are most commonly noted as a consequence of aortic dissection, in which the dissection extends to more distal vessels [119,121,[125][126][127]. Less well characterized are aneurysms and dissection of the peripheral vessels independent of aortic dissection.…”

“…In Marfan syndrome, the visceral arteries are most commonly involved after descending aortic dissection or graft repair, when the visceral arteries are at risk of dissection and patch aneurysm [127]. Rare cases of visceral artery aneurysms unrelated to dissection have also been reported in Marfan syndrome including in the gastric, celiac artery, splenic, and hepatic arteries, almost all necessitating intervention [120,121,125,137,[165][166][167].…”