Stent Grafting of Dissected Descending Aorta in Patients With Marfan's Syndrome

Abstract

Endovascular stent grafting of the dissected DTA is feasible in selected Marfan patients with low mortality and morbidity rates. Nevertheless, the rate of primary and secondary endoleak is high. Close imaging surveillance is crucial to detect secondary aortic complications and to assess long-term results.

Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome

“…Marcheix et al showed a similarly high primary treatment failure rate to our study, with primary endoleak occurring in 33% of patients, secondary endoleak occurring in another 33%, and an additional two patients requiring subsequent intervention for unrelated aortic pathology. 12 There was a 60% mortality in the primary endoleak group, and eight of 15 patients in this study required subsequent operative intervention, mirroring the results in our series.…”

“…However, the use of endovascular therapy for patients with Marfan syndrome is still controversial and has been reported only in small series and isolated case reports. [6][7][8][9][10][11][12] To our knowledge, our analysis represents the largest series published to date. In this series, TEVAR was performed in 15 patients and EVAR in one patient.…”

“…Selected use of endovascular therapies in the Marfan population has been described in several case reports and series. [6][7][8][9][10][11][12] The short-and longterm benefits are unproven and this practice remains controversial. In the 2008 Expert Consensus Document on the Treatment of Descending Thoracic Aortic Disease Using Endovascular Stent Grafts, The Society of Thoracic Surgeons Endovascular Surgery Task Force stated that "stent grafting in patients with Marfan syndrome or any other known connective tissue disorder is not recommended [as] there is limited information regarding the impact of persistent radial forces of a stent graft in the abnormal and weak aorta."…”

Endovascular Repair of Descending Thoracic Aorta in Loeys-Dietz II Syndrome

“…Cardiac auscultation demonstrated a systolic ejection click and crisp mechanical second sound. The patient did not M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT 4 have orbital hypertelorism or bifid uvula. Medications included losartan, simvastatin, aspirin and warfarin.…”

Acute Aortic Syndrome: Medical Management

“…Current observational evidence suggests that TEVAR improves survival in complicated distal dissection [ 21 ]. In patients with connective tissue disease, however, remodeling is achieved less frequently and endovascular strategies are discouraged or considered as bridging to definitive open repair [ 36 ].…”