Acute Aortic Dissection Determines the Fate of Initially Untreated Aortic Segments in Marfan Syndrome

Abstract

Background-The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome. Methods and Results-Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y.Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD (P=0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; P=0.0002). Conclusions-The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria. (Circulation. 2013;127:1569-1575.)

Recurrent aortic dissection: a challenging but rare dilemma

“…Initial AD in the entire cohort was Type A in 66%, and RAD was Type B in 53%, but in the MFS group of patients, 79% of initial AD was Type A and 62% of RAD was Type B. This finding is consistent with prior reports of patients with MFS who initially present with Type A AD in that a significant portion of this patient cohort will ultimately need the remaining thoracic aorta replaced at some point for either progressive aneurysmal disease or recurrent distal dissection (2).…”

Type A Aortic Dissection in Marfan Syndrome

“…One of their main conclusions was that the current rate of acute type A dissection surgery in patients with MFS remains very high, ranging between 16% and 35% of all aortic procedures performed in this cohort. [4][5][6][7] Here, we report 25 years of international MFS clinical experience in 2 large cardiac centers with primary surgery for Stanford type A dissection and the management of later aortic complications. Our main observations are the following: (1) Although surgical treatment for type A dissection in patients with MFS has a very low in-hospital mortality risk (3%), it is associated with the high risk of later aortic reinterventions (45%); (2) supracoronary ascending replacement at the time of aortic dissection type A is associated with a high need for root reintervention compared with patients receiving CVG or V-SARR, and leaving the diseased root tissue behind at the time of initial operation leads to high aortic root complication rates; and (3) patients with dissection extending beyond the aortic arch at the initial surgery carry an increased risk of later reinterventions on the descending thoracic aorta.…”

“…Several groups have described their general aortic surgery experience in patients with MFS, [4][5][6][7] but to date no large series on Stanford type A aortic dissection in patients with MFS with long-term aortic outcome have been published. Our pooled databases covering 25 years of aortic dissection type A from 2 tertiary cardiac centers in the United States and Europe provide a unique opportunity to analyze clinical data on individuals with MFS.…”

“…In the most recently published MFS cohorts, Schoenhoff et al 4 and Cameron et al 6 reported series of 86 and 372 patients, respectively, with no in-hospital mortality after elective aortic root surgery. However, there are very few data on outcome after emergency surgery for type A dissection in patients with MFS.…”

Total arch replacement and frozen elephant trunk for type A aortic dissection after Bentall procedure in Marfan syndrome

“…According to recent data from the International Registry on Acute Aortic Dissection (4), acute type A dissection (TAAD) in Marfan patients is usually managed with an emergency root and/or ascending replacement, which is associated with low operative risks but leaves the aortic arch and distal aorta untreated (1). Consequently, reoperation is required to manage the patent false lumen (FL), seen in 29-78% of patients (7)(8)(9)(10)(11)(12), and the expanding aneurysm in the distal aorta (7,13,14). In addition, reoperation for arch dissection was required in 40.9-49% of patients (15,16).…”