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

Study Questions:

In patients with Marfan syndrome undergoing initial aortic surgery, how often is redo aortic surgery needed?

Methods:

This study retrospectively examined 86 consecutive patients with Marfan syndrome at a single institution following initial aortic surgery over a mean follow-up of 8.8 ± 6.8 years. The frequency and patterns of subsequent aortic surgery were evaluated.

Results:

Mean age was 35 years, and 57% were male. The indication for initial surgery included acute aortic dissection (AAD) in 31 (36%) cases (24 type A, 7 type B), and chronic aortic dilation in 55 patients (64%). Initial presenting lesions included the aortic root, ascending aorta, or aortic arch in 79 cases (92%), with primary involvement of the descending or abdominal aorta in seven patients (8%). In patients with type A AAD, 67% received a composite graft and 33% had a supracoronary replacement. In patients undergoing elective surgery, 43% received a composite graft and 50% received a valve-sparing root replacement. Mortality at 1 month, 6 months, 1 year, and at end of follow-up was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Between patients with and without AAD on presentation, secondary complete aortic arch replacement was needed in 36% (11/31) and 6% (3/55) of patients, respectively (p < 0.001). In patients who did not present with AAD, 11% (6/55) needed redo surgery on initially nontreated aortic segments, which in 5/6 cases was due to type B AAD during the follow-up period. In patients initially presenting with AAD, redo surgery on initially nontreated aortic segments was needed in 48% (15/31) of patients, including 42% and 86% of patients initially presenting with type A and type B AAD, respectively (p < 0.001).

Conclusions:

AAD is a common initial presentation in patients with Marfan syndrome undergoing aortic surgery. Further, a large number of patients with Marfan syndrome undergoing initial aortic surgery required repeat aortic surgery, particularly those who initially presented with an AAD.

Perspective:

This study highlights the frequent need for redo aortic surgery in patients with Marfan syndrome following initial aorta surgery. While the need for redo surgery was relatively lower in patients initially presenting for surgery due to chronic aortic dilation (11%), redo surgery was needed in 48% of patients initially presenting with AAD, including 42% of patients with initial type A AAD and 86% of patients with initial type B AAD. These findings suggest a need for particularly close follow-up in patients with Marfan syndrome following initial aortic surgery for AAD.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease, CHD & Pediatrics and Quality Improvement

Keywords: Follow-Up Studies, Aortic Aneurysm, Thoracic, Perioperative Care, Dilatation, Pathologic, Marfan Syndrome, Cardiovascular Diseases


< Back to Listings