Abstract and Introduction
Abstract
Aims To better characterize patients with Marfan syndrome who have survived an acute aortic dissection and to estimate the risks of events in the descending aorta. Up until now, this portion of the aorta has not been well studied but is gaining importance due to improved patient survival.
Methods and results We report a retrospective cohort of 100 Marfan patients who survived an aortic dissection. Dissection occurred in either the ascending aorta (AscAo) (n = 37), the descending aorta (DescAo) (n = 20), or both (As + DescAo, n = 43). During a mean follow-up of 9.8 ± 6.0 years (complete for 88% of the patients), 17 patients died and 52 had a clinical event (new aortic dissection, surgery, ischaemia, haemorrhage), 60% of which involved the descending aorta. Event-free survival was similar whatever the location of the aortic dissection. However, a better event-free survival was observed when no dissected portion of the aorta remained after surgery, which was the case in 62% (23/37) of the AscAo patients (30% incurred an event vs. 86%; P = 0.008 by log-rank test).
Interestingly, the diameter of the ascending aorta was below the surgical threshold in 60% of the patients who incurred a dissection of the descending aorta, and within the normal range in 25%.
Conclusion The descending aorta may dissect whatever the diameter of the ascending aorta. The descending aorta is the location of most late clinical events after any dissection of the aorta. The rate of clinical events is much lower when all the dissected aorta has been removed in patients with AscAo dissection.
Introduction
Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a prevalence of around 1/5000, mostly related to mutations in the gene coding for fibrillin 1 (FBN1). Fibrillin 1 is a ubiquitous protein and FBN1 mutations induce modifications in many organs. The cardinal features of the MFS involve the ocular, cardiovascular, and skeletal systems, but aortic enlargement and dissection, mostly of the ascending aorta, was the primary cause of early death before 1970. However, the life expectancy of MFS patients has improved by 30 years over the last 30 years as the result of medical therapy and monitoring of the aortic root diameter allowing for preventive aortic root replacement. Therefore, complications other than those occurring in the ascending aorta are becoming more important in patients with the MFS.
It is well recognized that both the ascending and descending aorta are abnormal in the MFS: fibrillin 1 is present in the entire aortic wall—both ascending and descending—and the aorta has been repeatedly shown to possess abnormal elastic properties at the abdominal level. Dilatation or dissection of the descending aorta is a minor criterion in the Ghent nosology. However, although there is abundant literature regarding the early clinical course after aortic dissection of the proximal aorta in the MFS, there are still little data about dissection of the descending aorta and long-term follow-up after aortic dissection of any type.
Here, we take advantage of a large Marfan population followed according to modern standards, to study patients surviving a first episode of aortic dissection on either the ascending or the descending aorta.