Beyond the root: Dilatation of the distal aorta in the marfan syndrome

Peter M Engelfriet ¹, Eric Boersma ², Jan G Tijssen ¹, Berto J Bouma ¹ and Barbara J.M. Mulder ^1*

¹ Academic Medical Centre, Amsterdam, Netherlands
² Thoraxcentre, ErasmusMC, Rotterdam, Netherlands

^* To whom correspondence should be addressed. E-mail: b.j.mulder{at}amc.uva.nl.

Accepted 10 February 2006

Abstract

Objective To investigate dilatory changes of the aorta distal from the root in patients with the Marfan syndrome.

Methods and results We analyzed data of 268 Marfan patients who were enrolled in the Euro Heart Survey on adult congenital heart disease. Data used for this study included baseline characteristics, diameters at 4 levels of the aorta, and events during follow-up (dissection, aortic repairs, death). At inclusion, 26 patients had a previous dissection, and 53 patients without a previous dissection had undergone elective aortic repair, thus leaving 189 patients without previous dissection or repair. During follow-up (median 5.4 years), four patients died. A total of 46 "aortic events" (dissection or elective surgery) occurred in 45 patients, involving the distal aorta in 14 patients (31%). Baseline aortic diameters at the levels distal to the root (arch, descending aorta, abdominal aorta) were greater in patients with than in those without a previous elective aortic root intervention: median 26 mm vs. 24 mm (P=0.01), 25 mm vs. 20 mm (P < 0.01), and 20 mm vs. 17 mm (P < 0.01), respectively. Multivariate analysis showed that a previous elective aortic intervention was associated with a four-fold increased risk of dilatation of the distal aorta, after adjustment for age and sex (P < 0.01). In patients without a previous intervention, the baseline diameter of the descending aorta was found to be an independent predictor of aortic events (hazard ratio 3.0 per quartile; 95%CI 1.5-5.9; P=0.002). Cause for concern is our finding that complete measurements of the aorta (at least 1 measurement at each level at baseline or during follow-up) were available for only 37% of the patients.

Conclusions Almost one out of every three aortic events occurring during follow-up of these patients involved the distal aorta. After elective aortic root replacement, a dilated distal aorta is more common than before. Moreover, an increased diameter of the descending aorta is associated with a higher risk of aortic events in patients without previous dissection or aortic root replacement, independent from the diameter of the aortic root. Careful monitoring of the entire aorta is essential for the optimal management of Marfan patients, especially after elective surgery, but is insufficiently performed in Europe.

Keywords: aortic dilatation, distal aorta, euro heart survey, marfan syndrome, aortic diameters

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