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Prophylactic aortic root surgery in patients with Marfan syndrome: 10 years’ experience with a protocol based on body surface area

^a Department of Cardiothoracic Surgery, University Medical Center Groningen, The Netherlands
^b Department of Genetics, University Medical Center Groningen, The Netherlands
^c Trial Coordination Center, University Medical Center Groningen, The Netherlands
^d Department of Cardiology, University Medical Center Groningen, The Netherlands

Received 25 November 2007; received in revised form 21 April 2008; accepted 28 April 2008.

^_* Corresponding author. Address: Department of Cardiothoracic Surgery, University Medical Center Groningen, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands. Tel.: +31 503611719; fax: +31 503611347. (Email: j.j.j.aalberts{at}thorax.umcg.nl).

Background: Current guidelines recommending prophylactic aortic root replacement in Marfan syndrome are based on absolute diameters of the aortic root. However, aortic root diameter is a function of body surface area (BSA). Here, we report our experience with a protocol for prophylactic aortic root replacement based on BSA. Methods: Patients with established Marfan syndrome (Ghent criteria) and without prior aortic surgery were eligible for our study. Aortic root ratio was defined as the ratio between the observed aortic root diameter (as measured during annual echocardiography) and the maximum predicted aortic root diameter as calculated according to age and BSA. Replacement surgery was performed if dilatation of the aortic root during follow-up resulted in an aortic root ratio 1.3. Results: Fifty-three patients fulfilled the entry criteria (24 men/29 women, median age at baseline 27 years, range 18–59 years). During follow-up between 1997 and 2007 (mean 4.7 ± 3.2 years) four patients underwent uncomplicated aortic root replacement; two had an aortic root ratio 1.3 (aortic root diameters were 4.9 and 5.2 cm, respectively), one had aortic root dilatation of 0.4 cm/year and a positive family history for aortic dissection and one had an aneurysm of the ascending aorta as the primary indication. None of the patients in the whole group suffered from type A aortic dissection and there was no mortality. Conclusions: Although numbers are small, our protocol for prophylactic aortic root replacement in patients with Marfan syndrome based on BSA was effective in terms of preventing aortic dissection and mortality.

Key Words: Marfan syndrome • Prophylactic aortic root surgery • Body surface area