Prophylactic aortic root surgery in patients with Marfan syndrome: 10 years' experience with a protocol based on body surface area.
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.
Department of Cardiothoracic Surgery, University Medical Center Groningen, The Netherlands. j.j.j.aalberts@thorax.umcg.nl
Jan J J
JJ
Aalberts
Tjalling W
TW
Waterbolk
J Peter
JP
van Tintelen
Hans L
HL
Hillege
Piet W
PW
Boonstra
Maarten P
MP
van den Berg
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000375:Aging / Q000473:pathology; D000784:Aortic Dissection / Q000517:prevention & control* / Q000601:surgery; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000517:prevention & control* / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D001830:Body Surface Area; D002985:Clinical Protocols; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology / Q000601:surgery*; D008875:Middle Aged; D018579:Patient Selection; D055815:Young Adult