[Surgical treatment of the aortic root aneurysm related to Marfan syndrome].
OBJECTIVE
To review the experience of surgical treatment of aortic root aneurysm of Marfan syndrome.


METHODS
We The clinical data of 84 Marfan syndrome patients, 61 males and 23 females, aged 35 +/- 12 (5 - 62), 41 cases presenting with aortic dissection (Debakey type I in 32 cases and type II in 9), 52 cases with moderate to severe aortic regurgitation, and 9 cases with moderate to severe mitral regurgitation, 43 cases with cardiac function of class I - II, 30 with class III and 11 with class IV according the New York Heart Association (NYHA) standard, who underwent surgical treatment for aortic root aneurysm with a mean diameter of 68 mm +/- 14 mm, were analyzed. Bentall procedure was performed in 68 cases, Wheat procedure in 6, Cabrol procedure in 5, and aortic valve replacement and aortoplasty in 5. Concomitant procedures included mitral value replacement and mitral valvuoplasty in 3 cases respectively. Urgent surgery was conducted in 28 cases, and elective operation in 56 cases.


RESULTS
There were 3 in-hospital deaths (3.57%). 76 cases were followed up for a mean duration of 55 +/- 31 months. Three patients underwent reoperation. The cardiac function returned to class I - II except for 2 cases that remained at the class III.


CONCLUSIONS
Bentall procedure should be the first choice of the surgery for aortic root aneurysm of Marfan syndrome with a low mortality and a good late outcome.
Zheng
Si-hong
SH
Department of Cardiac surgery, Beijing Anzhen Hospital, Capital University of Medical Sciences, Beijing 100029, China.
Sun
Yan-qing
YQ
Meng
Xu
X
Zhang
Hong
H
Hou
Xiao-tong
XT
Wang
Jian-gang
JG
Gao
Feng
F
D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000209:etiology* / Q000601:surgery*; D001014:Aortic Aneurysm / Q000209:etiology* / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged