Mitral valve surgery in the adult Marfan syndrome patient.
BACKGROUND
Because mitral valve dysfunction in adults with Marfan syndrome is poorly characterized, this study compares mitral valve pathophysiology and morphology with that of myxomatous mitral disease, documents types of mitral valve operations, and assesses long-term survival and durability of mitral valve surgery in Marfan patients.


METHODS
From May 1975 to June 2000, 27 adults with Marfan syndrome underwent mitral valve surgery. Their valve pathophysiology and morphology was compared with that of 119 patients with myxomatous mitral disease undergoing surgery from September 1995 to March 1999. Survival and repair durability were assessed at follow-up.


RESULTS
Compared with myxomatous disease patients, Marfan patients had less posterior leaflet prolapse (44% versus 70%, p = 0.01), more bileaflet (44% versus 28%, p = 0.09) and anterior leaflet prolapse (11% versus 3%, p = 0.07), and presented earlier for surgery (age 41 +/- 12 years versus 57 +/- 13, p < 0.0001). Marfan patients had longer and thinner leaflets. Mitral valve repair was performed less frequently in Marfan (16 of 27, 59%) than myxomatous disease patients (112 of 119, 94%). There were no hospital deaths; at 10 years, survival was 80% and freedom from reoperation 96%, with only 1 reoperation among the 16 repairs.


CONCLUSIONS
Mitral valve pathophysiology and morphology differ between Marfan and myxomatous mitral valve diseases. Valve repair in Marfan patients is durable and gives acceptable long-term results, even in adults who present with advanced mitral valve pathology. With increasing use of the modified David reimplantation operation and sparing of the aortic valve, mitral valve repair is a greater imperative, particularly since we have not had to reoperate on any Marfan patients with reimplantations.
Marfan Syndrome and Connective Tissue Disorder Clinic, Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Sunil K
SK
Bhudia
Richard
R
Troughton
Buu-Khanh
BK
Lam
Jeevanantham
J
Rajeswaran
William R
WR
Mills
A Marc
AM
Gillinov
Brian P
BP
Griffin
Eugene H
EH
Blackstone
Bruce W
BW
Lytle
Lars G
LG
Svensson
D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000601:surgery*; D008875:Middle Aged; D008943:Mitral Valve / Q000473:pathology / Q000503:physiopathology*; D008945:Mitral Valve Prolapse / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome