Aortic valve-sparing surgery in Marfan syndrome.
BACKGROUND
Patients with Marfan syndrome are referred for cardiac surgery due to root aneurysm with or without aortic valve regurgitation. Because these patients are young and frequently present with normal-appearing aortic cusps, valve sparing is often recommended. However, due to the genetic nature of the disease, the durability of such surgery remains uncertain.


METHODS
Between February 2004 and June 2012, 100 patients in our department suffering from aortic aneurysm with aortic valve regurgitation underwent elective aortic valve-sparing surgery. Of them, 30 had Marfan syndrome, were significantly younger (30 +/- 13 vs. 53 +/- 16 years), and had a higher percentage of root aneurysm, compared with ascending aorta aneurysm in their non-Marfan counterparts. We evaluated the safety, durability, clinical and echocardiographic mid-term results of these patients.


RESULTS
While no early deaths were reported in either group, there were a few major early complications in both groups. At follow-up (reaching 8 years with a mean of 34 +/- 26 months) there were no late deaths, and few major late complications in the Marfan group. Altogether, 96% and 78% of the patients were in New York Heart Association functional class I-II in the Marfan and non-Marfan groups respectively. None of the Marfan patients needed reoperation on the aortic valve. Freedom from recurrent aortic valve regurgitation > 3+ was 94% in the Marfan patients.


CONCLUSIONS
Aortic valve-sparing surgery in Marfan symdrome patients is safe and yields good mid-term clinical outcomes.
Department of Cardiac Surgery, Sheba Medical Center, Tel Hashomer, Israel. Eyal.Nachum@sheba.health.gov.il
Eyal
E
Nachum
Amichay
A
Shinfeld
Alexander
A
Kogan
Sergey
S
Preisman
Shany
S
Levin
Ehud
E
Raanani
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001014:Aortic Aneurysm / Q000209:etiology / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000209:etiology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000235:genetics; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult