title: Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?
abstract: We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to 7 years, but new dissection developed in 5 (33.3%) of the 15 patients who were followed up for >7 years after the root repair. Actuarial survival including operative mortality was 88.1 and 65.0% at 10 and 20 years, respectively; groups A and B did not significantly differ. Rates of freedom from all-cause death, new dissection or repeated aortic surgery were 60.1, 44.5 and 26.0% at 5, 10 and 15 years, respectively. Group A was significantly better than group B. Prophylactic aortic root repair apparently reduces the likelihood of overall adverse events, but it cannot guarantee the prevention of further aortic dissection. A multidisciplinary approach is needed for patients with Marfan syndrome.
authors.affiliation: Department of Cardiovascular Surgery, Keio University, Tokyo, Japan. hideyuki@sc.itc.keio.ac.jp
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Shimizu
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Kasahara
authors.forename: Atsushi
authors.initials: A
authors.lastname: Nemoto
authors.forename: Kentaro
authors.initials: K
authors.lastname: Yamabe
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ueda
authors.forename: Ryohei
authors.initials: R
authors.lastname: Yozu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001014:Aortic Aneurysm / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007564:Japan; D053208:Kaplan-Meier Estimate; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D012008:Recurrence; D012086:Reoperation; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D055815:Young Adult