title: Early and late results of graft replacement for dissecting aneurysm of thoracoabdominal aorta in patients with Marfan syndrome.
abstract: BACKGROUND
  When treating dissecting aneurysm of the thoracoabdominal aorta surgically in patients with Marfan syndrome, we have usually performed graft replacement- including the entire thoracoabdominal aorta and reconstruction of all visceral branches, even if dilatation is mild in some segments-to avoid further aortic operations in the follow-up period.
  
  
  METHODS
  From October 1999 through July 2011, 20 consecutive patients with Marfan syndrome underwent repair of dissecting aneurysm of the thoracoabdominal aorta (median age, 45 years; range, 19-65 years). All patients underwent surgical intervention with cerebrospinal fluid (CSF) drainage and distal aortic and selective organ perfusion. Deep hypothermia was used in 13 patients for spinal cord protection.
  
  
  RESULTS
  No in-hospital mortality was observed. One patient had temporary spinal cord ischemia but was fully recovered by discharge. Other complications included exploration for bleeding (n=1), prolonged ventilation (n=1), and graft infection (n=1). At a mean follow-up of 54 months (range, 9-129 months), 1 patient had died of interstitial pneumonia at 38 months postoperatively. Survival at 8% years was 91.2±9.0%. Two patients required additional aortic procedures (total arch replacement and aortic valve-sparing surgery). Actuarial rate of freedom from aortic operations at 8 years was 83.9%±10.5%, but no patient needed required repeated thoracotomy for an aortic procedure. Neither false nor patch aneurysms were observed using computed tomography (CT) during follow-up surveillance.
  
  
  CONCLUSIONS
  Graft replacement for dissecting aneurysm of the thoracoabdominal aorta in Marfan syndrome offers good early and long-term results. We believe total aortic replacement including the entire thoracoabdominal aorta and reconstruction of all visceral arteries should be recommended for selected patients with Marfan syndrome.
authors: Department of Cardiovascular Surgery, Kobe University Graduate School of Medicine, Kobe, Japan.
authors: Atsushi
authors: A
authors: Omura
authors: Akiko
authors: A
authors: Tanaka
authors: Shunsuke
authors: S
authors: Miyahara
authors: Toshihito
authors: T
authors: Sakamoto
authors: Yoshikatsu
authors: Y
authors: Nomura
authors: Takeshi
authors: T
authors: Inoue
authors: Takanori
authors: T
authors: Oka
authors: Hitoshi
authors: H
authors: Minami
authors: Kenji
authors: K
authors: Okada
authors: Yutaka
authors: Y
authors: Okita
mesh_terms: D000328:Adult; D000368:Aged; D000714:Anastomosis, Surgical / Q000379:methods / Q000401:mortality; D000784:Aortic Dissection / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D050792:Circulatory Arrest, Deep Hypothermia Induced / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection; D006085:Graft Survival; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality* / Q000601:surgery; D008875:Middle Aged; D011183:Postoperative Complications / Q000401:mortality / Q000503:physiopathology; D011300:Preoperative Care / Q000379:methods; D016019:Survival Analysis; D017741:Survivors; D013908:Thoracotomy / Q000379:methods; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods* / Q000401:mortality; D055815:Young Adult