title: Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome.
abstract: OBJECTIVE
  We assessed the surgical outcome of descending thoracic aortic aneurysm repair (DTAA) and thoracoabdominal aortic aneurym (TAAA) repair in patients with Marfan syndrome.
  
  
  METHODS
  During a six year period, 206 patients underwent DTAA and TAAA repair. In 22 patients, Marfan syndrome was confirmed. The median age was 40 years with a range between 18 and 57 years. The extend of the aneurysms included 6 DTAA (1 with total arch, 2 with distal hemi-arch), 11 type II TAAA (2 with total arch, 3 with distal hemi-arch), 4 type III and one type IV TAAA. All patients suffered from previous type A (n=6) or type B (n=16) aortic dissection and 15 already underwent aortic procedures like Bentall (n=7) and ascending aortic replacement (n=8). All patients were operated on according to the standard protocol with cerebrospinal fluid drainage, distal aortic and selective organ perfusion and monitoring motor evoked potentials. In patients undergoing simultaneous arch replacement (via left thoracotomy), transcranial Doppler and EEG assessed cerebral physiology during antegrade brain perfusion. In four patients circulatory arrest under moderate hypothermia was required.
  
  
  RESULTS
  In-hospital mortality did not occur. Major postoperative complications like paraplegia, renal failure, stroke and myocardial infarction were not encountered. Mean pre-operative creatinine level was 125mmol/L, which peaked to a mean maximal level of 130 and returned to 92mmol/L at discharge. Median intubation time was 1.5 days (range 0.33-30 days). Other complications included bleeding requiring surgical intervention (n=1), arrhythmia (n=2), pneumonia (n=2) and respiratory distress syndrome (n=1). At a median follow-up of 38 months all patients were alive. Using CT surveillance, new or false aneurysms were not detected, except in one patient who developed a visceral patch aneurysm six years after open type II repair.
  
  
  CONCLUSIONS
  Surgical repair of descending and thoracoabdominal aortic aneurysms provides excellent short- and mid-term results in patients with Marfan syndrome. In this series, a surgical protocol with cerebrospinal fluid drainage, distal aortic and selective organ perfusion and monitoring motor evoked potentials resulted in low morbidity and absent mortality. These outcomes of open surgery should be considered when discussing endovascular aneurysm repair in Marfan patients.
authors: European Vascular Center Aachen-Maastricht, Department of Vascular Surgery, University Hospital Aachen and Maastricht, Germany.
authors: G
authors: G
authors: Mommertz
authors: F
authors: F
authors: Sigala
authors: S
authors: S
authors: Langer
authors: T A
authors: TA
authors: Koeppel
authors: W H
authors: WH
authors: Mess
authors: G W H
authors: GW
authors: Schurink
authors: M J
authors: MJ
authors: Jacobs
mesh_terms: D000328:Adult; D000714:Anastomosis, Surgical; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D004322:Drainage; D004558:Electric Stimulation; D019054:Evoked Potentials, Motor; D005500:Follow-Up Studies; D006801:Humans; D007902:Length of Stay; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality / Q000601:surgery; D008875:Middle Aged; D016343:Monitoring, Intraoperative / Q000379:methods; D012008:Recurrence; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects