title: Durability of thoracoabdominal aortic aneurysm repair in patients with connective tissue disorders.
abstract: OBJECTIVE
  Thoracoabdominal aortic aneurysm (TAAA) repair is a durable procedure performed with reasonable perioperative mortality and morbidity in patients with atherosclerotic aortic disease. However, the long-term outcome and durability of TAAA repair performed in patients with a connective tissue disorder (CTD) is not well known.
  
  
  METHODS
  The records of 257 patients who underwent TAAA repair at the Johns Hopkins Hospital between January 1992 and December 2001 were reviewed. Survival analysis was performed with Kaplan-Meier analysis, and subgroups were compared with the log-rank test. Multivariable analysis was performed with the Cox proportional hazards model and logistic regression.
  
  
  RESULTS
  Patients with CTD (n = 31) were seen earlier (mean age, 48.6 +/- 2.9 years) than patients without CTD (mean age, 69.1 +/- 0.6 years; P <.0001, Mann-Whitney U test) and had a greater incidence rate of aortic dissection (52% versus 19%; P <.0001, chi(2) test) and extent I or II aneurysm (77% versus 64%; P =.04). The perioperative (30-day) mortality rate was 6.5% in patients with CTD, which was similar to the rest of the cohort (P =.39, Fisher exact test). The incidence rate of paraparesis/paraplegia was 12.9%/6.5% in patients with CTD, and CTD was the only factor predictive of paraparesis (P =.03; odds ratio, 9.3; logistic regression). The cumulative survival rate among the entire cohort was 53.4% +/- 4.4% at 5 years (Kaplan-Meier), and no difference was seen among patients with or without CTD (P =.16, log-rank test) or among different Crawford extents (P =.29). Of the two late (>6 months) deaths in patients with CTD, none were from aortic rupture or dissection, compared with two of 31 late deaths in patients without CTD. Multivariable analysis confirmed that postoperative renal failure (P =.03) predicted mortality but neither CTD (P =.93), nor Crawford extent (P =.21, Cox regression) predicted mortality. Among survivors, no mean difference was found in largest aortic diameter on follow-up imaging in patients with or without CTD (4.7 +/- 0.3 cm versus 4.4 +/- 0.3 cm; P =.47, Mann-Whitney U test). The cumulative graft patency rate, representing long-term graft stability and with death, rupture, dissection, or recurrent aneurysm as endpoints, was 47.5% +/- 4.6% at 5 years (Kaplan-Meier) and was similar in patients with or without CTD (P =.10, log-rank test).
  
  
  CONCLUSIONS
  TAAA repair appears to be a durable operation, with a reasonable 5-year patient survival rate and a low risk of postoperative paraplegia or additional aortic events. Patients with CTD can expect their outcome, including long-term survival and aortic stability, to be similar to patients without CTD.
mesh_terms: D000368:Aged; D017544:Aortic Aneurysm, Abdominal / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D003240:Connective Tissue Diseases / Q000150:complications* / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011183:Postoperative Complications; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors; D014656:Vascular Surgical Procedures / Q000009:adverse effects* / Q000401:mortality