Document Detail

Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome.
MedLine Citation:
PMID:  18069021     Owner:  NLM     Status:  MEDLINE    
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. CONCLUSION: 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.
G Mommertz; F Sigala; S Langer; T A Koeppel; W H Mess; G W H Schurink; M J Jacobs
Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery     Volume:  35     ISSN:  1532-2165     ISO Abbreviation:  Eur J Vasc Endovasc Surg     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-01     Completed Date:  2008-02-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9512728     Medline TA:  Eur J Vasc Endovasc Surg     Country:  England    
Other Details:
Languages:  eng     Pagination:  181-6     Citation Subset:  IM    
European Vascular Center Aachen-Maastricht, Department of Vascular Surgery, University Hospital Aachen and Maastricht, Germany.
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MeSH Terms
Anastomosis, Surgical
Aortic Aneurysm, Thoracic / etiology,  mortality,  surgery*
Electric Stimulation
Evoked Potentials, Motor
Follow-Up Studies
Length of Stay
Marfan Syndrome / complications*,  mortality,  surgery
Middle Aged
Monitoring, Intraoperative / methods
Retrospective Studies
Severity of Illness Index
Time Factors
Treatment Outcome
Vascular Surgical Procedures* / adverse effects

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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