Invited commentary.

Department of Cardiothoracic Surgery, Stanford University Medical Center, Falk CVRC, 300 Pasteur Dr, Stanford, CA 94305, USA.
The Annals of thoracic surgery (Impact Factor: 3.65). 05/2012; 93(5):1501. DOI: 10.1016/j.athoracsur.2012.02.043
Source: PubMed
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    ABSTRACT: We undertook a retrospective study of the pattern of reoperations in surgical patients with Marfan disease. Between 1985 and 2008, 83 Marfan patients (60 males, 23 females) underwent 155 aortic operations in our institution. Twenty-eight patients had acute dissection (22 type A, 6 type B), and two had aortic rupture. Mean age at initial operation was 32±13 years. Operations included valve-sparing or Bentall aortic root repair, and ascending aorta, arch, descending thoracic, thoracoabdominal aorta, and infrarenal aortic replacement. Sixty-one patients whose initial operation was elective (Group I) were compared with 22 patients with initial emergency surgery (Group II). Overall, 81/83 patients ultimately underwent root/ascending repair: 64% initially and 36% at reoperation. Operative mortality in Group I was 1.6% for both initial operations and reoperations vs 9.0% and 0% in Group II. Significant differences between Group I and Group II patients included: total reoperations (1 vs 3, p=0.05); arch operations (0 vs 1, p=0.003); descending thoracic aortic operations (0 vs 0.5, p=0.003); and total aortic segments replaced (1.6±1.0 vs 2.4±1.1, p=0.001). Survival at 5 and 10 years did not differ between Group I and II patients (87% and 71% vs 82% and 56%, p=0.19). Although reoperation occurs in about half of surgical Marfan patients, reoperative mortality is low. Patients with initial elective procedures fare better than those with initial emergency surgery: they have fewer subsequent operations, fewer aortic segments replaced, and trend toward improved survival. Elective root replacement should be seriously considered in any Marfan patient with significant root dilatation.
    The Annals of thoracic surgery 03/2012; 93(5):1496-501. DOI:10.1016/j.athoracsur.2011.12.068 · 3.65 Impact Factor
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    ABSTRACT: Development of surgical therapy for aortic aneurysms and dissections has led to treatment of the life-threatening cardiovascular complications associated with Marfan syndrome. The present study determines the effect of surgical therapy on the life expectancy of patients with Marfan syndrome and the clinical course of these patients after aortic aneurysm repair. Medical records were reviewed on 192 patients with Marfan syndrome who underwent aortic aneurysm repair during the past 26 years; 103 patients were interviewed, and complete preoperative and postoperative medical information was obtained. Survival curves were generated, and data were analyzed. The median cumulative probability of survival was 61 years, significantly increased compared with the median survival of 47 years for patients with Marfan syndrome determined 30 years ago (P < .0006). The majority of patients (53%) had second surgeries to repair subsequent aneurysms or dissections at other sites, the vast majority of which involved the aorta. The most common pattern of aneurysm repair was proximal ascending aortic aneurysm repair, followed by descending thoracic aneurysm surgery. The following variables predicted patients requiring second vascular surgeries: presence of acute or chronic dissection at the time of the first surgery, hypertension after the first surgery, and a history of smoking. The life expectancy of patients with Marfan syndrome undergoing surgical repair of aortic aneurysms has improved and is consistent with increased survival. After initial repair of an ascending aortic aneurysm, a significant number of patients have subsequent surgeries at other sites throughout the aorta, indicating Marfan syndrome is a disease involving the entire aorta. Patients who had a dissection at the time of the first aortic surgery were more likely to require subsequent aortic surgery than were patients who underwent prophylactic composite graft repair of an aortic aneurysm.
    Circulation 03/1995; 91(3):728-33. DOI:10.1161/01.CIR.91.3.728 · 14.95 Impact Factor
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    ABSTRACT: We assessed the surgical outcome of descending thoracic aortic aneurysm repair (DTAA) and thoracoabdominal aortic aneurym (TAAA) repair in patients with Marfan syndrome. 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. 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. 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.
    European journal of vascular and endovascular surgery: the official journal of the European Society for Vascular Surgery 03/2008; 35(2):181-6. DOI:10.1016/j.ejvs.2007.10.013 · 3.07 Impact Factor