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Ann Thorac Surg 2009;87:1344-1350. doi:10.1016/j.athoracsur.2009.01.073
© 2009 The Society of Thoracic Surgeons

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Duke E. Cameron
Diane E. Alejo
Nishant D. Patel
Lois U. Nwakanma
Luca A. Vricella
Jason A. Williams
Brian T. Bethea
Torin P. Fitton
Vincent L. Gott
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J. Maxwell Chamberlain Memorial Paper for Adult Cardiac Surgery

Aortic Root Replacement in 372 Marfan Patients: Evolution of Operative Repair Over 30 Years

Duke E. Cameron, MD*, Diane E. Alejo, BA, Nishant D. Patel, BA, Lois U. Nwakanma, MD, Eric S. Weiss, MD, Luca A. Vricella, MD, Harry C. Dietz, MD, Philip J. Spevak, MD, Jason A. Williams, MD, Brian T. Bethea, MD, Torin P. Fitton, MD, Vincent L. Gott, MD

Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland

Accepted for publication January 30, 2009.

* Address correspondence to Dr Cameron, Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, 600 N Wolfe St, Blalock 618, Baltimore, MD 21287 (Email: dcameron{at}jhmi.edu).

Presented at the Forty-fourth Annual Meeting of The Society for Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008. Winner of the J. Maxwell Chamberlain Memorial Paper for Adult Cardiac Surgery.

Background: We reviewed the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients at our institution.

Methods: A retrospective clinical review of Marfan patients undergoing ARR at our institution was performed. Follow-up data were obtained from hospital and office records and from telephone contact with patients or their physicians.

Results: Between September 1976 and September 2006, 372 Marfan syndrome patients underwent ARR: 269 had a Bentall composite graft, 85 had valve-sparing ARR, 16 had ARR with homografts, and 2 had ARR with porcine xenografts. In the first 24 years of the study, 85% received a Bentall graft; during the last 8 years, 61% had a valve-sparing procedure. There was no operative or hospital mortality among the 327 patients who underwent elective repair; there were 2 deaths among the 45 patients (4.4%) who underwent emergent or urgent operative repair. There were 74 late deaths (70 Bentalls, 2 homograft, and 2 valve-sparing ARRs). The most frequent causes of late death were dissection or rupture of the residual aorta (10 of 74) and arrhythmia (9 of 74). Of the 85 patients who had a valve-sparing procedure, 40 had a David II remodeling operation; there was 1 late death in this group, and 5 patients required late aortic valve replacement for aortic insufficiency. A David I reimplantation procedure using the De Paulis Valsalva graft has been used exclusively since May 2002. All 44 patients in this last group have 0 to 1+ aortic insufficiency.

Conclusions: Prophylactic surgical replacement of the ascending aorta in patients with Marfan syndrome has low operative risk and can prevent aortic catastrophe in most patients. Valve-sparing procedures, particularly using the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.







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