• Hiroshi Tanaka, Hitoshi Ogino, Hitoshi Matsuda, Kenji Minatoya, Hiroaki Sasaki, and Yutaka Iba.
• Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan. hirtanak@hsp.ncvc.go.jp
• Ann. Thorac. Surg. 2011 Nov 1; 92 (5): 1646-9; discussion 1649-50.

BackgroundThis study determined the midterm outcome of valve-sparing aortic root replacement for patients with inherited connective tissue disorders.MethodsFrom 1993 to 2008, 94 patients underwent valve-sparing aortic root replacement. Sixty patients (64%), average age 33 years (range, 15 to 61 years), had inherited connective tissue disorders: Marfan syndrome, 54 (92%); Loeys-Dietz syndrome, 5 (8%); and smooth muscle α-actin (ACTA2) mutation in 1. Median preoperative sinus diameter was 52 mm (range, 42 to 76 mm), and moderate/severe aortic regurgitation was present in 14 (23%). Seven (12%, 1993 to 1999) underwent remodeling procedures, and 53 had reimplantation procedures. Cusp repair was performed in 4. Median follow-up was 55 months (range, 1 to 149 months). There were 15 patients in the early term (1993 to 2000) and 45 in the late term (2001 to 2008).ResultsFour late deaths occurred (cardiac, 3; aortic, 1), with 10-year survival of 86%. Rates of freedom from aortic valve replacement at 5 and 10 years were 85% and 58% in remodeling and 96% and 58% in reimplantation. Risk factors for reoperations were postprocedure intraoperative aortic insufficiency greater than mild (p = 0.046), remodeling procedure (p = 0.016), and early term (p = 0.0002). One patient (2%) with none/trivial postprocedure aortic insufficiency required aortic valve replacement. Freedom from reoperation in patients with none/trivial postprocedure aortic insufficiency at 5 and 10 years was 100% and 67%.ConclusionsMeticulous control of aortic insufficiency during operation would bring favorable midterm durability in valve-sparing aortic root replacement using a reimplantation technique, even in patients with inherited connective tissue disorders.Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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