HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John A. Hawkins Max B. Mitchell Peter C. Kouretas Permission Requests Google Scholar Articles by Everitt, M. D. Articles by Yetman, A. T. PubMed Articles by Everitt, M. D. Articles by Yetman, A. T. Related Collections Congenital - acyanotic Great vessels Valve disease

J Thorac Cardiovasc Surg 2009;137:1327-1333
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome

^a Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
^b Intermountain West Marfan Cardiology Center, Division of Cardiovascular Surgery at Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
^c The Children's Hospital, University of Colorado, Denver, Colo

Received for publication June 19, 2008; revisions received September 27, 2008; accepted for publication February 1, 2009.

^_* Address for reprints: Anji T. Yetman, MD, Professor of Pediatrics, Director, Intermountain West Marfan Cardiology Center, Division of Cardiology, Primary Children's Medical Center, 100 N. Mario Capecchi Drive, Salt Lake City, UT 84113. (Email: Angela.Yetman{at}imail.org).

Objective: This study was undertaken to assess the frequency and outcome of cardiovascular surgery in children with Marfan or Loeys-Dietz syndrome.

Methods: A retrospective review from 2 regional Marfan subspecialty clinics was performed. Between 1997 and 2007, 204 children with Marfan syndrome and 17 children with Loeys-Dietz syndrome were followed serially. Of these patients, 35 were identified who had undergone cardiovascular surgery at 18 years of age or less. Demographic, echocardiographic, and surgical data were collected.

Results: Surgery was performed at a median of 3 years (0–15 years) after diagnosis and a mean age of 11.5 ± 6.2 years. Aortic root replacement was the initial surgery in 30 patients, and mitral valve surgery was the initial surgery in 8 patients, with 3 patients undergoing both. Aortic root replacement was performed using a composite root replacement in 9 patients and valve-sparing techniques in 21 patients (remodeling in 8 patients and reimplantation in 13 patients). Eight patients underwent reoperation at a mean of 4.7 ± 3.0 years after aortic surgery: 3 for aortic insufficiency, 2 for dissection, 2 for valve thrombosis, and 1 for a distal aneurysm. Adverse outcomes included reoperation in 8 patients, aneurysm in 1 patient, and death due to dissection or stroke in 3 patients. Variables associated with an adverse outcome included preoperative aortic insufficiency, valve replacement, and absence of angiotensin-converting enzyme inhibitor therapy.

Conclusion: Patients with Marfan or Loeys-Dietz syndrome requiring surgery during childhood have a favorable long-term outcome. Those undergoing valve-sparing root replacement or mitral valve repair have a low risk for reoperation. Postoperative angiotensin-converting enzyme inhibitor therapy confers clinical benefit.