Document Detail


Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome.
MedLine Citation:
PMID:  19464442     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Melanie D Everitt; Nelangi Pinto; John A Hawkins; Max B Mitchell; Peter C Kouretas; Anji T Yetman
Publication Detail:
Type:  Journal Article     Date:  2009-04-11
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  137     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-05-25     Completed Date:  2009-06-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1327-32; discussion 1332-3     Citation Subset:  AIM; IM    
Affiliation:
Division of Cardiology, Intermountain West Marfan Cardiology Center, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah 84113, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Aorta, Thoracic / surgery*
Aortic Aneurysm, Thoracic / surgery*
Aortic Valve / surgery*
Child
Craniofacial Abnormalities*
Female
Humans
Male
Marfan Syndrome / surgery*
Mitral Valve / surgery*
Postoperative Complications
Reoperation
Syndrome

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


Previous Document:  Weathering the storm: how can thoracic surgery training programs meet the new challenges in the era ...
Next Document:  Analysis of the US Food and Drug Administration Manufacturer and User Facility Device Experience dat...