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

Melanie D. Everitt, Nelangi Pinto, John A. Hawkins, Max B. Mitchell, Peter C. Kouretas, Angela T Yetman

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1327-1333
Number of pages7
JournalJournal of Thoracic and Cardiovascular Surgery
Volume137
Issue number6
DOIs
StatePublished - Jun 1 2009

Fingerprint

Loeys-Dietz Syndrome
Marfan Syndrome
Reoperation
Enzyme Therapy
Mitral Valve
Angiotensin-Converting Enzyme Inhibitors
Aneurysm
Dissection
Aortic Valve Insufficiency
Replantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

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Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome. / Everitt, Melanie D.; Pinto, Nelangi; Hawkins, John A.; Mitchell, Max B.; Kouretas, Peter C.; Yetman, Angela T.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 137, No. 6, 01.06.2009, p. 1327-1333.

Research output: Contribution to journalArticle

Everitt, Melanie D. ; Pinto, Nelangi ; Hawkins, John A. ; Mitchell, Max B. ; Kouretas, Peter C. ; Yetman, Angela T. / Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome. In: Journal of Thoracic and Cardiovascular Surgery. 2009 ; Vol. 137, No. 6. pp. 1327-1333.
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