title: Aortic Root Replacement for Children With Loeys-Dietz Syndrome.
abstract: BACKGROUND
  Loeys-Dietz syndrome (LDS) is an aggressive aortopathy with a proclivity for aortic aneurysm rupture and dissection at smaller diameters than other connective tissue disorders. We reviewed our surgical experience of children with LDS to validate our guidelines for prophylactic aortic root replacement (ARR).
  
  
  METHODS
  We reviewed all children (younger than 18 years) with a diagnosis of LDS who underwent ARR at our institution. The primary endpoint was mortality, and secondary endpoints included complications and the need for further interventions.
  
  
  RESULTS
  Thirty-four children with LDS underwent ARR. Mean age at operation was 10 years, and 15 (44%) were female. Mean preoperative root diameter was 4 cm. Three children (9%) had composite ARR with a mechanical prosthesis, and 31 (91%) underwent valve-sparing ARR. Concomitant procedures included arch replacement in 2 (6%), aortic valve repair in 1 (3%), and patent foramen ovale closure in 16 (47%). There was no operative mortality. Two children (6%) required late replacement of the ascending aorta, 5 (15%) required arch replacement, 1 (3%) required mitral valve replacement, and 2 (6%) had coronary button aneurysms/pseudoaneurysms requiring repair. Three children required redo valve-sparing ARR after a Florida sleeve procedure, and 2 had progressive aortic insufficiency requiring aortic valve replacement after a valve-sparing procedure. There were 2 late deaths (6%).
  
  
  CONCLUSIONS
  These data confirm the aggressive aortopathy of LDS. Valve-sparing ARR should be performed when feasible to avoid the risks of prostheses. Serial imaging of the arterial tree is critical, given the rate of subsequent intervention.
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Diane
authors.initials: D
authors.lastname: Alejo
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Todd
authors.initials: T
authors.lastname: Crawford
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Narutoshi
authors.initials: N
authors.lastname: Hibino
authors.affiliation: The McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland. Electronic address: lvricella@jhmi.edu.
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
mesh_terms: D001011:Aorta / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000601:surgery; D001807:Blood Vessel Prosthesis; D002648:Child; D005260:Female; D054092:Foramen Ovale, Patent / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D055947:Loeys-Dietz Syndrome / Q000401:mortality / Q000601:surgery*; D008297:Male; D058017:Vascular Grafting