title: Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results.
abstract: Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management.
  
  
  
  We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention.
  
  
  
  Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years.
  
  
  
  Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Todd
authors.initials: T
authors.lastname: Crawford
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: J Trent
authors.initials: JT
authors.lastname: Magruder
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Diane E
authors.initials: DE
authors.lastname: Alejo
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Narutoshi
authors.initials: N
authors.lastname: Hibino
authors.affiliation: Division of Vascular Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: James
authors.initials: J
authors.lastname: Black
authors.affiliation: Department of Medicine, The McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md. Electronic address: npatel4@jhmi.edu.
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
mesh_terms: D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000601:surgery*; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D006348:Cardiac Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000601:surgery*; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000601:surgery*; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods*; D055815:Young Adult
keywords: Loeys-Dietz syndrome; aortic aneurysm