title: Simplified mitral valve repair in pediatric patients with connective tissue disorders.
abstract: In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.
  
  
  
  Retrospective review of clinical and echocardiographic data of all pediatric patients (age < 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).
  
  
  
  Eighteen children who underwent surgery for severe MR and bileaflet prolapse were identified. All were treated with ring annuloplasty and Alfieri edge-to-edge repair. Median age and weight were 8.2 years (range, 0.4-17.2 years) and 24.9 kg (5.6-63.3 kg), respectively. Median left ventricular end diastolic dimension median z score was 4.9 (2.1-11.9). One patient died (5.6%), and there were no other major complications. Among survivors, 94.4% had mild regurgitation or less, with no stenosis or SAM at median clinical follow-up of 2.4 years (range, 0-13.9 years). Median left ventricular end-diastolic dimension z score regressed to 1.3 (-0.5 to 4.3).
  
  
  
  In pediatric patients with CTD and severe MR, a simplified approach is associated with intermediate-term competence, absence of SAM or significant stenosis, and regression of left ventricular enlargement.
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md. Electronic address: lvricella@jhmi.edu.
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: William A
authors.initials: WA
authors.lastname: Ravekes
authors.affiliation: Center for Inherited Cardiovascular Diseases, IRCCS Foundation, University Hospital Policlinico San Matteo, Pavia, Italy.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Arbustini
authors.affiliation: Division of Cardiac Surgery, Duke University, Durham, NC.
authors.forename: Robert D B
authors.initials: RD
authors.lastname: Jaquiss
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Constantine
authors.initials: C
authors.lastname: Mavroudis
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Marshall L
authors.initials: ML
authors.lastname: Jacobs
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Narutoshi
authors.initials: N
authors.lastname: Hibino
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
mesh_terms: D002648:Child; D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000150:complications*; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D007223:Infant; D008297:Male; D008943:Mitral Valve / Q000000981:diagnostic imaging / Q000601:surgery*; D058386:Mitral Valve Annuloplasty / Q000379:methods*; D008944:Mitral Valve Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
keywords: connective tissue disorders; mitral valve repair