title: Partial zone of apposition closure in atrioventricular septal defect: are papillary muscles the clue.
abstract: BACKGROUND
  Long-term survival after atrioventricular septal defect repair is excellent; however, postoperative left atrioventricular valve regurgitation affects morbidity and quality of life. Left atrioventricular valve regurgitation is the most common reason for reoperation after repair and it is critical that clinicians recognize pathologic mechanisms pre-repair.
  
  
  METHODS
  In this single-center experience, we identified a pattern of left atrioventricular valve abnormality in 5 cases presenting for routine surgical repair between 1 month and 24 years of age. We reviewed two-dimensional and real-time three-dimensional echocardiographic and surgical findings to assess for specific valvar or sub-valve abnormalities, including short chordae, commissural deformities, and an eccentric zone of apposition. Two-dimensional echocardiography was used to assess the degree of preoperative and postoperative left atrioventricular valve regurgitation.
  
  
  RESULTS
  Abnormal features identified included short, thickened chordae, poorly formed superior-mural commissure, and an eccentric zone of apposition. At surgical repair, 2 patients had limited closure of the zone of apposition, as part of a complete repair, and developed only mild left atrioventricular valve regurgitation in short-term follow-up. Two further patients had attempted complete closure of the zone of apposition with moderate postoperative regurgitation ultimately necessitating left atrioventricular valve replacement.
  
  
  CONCLUSIONS
  This uncommon form of atrioventricular septal defect is identifiable with echocardiography and may be associated with significant postoperative regurgitation if the zone of apposition is completely sutured at time of repair. Limited closure of the zone of apposition may improve postoperative regurgitation.
authors.affiliation: Department of Pediatrics, University of Alberta and Stollery Children's Hospital, Walter C. Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada.
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Colen
authors.forename: Nee S
authors.initials: NS
authors.lastname: Khoo
authors.forename: David B
authors.initials: DB
authors.lastname: Ross
authors.forename: Jeffrey F
authors.initials: JF
authors.lastname: Smallhorn
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D019560:Echocardiography, Three-Dimensional; D006343:Heart Septal Defects / Q000601:surgery*; D006801:Humans; D007223:Infant; D008944:Mitral Valve Insufficiency / Q000000981:diagnostic imaging* / Q000517:prevention & control*; D010210:Papillary Muscles; D011183:Postoperative Complications / Q000000981:diagnostic imaging* / Q000517:prevention & control*; D012086:Reoperation; D055815:Young Adult
keywords: 21