title: Congenital supravalvar mitral ring: an underestimated anomaly.
abstract: OBJECTIVE
  Congenital mitral ring is a rare subtype of congenital mitral stenosis. Our objective is to review the anatomic findings and surgical results of this lesion and to identify early predictors of outcome.
  
  
  METHODS
  Clinical reports, echocardiographic studies, cardiac catheterizations, surgical reports, and follow-up data of all patients with mitral ring diagnosed at the Bambino Gesù Hospital were retrospectively reviewed.
  
  
  RESULTS
  Between January of 1987 and June of 2007, a mitral ring was diagnosed in 25 patients (13 male) with a mean age at diagnosis of 36 months. The ring was identified in a single neonate but seemed to develop and progress during infancy. All but 1 patient had associated cardiac anomalies. We identified 2 distinct subtypes: "intramitral ring" in 18 of 25 patients (72%), associated with complex valve pathology and a worse outcome, and "supramitral ring" in 7 of 25 patients (18%), in whom the mitral apparatus was usually normal and the outcome was better. The ring was surgically removed in 13 of 25 patients (52%) (mean Doppler gradient 15 +/- 4 mm Hg). The gradient decreased in 9 of 13 patients (mean diastolic transmitral gradient <or= 5 mm Hg) and has remained stable during a mean follow-up of 90 months. The mean diastolic gradient remained high (>8 mm Hg) in the other 4 patients, all with intramitral ring. All 4 patients underwent repeat mitral valvuloplasty. There were no operative or late deaths.
  
  
  CONCLUSIONS
  Two types of congenital mitral ring, with diverse valve pathology and outcome, were identified. Rarely seen in neonates, the ring develops and often progresses during infancy. Recognition is important because surgical results are better than for other forms of congenital mitral stenosis.
authors.affiliation: Department of Cardiology, Bambino Gesù Pediatric Hospital, Rome, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Toscano
authors.forename: Luciano
authors.initials: L
authors.lastname: Pasquini
authors.forename: Roberta
authors.initials: R
authors.lastname: Iacobelli
authors.forename: Roberto M
authors.initials: RM
authors.lastname: Di Donato
authors.forename: Francesca
authors.initials: F
authors.lastname: Raimondi
authors.forename: Adriano
authors.initials: A
authors.lastname: Carotti
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Di Ciommo
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Sanders
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D008946:Mitral Valve Stenosis / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D012189:Retrospective Studies