Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve

Eli Zalzstein1, Robert Hamilton, Nili Zucker

  • 1Pediatric Cardiology Unit, Soroka University Medical Center, Beer Sheva, Israel. eliz@bgumail.bgu.ac.il

Insights

Double orifice mitral valve (DOMV) in children is often associated with other heart defects. Most patients with DOMV experienced low long-term morbidity, even with surgical repair of related cardiac issues.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Double orifice mitral valve (DOMV) is a rare congenital heart anomaly.
  • DOMV can present with varying degrees of mitral regurgitation and stenosis.
  • Associated cardiac lesions are common in patients with DOMV.

Purpose of the Study:

  • To investigate the clinical presentation and long-term outcomes of children with double orifice mitral valve.
  • To determine the frequency of surgical intervention specifically for DOMV.
  • To assess the morbidity associated with DOMV in pediatric patients.

Main Methods:

  • Retrospective review of 46 pediatric patients diagnosed with DOMV.
  • Analysis of associated cardiac lesions, presenting symptoms, and interventions.
  • Evaluation of long-term follow-up data regarding morbidity.

Main Results:

  • The median age of the 46 studied children was 2.4 years.
  • Partial atrioventricular septal defect was the most frequent coexisting cardiac lesion.
  • Symptoms correlated with the severity of mitral valve dysfunction (insufficiency/stenosis).
  • Surgical repair of DOMV was uncommon; most interventions addressed associated defects.
  • Long-term morbidity directly attributed to DOMV was found to be low.

Conclusions:

  • Double orifice mitral valve in children frequently occurs with other congenital heart defects, particularly partial atrioventricular septal defects.
  • While symptoms depend on mitral valve function, the long-term outlook for patients with DOMV is generally favorable with low morbidity.
  • Surgical focus is often on associated lesions, with DOMV itself rarely requiring primary surgical correction and having minimal long-term impact.

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