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Simplified mitral valve repair in pediatric patients with connective tissue disorders

Luca A Vricella1, William A Ravekes1, Eloisa Arbustini2

  • 1Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.

The Journal of Thoracic and Cardiovascular Surgery
|January 21, 2017
PubMed

Insights

A simplified surgical repair effectively treats severe mitral regurgitation in pediatric patients with connective tissue disorders, preventing complications and reducing heart enlargement. This approach offers intermediate-term competence and positive cardiac remodeling.

Area of Science:

  • Pediatric Cardiology
  • Cardiac Surgery
  • Connective Tissue Disorders

Background:

  • Pediatric patients with connective tissue disorders (CTDs) often present with severe mitral regurgitation (MR) and bileaflet prolapse.
  • Aortic root enlargement is a less common cardiac manifestation in this population.
  • Systolic anterior motion (SAM) is a potential complication that needs to be addressed.

Purpose of the Study:

  • To evaluate the efficacy of a simplified surgical repair for severe MR in pediatric patients with CTDs.
  • To assess the prevention of SAM and other complications associated with this repair technique.
  • To determine the intermediate-term outcomes and cardiac remodeling following the simplified repair.

Main Methods:

  • Retrospective review of clinical and echocardiographic data from pediatric patients (age < 18 years) with CTD and MR.
  • Analysis of data from three institutions between 2000 and 2014.
  • Patients underwent a simplified repair involving ring annuloplasty and Alfieri edge-to-edge repair.

Main Results:

  • Eighteen pediatric patients with CTD and severe MR underwent the simplified repair.
  • The procedure was associated with a low mortality rate (5.6%) and no other major complications.
  • At a median follow-up of 2.4 years, 94.4% of survivors had mild or less regurgitation, with no SAM or significant stenosis.
  • Left ventricular dimensions regressed significantly post-repair.

Conclusions:

  • A simplified surgical approach is effective for managing severe MR in pediatric patients with CTDs.
  • This technique achieves intermediate-term mitral valve competence and prevents adverse events like SAM.
  • The simplified repair promotes regression of left ventricular enlargement in this patient cohort.
Abstract

Keywords:
connective tissue disordersmitral valve repair

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