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Comparative Lesions Analysis Through a Targeted Sequencing Approach
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Published on: November 5, 2019

The scimitar syndrome: an Italian multicenter study.

Vladimiro L Vida1, Simone Speggiorin, Massimo A Padalino

  • 1Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Padua, Italy.

The Annals of Thoracic Surgery
|July 28, 2009
PubMed
Summary

Intraatrial baffle repair for scimitar syndrome (a rare congenital heart defect) showed fewer complications and better long-term results than vein reimplantation. This multicenter study highlights surgical management of this rare condition.

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Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Scimitar syndrome is a rare congenital heart malformation.
  • Surgical management is complex and outcomes vary.
  • This multicenter study presents the largest published series on surgical correction.

Purpose of the Study:

  • To evaluate the surgical management outcomes of scimitar syndrome.
  • To compare the efficacy of different surgical repair techniques.
  • To assess hospital mortality and long-term repair efficacy.

Main Methods:

  • A multicenter study included 26 patients with scimitar syndrome undergoing surgical correction from 1997 to 2007.
  • Patients were divided into two groups: intraatrial baffle repair (n=18) and "scimitar vein" reimplantation (n=8).
  • Primary outcomes were hospital mortality and repair efficacy at follow-up.

Main Results:

  • The median age was 11 years, with common symptoms including respiratory infections and pneumonia.
  • Intraatrial baffle repair (group 1) had lower postoperative complications (22%) and a lower incidence of scimitar vein occlusion (12%) compared to reimplantation (group 2: 62% complications, 25% occlusion).
  • One hospital death occurred in group 1, and one late death in group 2. Stenosis requiring intervention occurred in 6% of group 1 and 25% of group 2.

Conclusions:

  • Intraatrial baffle repair appears to be associated with a lower incidence of postoperative complications.
  • The intraatrial baffle technique demonstrated a better patency rate at follow-up compared to scimitar vein reimplantation.
  • Surgical repair using the intraatrial baffle method is a favorable option for managing scimitar syndrome.