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Scimitar syndrome presenting in infancy.

C B Huddleston1, V Exil, C E Canter

  • 1Department of Surgery, Washington University School of Medicine, St. Louis Children's Hospital, Missouri 63110, USA.

The Annals of Thoracic Surgery
|March 23, 1999
PubMed
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Infants with Scimitar syndrome often present with tachypnea and dextrocardia. Right pneumonectomy showed similar outcomes to surgical repair, offering a viable treatment option for this rare congenital condition.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Thoracic Surgery

Background:

  • Scimitar syndrome presentation varies with age at diagnosis.
  • Infants with heart failure have more anomalies and worse prognosis.

Purpose of the Study:

  • To analyze the presentation and outcomes of infants diagnosed with Scimitar syndrome.
  • To evaluate different treatment strategies for infantile Scimitar syndrome.

Main Methods:

  • Retrospective review of 12 infants with Scimitar syndrome at St. Louis Children's Hospital.
  • Analysis of clinical presentation, diagnostic findings, and treatment outcomes.

Main Results:

  • Tachypnea and dextrocardia were common presenting symptoms.

Related Experiment Videos

  • Pulmonary hypertension and anomalous venous return were significant findings.
  • Right pneumonectomy demonstrated comparable early and late results to surgical repair.

    • Conclusions:

    • Infantile Scimitar syndrome typically presents with tachypnea, dextrocardia, and hypoplastic right lung.
    • Right pneumonectomy is a successful therapeutic option, either primarily or after failed repair.